Peripheral Vascular Disease for Cardiologists: A Clinical Approach

Peripheral Vascular Disease for Cardiologists A CLINICAL APPROACH John A. Spittell, Jr, MD, MACP, FACC Emeritus Profes...

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Peripheral Vascular Disease for Cardiologists A CLINICAL APPROACH John A. Spittell, Jr,

MD, MACP, FACC

Emeritus Professor of Medicine, Mayo Medical School

Futura, an imprint of Blackwell Publishing

Peripheral Vascular Disease for Cardiologists A CLINICAL APPROACH

To my wife Beverly Spittell, in appreciation of her encouragement, patience, and loyalty

Peripheral Vascular Disease for Cardiologists A CLINICAL APPROACH John A. Spittell, Jr,

MD, MACP, FACC

Emeritus Professor of Medicine, Mayo Medical School

Futura, an imprint of Blackwell Publishing

# 2004 by Futura, an imprint of Blackwell Publishing Blackwell Publishing, Inc./Futura Division, 3 West Main Street, Elmsford, New York 10523, USA Blackwell Publishing, Inc., 350 Main Street, Malden, Massachusetts 02148-5020, USA Blackwell Publishing Ltd, 9600 Garsington Road, Oxford OX4 2DQ Blackwell Science Asia Pty Ltd, 550 Swanston Street, Carlton, Victoria 3053, Australia All rights reserved. No part of this publication may be reproduced in any form or by any electronic or mechanical means, including information storage and retrieval systems, without permission in writing from the publisher, except by a reviewer who may quote brief passages in a review. 04 05 06 07 5 4 3 2 1 ISBN: 1-4051-0366-3 Library of Congress Cataloging-in-Publication Data Spittell, John A., 1925– Peripheral vascular disease for cardiologists : a clinical approach / John A. Spittell Jr. p. ; cm. Includes bibliographical references and index. ISBN 1–4051–0366–3 1. Peripheral vascular disease. 2. Cardiologists. [DNLM: 1. Peripheral Vascular Diseases. 2. Heart Diseases—etiology. 3. Peripheral Vascular Diseases—complications. WG 500 S761p 2003] I. Title. RC694 .S67 2003 2003019458 616.10 31—dc22 A catalogue record for this title is available from the British Library Acquisitions: Steven Korn Production: Julie Elliott Typesetter: Kolam Information Services Pvt. Ltd, Pondicherry, India Printed and bound by MPG Books Ltd, Bodmin, Cornwall, UK For further information on Blackwell Publishing, visit our website: www.futuraco.com

Illustration on cover reprinted from Fairbairn JFII. Clinical manifestations of peripheral vascular disease. In: Juergens JL, Spittell JA Jr., Fairbairn JFII (eds). Peripheral Vascular Diseases, 5th edn. Philadelphia: W. B. Saunders Co., 1980: 17, by permission of Mayo Foundation.

Contents

Foreword, vi Preface, vii 1 Occlusive peripheral arterial disease, 1 2 Aneurysmal disease, 30 3 Aortic dissection, penetrating atherosclerotic aortic ulcer, and intramural

hematoma, 50 4 Arteritis, 61 5 Vasospastic disorders, 68 6 Venous disorders, 77 7 Leg edema, 92 8 Leg and foot ulcers, 100 9 Vascular clues to a diagnosis, 106 10 Some uncommon peripheral vascular disorders, 117

Index, 131

v

Foreword

It is a pleasure to write this Foreword for the book Peripheral Vascular Disease for Cardiologists: A Clinical Approach by John A. Spittell Jr, MD. I have known Dr Spittell since 1972 (when he recruited me to the Mayo Clinic) and I have great respect for the work that he has done in the area of peripheral vascular diseases and for his training and impact on so many clinicians interested in this field. In the Preface, Dr Spittell has stated that the purpose of writing this book is to improve the cognitive skills of the cardiologist in the field of peripheral vascular diseases. As he rightly points out, ‘‘With the current difficulty that some patients encounter in gaining access to continuing primary care, they may call upon their cardiologist for noncardiac care, some of which may be in the peripheral vascular domain. In addition, achievement and maintenance of cardiovascular subspecialty certification will be facilitated by a working knowledge of peripheral vascular disorders . . . .’’ Indeed, Dr Spittell has successfully provided an excellent and simplified clinical approach to the wide variety of peripheral vascular diseases which will fulfill the interest of cardiologists, internists, other health professionals, and students. The clinical ‘‘pearls’’ of this book relate to the vast clinical experience of Dr Spittell. For this reason, there are a large number of statements based on his opinion which are quite fair since we don’t have the answers yet except for the experience of great clinicians as the author of this book. Such clinical ‘‘pearls’’ are particularly relevant in the chapters on Leg Edema, Leg and Foot Ulcers, Vascular Clues to a Diagnosis, and Some Uncommon Peripheral Vascular Disorders, which are excellent. Dr John A. Spittell has done more to encourage involvement of the cardiovascular community in peripheral vascular diseases than any other individual I know. This book, based on his vast experience as a clinician and present knowledge, is a valuable learning aid for cardiologists, other health professionals, and students who wish to gain practical information about the important field of peripheral vascular diseases. Valentin Fuster, MD, PhD Director, Cardiovascular Institute and Health Center Mount Sinai Medical Center, New York Past President AHA President Elect World Heart Federation

vi

Preface

There are a number of reasons for cardiologists to be knowledgeable about at least the common peripheral vascular disorders that can coexist with, or complicate the management of, their patients with cardiac disease. There are instances as well when clinical manifestations suggestive of a cardiac disease are actually due to an important vascular problem which may be overlooked unless the clinician is aware of it. Peripheral vascular findings may also alert the informed cardiologist of an otherwise significant but occult clinical problem. With the current difficulty that some patients encounter in gaining access to continuing primary care, they may call upon their cardiologist for noncardiac care, some of which may be in the peripheral vascular domain. In addition, achievement and maintenance of cardiovascular subspecialty certification will be facilitated by a working knowledge of peripheral vascular disorders since the cardiovascular subspecialty examinations have had an increasing content of peripheral vascular material. Furthermore, the topic has been ‘‘inadequately addressed in many, if not most, training programs in internal medicine and its subspecialty of cardiology’’;1 hopefully, this will be corrected with the recent recommendation of the vascular medicine task force ‘‘that cardiologists must have adequate basic training in vascular medicine to acquire a sufficient knowledge base to care for the many patients with peripheral vascular disease.’’2 The importance of this area of cardiovascular disease has been emphasized further by the Atherosclerotic Vascular Disease Conference (July 2002) sponsored by the American Heart Association to outline strategies for research and educational programs in atherosclerotic peripheral vascular disease. This book is intended to fulfill a need for those cardiovascular specialists who wish to broaden their knowledge base and clinical skills in the recognition and management of common peripheral vascular disorders that occur in cardiologic practice. Accordingly, peripheral vascular disease will be presented in a practical clinical framework rather than the usual textbook format. For rapid reference to specific disorders or findings, a detailed index has been provided. In selecting references, I have tried to cite original descriptions or those which are, in my opinion, key references or those of particular clinical value. Many persons, colleagues, paramedical personnel, and patients have been extremely helpful to me over the past four and a half decades of my clinical practice. Obviously, they are so numerous that I cannot mention all, but several were absolutely essential to my choice of peripheral vascular disease as an area of special interest. Drs E. V. Allen, N. W. Barker, and E. A. Hines, vii

viii Preface

pioneers in the field of peripheral vascular disease, were the persons who influenced and attracted many of us in our residency and fellowship years at Mayo, by their enthusiasm, knowledge, and clinical skills in an area of cardiovascular disease that had been given little, if any, attention in our medical school curriculum.3 Many other colleagues (coauthors and other associates) have been important teachers, critics, and sources of ‘‘clinical pearls.’’ Likewise, many paramedical persons (librarians, artists, photographers, and secretaries) have been generous in their assistance to me over the years and I am grateful to them. I would be remiss if I did not credit Dr Suzanne B. Knoebel for really starting this project when she was the editor of the ACC Current Journal Review and asked me in 1993, and again in 2000, to write an article on ‘‘What the cardiologist should know about peripheral vascular disease.’’4,5 The assistance of Mrs Roberta Schwartz in the Section of Publications at Mayo Clinic in getting this book-writing effort ‘‘in focus’’ for me in the very beginning was most helpful. Of special note is my former secretary, Mrs Debra DeCook, who in her own time has patiently typed and retyped the manuscript and all the correspondence required to produce this book.

References 1 O’Rourke R.A. (1990) Foreword. Curr Prob Cardiol 15: 3. 2 Beller G.A., Bonow R.O. & Fuster V. (2002) ACC revised recommendations of the 1995 COCATS training states. Introduction. JACC 39: 1242–1246. 3 Spittell J.A. Jr & Fairbairn J.F. II (1992) Vascular medicine at Mayo Clinic. Some highlights of the early history and development as a subspeciality. Int Angiol 11: 2–7. 4 Spittell J.A. Jr. (1993) What the cardiologist should know about peripheral vascular disease. ACC Curr J Rev 2: 19–20. 5 Ibid. (2000) 9: 51–53.

CHAPTER 1

Occlusive peripheral arterial disease

Atherosclerotic peripheral vascular disease Disorders caused by atherosclerosis head the list of peripheral vascular diseases that a cardiologist may encounter as comorbid conditions in patients with coronary artery disease. These include occlusive peripheral arterial, carotid, and renal disease, and aneurysmal disease of the aorta and the extremity arteries and their complications.

Occlusive peripheral artery disease First of all, atherosclerotic occlusion (ASO) in the extremities, whether manifested by symptoms, absent pulses, or an abnormal ankle–brachial index (ABI),1 is associated with a more-than-four-times higher risk of cardiovascular mortality. In patients with intermittent claudication due to ASO, significant coronary artery disease is present by angiography in more than 50%.2 Clearly then, inclusion of the extremity arterial circulation in the medical history and physical examination of coronary disease patients provides prognostic information warranting aggressive risk factor management. A point worth noting here is that a decreased or absent posterior tibial pulse is the best noninvasive indication of occlusive arterial disease upstream;3 the posterior tibial artery is virtually never absent congenitally while the dorsal pedis pulse may be absent in about 12% of patients.4 Auscultation over large arteries such as the carotid, subclavian, abdominal aorta, and renal and common femoral artery in the groin for bruits should be a part of the physical examination. The presence of a bruit usually indicates turbulence of flow due to stenosis upstream. While most bruits are systolic in timing, when the bruit flows into diastole the stenosis upstream is often in the range of 80% of the lumen (Figure 1.1), i.e. severe enough to cause a gradient and, therefore, flow in diastole – a significant clinical finding. While on the subject of bruits, examination of the carotid artery should be considered. It is preferable to palpate for the carotid artery pulsation with the patient supine and the stethoscope over the precordium during the examination. Monitoring the heart rate during the carotid palpation helps recognize any bradycardia (as a result of carotid sinus sensitivity) promptly and cease carotid artery palpation. Also in the supine position there is less chance of the patient falling if there is any unsteadiness or loss of consciousness. It is also 1

2 Chapter 1

Figure 1.1 Arterial bruits in occlusive arterial disease. (a) Systolic bruit with slight to moderate stenosis upstream. (b) Systolic bruit flowing into diastole in severe (80% or greater) arterial stenosis upstream.

helpful to have patients hold their breath while the clinician listens for carotid bruits. To minimize patient discomfort the clinician should hold his/her breath at the same time.

Chronic ASO in the lower extremity While intermittent claudication may variously be described as ‘‘cramping’’ or ‘‘fatigue’’ or some other type of discomfort, it has the consistent feature of occurring while walking and being relieved by standing still. Among the musculoskeletal disorders that may mimic true intermittent claudication, pseudoclaudication due to lumbar spinal stenosis is the principal one. Differentiation clinically is usually not difficult (Table 1.1) but an exercise study gives useful confirmation (Figure 1.2). Occasionally, true claudication due to ASO and pseudoclaudication due to spinal stenosis coexist. In these cases evaluation with a noninvasive test with exercise, CT scan or MRI of the lumbar spine, and angiography may be required to determine which is the most serious problem and whether it warrants correction. Indeed, at times both the occlusive arterial disease and the spinal stenosis need treatment to restore the quality of life the patient desires. In addition to examination and grading of the extremity pulses, elevationdependency tests and noninvasive testing are useful to materially improve

Occlusive peripheral arterial disease 3 Table 1.1 True claudication vs. pseudoclaudication Claudication

Pseudoclaudication

Onset

Walking

Erect posture, i.e. walking or standing

Discomfort

Cramp, ache, fatigue

Paresthesia, pain, weakness

Bilateral?

+

Generally bilateral

Relief

Stand still

Sit down, lean on something, flex spine

Cause

Occlusive peripheral arterial disease

Spinal stenosis

Figure 1.2 An office-based exercise study can be performed by determining the ankle–brachial index (ABI) before and after having the patient perform up to 50 consecutive plantar flexions in the erect posture. (a) Before plantar flexion. (b) Plantar flexion while keeping knees straight. (From McPhail I.R., Spittell P.C., Weston S.A., et al. (2001) Intermittent claudication: An office-based assessment. JACC 37: 1381–1385. By permission of the Elsevier Science, Oxford, UK.)

clinical evaluation of occlusive peripheral arterial disease. A clinically useful maneuver, often overlooked, is elevation of the lower extremities to 60 above the level and observing any change in the color of the skin of the soles. With this maneuver, no pallor of the skin will develop in 60 seconds if the arterial circulation in the lower extremity is normal or minimally occluded, but if definite pallor (Figure 1.3a) develops, then the occlusive arterial disease in that extremity is significant – a valuable determination that can be made in 60

4 Chapter 1

Figure 1.3 Elevation-dependency test for occlusive peripheral arterial disease. (a) Pallor of the right foot on elevation. Patient had an ankle–brachial index (ABI) of 0.32 in the right leg and 0.80 in the left leg. (b) Delayed color return and filling of the superficial veins of the foot, with dependency after elevation, in a patient with occlusive arterial disease in the left lower extremity. (From Spittell J.A. Jr (1990) Diseases and management of occlusive peripheral arterial disease. Curr Probl Cardiol 15: 3–35. By permission of Mosby, Inc.)

seconds in the office or at the bedside. After performing the elevation test for 60 seconds, the patient can then sit up and hang down his/her feet for the time required for the color to return to the skin and for the superficial veins on the dorsum of the foot to fill (Figure 1.3b). This can be a confirmatory finding of the adequacy of the circulation in the extremity (Table 1.2). When ischemia becomes severe and causes pain at rest, the patient will often hang the ischemic foot over the side of the bed to try to get relief. As a result a deep ruborous color develops along with dependent edema (Figure 1.4), indicative of critical limb ischemia. Although careful clinical examination is reliable in diagnosing occlusive arterial disease in the lower extremity, the ABI has become the objective standard for diagnosis. The ABI is readily obtained by measuring the systolic blood pressure at the brachial and ankle levels with the patient supine; a handheld Doppler instrument is most useful for detecting the systolic blood pressure (Figure 1.5). Normally the supine systolic pressure at the ankle level exceeds that at the brachial level, while with occlusive arterial disease in the lower extremity the systolic pressure at the ankle is lower than the brachial systolic pressure, usually in proportion to the degree of arterial insufficiency. However, the ABI is not a valid measure of arterial disease in the patient with

Occlusive peripheral arterial disease 5 Table 1.2 Elevation-dependency testing Elevation pallor * Grade

Appearance time (sec)

0 1 2 3 4

No pallor in 60 Definite pallor in 60 Definite pallor in < 60 Definite pallor in < 30 Pallor on level Dependency (Time to:)

Ischemia

Color return (sec)

Venous filling (sec)

None Moderate Severe

10 15–20 > 40

15 20–30 > 40

*Elevation of extremity at angle of 60 above level. Reproduced from Spittell J.A. Jr (1994) Peripheral arterial disease. Disease-A-Month 40: 641–704. By permission of Mosby, Inc.

Figure 1.4 Dependent edema and an ischemic ulcer of the toe in a patient with critical limb ischemia and ischemic rest pain. Not seen in this black-and-white photograph is the deep ruborous color – so-called dependent rubor – indicative of severe ischemia.

medial calcinosis – often a diabetic – that causes the systolic pressure at the ankle level to be spuriously elevated. In the diabetic patient with ASO and medial calcinosis causing spuriously elevated systolic ankle blood pressures, toe blood pressures or transcutaneous oximetry can be used to document the

6 Chapter 1

Figure 1.5 Determining the ankle–brachial index (ABI). (a) Determining supine systolic brachial blood pressure. (b) Determining supine systolic ankle blood pressure. (Reproduced from Spittell J.A. Jr & Spittell P.C. (2000) Peripheral Vascular Disease in ACCSAP. American College of Cardiology Foundation, Bethesda, MA. By permission of the American College of Cardiology.)

Occlusive peripheral arterial disease 7

Figure 1.6 The use of transcutaneous oximetry. (a) Ischemic ulcer of the right first toe of an 83-yearold diabetic woman. On examination, pulses were not palpable below the groin and definite pallor of the right foot developed after 40 seconds of elevation of the right lower extremity. However, systolic ankle pressures at the ankle level were more than 300 mm Hg, due to the presence of medial calcinosis as shown in (b). Transcutaneous oximetry showed regional perfusion indices of 0.35 (normal > 0.8) supine and 0 – 0.1 (normal > 0.65 after elevation of the right foot at 30 for 3 minutes), confirming the severe ischemia of the right foot, resulting from the distal occlusive arterial disease demonstrated arteriographically in (c). (From Spittell J.A. Jr (1990) Diagnosis and management of occlusive peripheral arterial disease. Curr Probl Cardiol 15: 3–35. By permission of Mosby, Inc.)

8 Chapter 1

Figure 1.7 Patient with left hip pain on walking short distances, relieved by stopping and standing still. Ankle–brachial indices (ABIs) were normal bilaterally before and after exercise. X-ray of left hip was normal. (a) MRA showing left hypogastric (internal iliac) artery stenosis. (b) Angiogram of the left hypogastric (internal iliac) artery pre- and post-percutaneous balloon angioplasty which relieved his left hip pain with walking.

occlusive disease and ischemia (Figure 1.6). When the ABI is measured before and after standard exercise, e.g. active pedal plantar flexion (Figure 1.2),5 an accurate estimate of any arterial insufficiency and the functional impairment it is imposing can be obtained. Angiography can give precise definition of the occlusive arterial disease but is not necessary for diagnosis. It should be reserved for those instances when restoration of pulsatile flow is planned, in the complex situation where true and pseudoclaudication coexist, or in the rare situation of isolated internal iliac (hypogastric) artery occlusive disease causing buttock and/or hip claudication but where on examination the femoral and more distal arterial pulses and the ABI remain essentially normal (Figure 1.7).


Today, of course, imaging of the aorta and peripheral arteries is possible without the use of contrast media, using instead ultrasound with color-flow Doppler or by magnetic resonance angiography (MRA) (Figure 1.7); these are preferable to contrast angiography in the patient with a history of allergy to contrast media and in the patient with renal insufficiency.

Chronic ASO in the upper extremity While most attention is focused on ASO in the lower extremities, the upper extremity arterial circulation has become important, particularly to cardiologists, for several reasons. The increasing use of the internal mammary artery, a branch of the subclavian artery, makes clinical detection of ASO of the origin of the subclavian artery (a ‘‘smoker’s lesion’’) desirable when coronary artery bypass is being considered. Determination of the blood pressure in both arms and simultaneous palpation of the radial arteries on a routine basis makes identification of a unilateral ‘‘delayed’’ radial pulsation and reduced blood pressure in the patients with ASO of the origin of the ipsilateral subclavian artery – so-called ‘‘subclavian steal’’6 (Figure 1.8) – easy. Likewise, if a patient

Figure 1.8 Subclavian ‘‘steal.’’ (a) Drawing of simultaneous radial artery palpation and clinically detectable delay of left radial artery pulsation. (From Spittell J.A. Jr & Spittell P.C. (2000) Peripheral Vascular Disease in ACCSAP. By permission of the American College of Cardiology.) (b) Arch aortogram in a 65-year-old woman, a veteran smoker, demonstrating stenosis of the proximal left subclavian artery (a ‘‘smoker’s lesion’’). (c) Later phase of arch aortogram showing opacification of the left vertebral artery – the subclavian ‘‘steal.’’ (From Spittell J.A. Jr (1994) Peripheral arterial disease. Disease-A-Month 40(12): 641–704. By permission of Mosby, Inc, St Louis, MA.)

10 Chapter 1

Figure 1.8 continued

who has had an internal mammary artery used as a bypass to a coronary artery (e.g. LIMA to LAD) develops recurrent angina, one of the causes to consider is ASO of the origin of the ipsilateral subclavian artery (Figure 1.9).7 Mentioning simultaneous palpation of both radial arteries brings to consideration another simultaneous arterial pulse palpation – the radial and


Figure 1.9 Drawings of the arch of the aorta with and without subclavian artery stenosis (right and left) and of the heart and aortic arch after coronary artery bypass using the left internal mammary artery to the left anterior descending coronary artery. (From Spittell J.A. Jr & Spittell P.C. (2000) Peripheral Vascular Disease in ACCSAP. American College of Cardiology Foundation, Bethesda, MA. By permission of the American College of Cardiology.)

femoral artery – that should be a part of every physical examination lest coarctation of the aorta be missed (Figure 1.10). Upper extremity arterial circulation deserves mention for another reason – the Allen test8 (Figure 1.11), which was originally described as an aid in the diagnosis of thromboangiitis obliterans (TAO) (Buerger’s disease), but is now a part of cardiologic practice when the radial artery is being considered for access or use as a conduit in patients with coronary artery disease. In their modification of the Allen test, Conklin and associates9 provide a timed endpoint indicating adequacy of the collateral circulation by the ulnar artery, i.e. while the radial artery compression is maintained, there is return of color to the thenar eminence and thumb within 5 seconds of release of the ulnar artery compression (Figure 1.11a).

Natural history and prognosis In planning management of an individual patient with symptomatic lower extremity ASO, it is useful to keep the prognosis and natural history of his/ her disease in mind. If the patient has only intermittent claudication and is

12 Chapter 1

Figure 1.10 Simultaneous palpation of the radial and femoral arteries. (a) Drawing of simultaneous radial and femoral artery palpation with delay of femoral pulsation due to coarctation of the aorta. (b) Simultaneous pulse tracings from a patient with coarctation of the aorta showing the delayed femoral artery pulsation.

not diabetic, the outlook for limb survival is favorable to the extent that only about 5% of such persons will lose a limb over the ensuing 5 years.10 Furthermore, the level of the occlusive arterial disease is not predictive of progression of the arterial disease to critical ischemia in the affected limb.11 On the other hand, when critical ischemia is present – ischemic pain at rest or ischemic ulceration (Figure 1.4) – the prognosis for limb loss is worsened


Ulnar artery MAYO 1981

MAYO 1981

Radial artery

(a)

Superficial palmar arch

Radial artery Ulnar artery

Occlusion

Occlusion

Radial artery

Ulnar artery

Ulnar artery

MAYO 1981

Radial artery

(b) Occlusion

Figure 1.11 The Allen test. (a) Normal (negative) result, indicating patency of ulnar artery and superficial palmar arch. (b) Abnormal (positive) results due to occlusion of ulnar artery (left), radial artery (right), and superficial palmar arch (center). (From Spittell J.A. Jr (1982) Occlusive peripheral arterial disease: Guidelines for office management. Postgrad Med 71: 137–151. By permission of McGraw-Hill Book Co, Inc.)

14 Chapter 1

severalfold, even in the nondiabetic person.12 When a person has both symptomatic ASO and diabetes mellitus, the risk for limb loss is about four times that seen in nondiabetic persons with ASO.13 In addition to the above, the details of the frequency of coronary artery disease in association with extremity ASO2 need to enter management decisions as treatment directed at the peripheral arterial disease will not affect coronary and cerebral atherosclerotic arterial diseases,14 which are the causes of a shortened life expectancy of these persons. With these prognostic parameters in mind, it is clear that restoration of pulsatile flow to the ischemic limb is indicated, whenever feasible, for the diabetic person with symptomatic ASO and for all patients who have critical limb ischemia, i.e. ischemic rest pain and/or ischemic ulceration. However, for the nondiabetic person whose only problem is intermittent claudication, restoration of pulsatile flow to the affected limb(s) is elective. It is therefore indicated only when the patient feels that his/her intermittent claudication is disabling or significantly affecting the quality of life and, in addition, understands the goals, risks, and limitations of the intervention or surgical procedure planned to improve their walking distance. In the case of ASO of the origin of the subclavian artery (Figures 1.8 and 1.9), the need for restoring pulsatile flow to the upper extremity depends on whether the patient is experiencing any symptoms from it. If the ipsilateral internal mammary artery has been used as a bypass to a coronary artery and angina recurs, relief of the narrowing of the origin of the subclavian artery, if present, is indicated.7 It should be pointed out that a favorite peripheral location for giant cell (cranial, temporal) arteritis (seen in persons over 50 years of age) to involve is the subclavian artery, and such patients may present with the dominant complaint of arm claudication (Figure 1.12).

Management In an effort to delay progression of the atherosclerosis, attention to the controllable risk factors – smoking, diabetes mellitus, hyperlipidemia, and hypertension – is basic. The regular use of aspirin, or clopidogrel, is reported to decrease the incidence of atherosclerotic complications15 and is desirable unless contraindications to their use exist. Education of the person with occlusive peripheral arterial disease in measures to protect their ischemic limbs from all types of trauma – mechanical, chemical, and thermal – can prevent many limb-threatening problems. Attention to footwear needs to be stressed. Properly fitting and protective shoes which do not have straps that injure the skin should be advised to prevent injury to the feet or toes (Figure 1.13). In addition, regular application of a bland


Figure 1.12 Giant cell arteritis. (a) & (b) Subclavian angiograms were done in a 65-year-old woman who presented with claudication and decreased pulses in arms. Note typical angiographic findings of tapered stenotic segments bilaterally in subclavian, axillary, and brachial arteries. (From Fulton R.E., Stanson A.W., Forbes G.S., et al. (1980) Vascular imaging. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)

lotion, or ointment, containing lanolin to callused skin of the feet can prevent fissuring (Figure 1.14) that can serve as a portal for infection. Since many amputations are the end result of such preventable trauma,16 regular foot-care by professionals trained in the care of ischemic extremities can also lessen limbthreatening trauma. Educating the patient regarding footcare provides an opportunity, if the patient is a smoker, to stress the need to stop the use of tobacco, citing the severalfold increase in limb loss that continued tobacco use invokes.17 For therapy of associated health problems (e.g. migraine, hypertension), it is desirable to use drugs that do not have a vasoconstrictive action when effective alternative therapeutic agents are available. At this point, it is worth noting that available vasodilator agents are not effective as treatment for claudication since their effect is on the cutaneous circulation and not on muscle.18 The drugs that may give modest improvement in the walking distance in selected patients are pentoxifylline19 and the more recently approved cilastazol;20 the latter, however, is contraindicated in patients with heart failure. For the person with intermittent claudication, whether restoration of pulsatile flow is planned or not, a regular walking program – e.g. walking for 30 minutes, and stopping as needed to relieve intermittent claudication or angina pectoris, 5 days a week – should be included in their therapy. Such a regular walking program for several months can increase the comfortable walking distance to a level acceptable to some patients with ASO. When restoration of pulsatile flow is indicated, the choice of the most appropriate procedure, percutaneous angioplasty or vascular surgery or both should be individualized for each patient. In the ideal situation this decision is jointly made by the clinician, the interventionalist, and the vascular surgeon.

16 Chapter 1

Figure 1.13 (a) Ischemic ulceration on dorsum of foot produced by strap of lady’s shoe. (From Spittell J.A. Jr (1998) Peripheral vascular disease. In Wenger N.K. (ed.) Cardiovascular Disease: Recognition and Management in the Octogenarian and Beyond. London: Martin Dunitz Ltd. By permission of Martin Dunitz Ltd.) (b) Gangrene of the right third toe of a 77-year-old man with asymptomatic occlusive peripheral arterial disease. Gangrenous toe resulted from his wearing the ‘‘too small’’ shoes of a recently deceased neighbor. (From Spittell J.A. Jr (1983) Office and bedside diagnosis of occlusive arterial disease. Curr Probl Cardiol 8: 5–34. By permission of Mosby, Inc.)

Figure 1.14 Fissures of the skin of the heel. These can be painful as well as a portal of infection in the ischemic limb.


Recently, it has been reported that complete wound healing with limb preservation was achieved by over 40% of patients with critical limb ischemia with the use of intermittent compression pump treatment.21 This may be an alternative approach for patients with inoperable critical limb ischemia. Therapeutic arteriogenesis for revascularizing ischemic tissue via various methods (gene therapy, protein infusion, or stem cells) has great potential but further work is needed before this technology can be applied to patients.22 What is needed is a way to create mature stable vessels.

Thromboangiitis obliterans (Buerger’s disease) TAO23 is uncommon and affects younger persons; so its occurrence in the usual cardiologic practice is infrequent. Nonetheless, it is desirable for cardiovascular specialists to be aware of its clinical features (Table 1.3; Figure 1.15) and the precise relationship of its activity to tobacco use – stopping tobacco permanently always halts the activity of TAO. Unless tobacco use is stopped, TAO activity continues and amputation often results. A recent study has confirmed our belief that the percentage of women with (TAO) is increasing.24 Table 1.3 Clinical features of thromboangiitis obliterans (TAO) (Buerger’s disease)

Onset before age 30 yr Men > women Tobacco use Symptoms . Raynaud’s phenomenon . Superficial thrombophlebitis . Claudication, arch or calf Physical findings . Small artery disease . Upper and lower extremities involved

Popliteal artery entrapment Like TAO, popliteal artery entrapment23 is usually symptomatic in younger persons. It is notable for several reasons, however; popliteal artery entrapment, if not recognized and treated surgically when the only symptom is calf claudication, can result in complete occlusion of the popliteal artery or development of a popliteal artery aneurysm. Thus, keeping popliteal artery entrapment in mind as a possible cause of calf claudication in younger persons is desirable; it can be readily diagnosed by MRA (Figure 1.16). The treatment is surgical release of the muscle or tendon that is compressing the popliteal artery.

18 Chapter 1

Figure 1.15 Thromboangiitis obliterans (TAO) (Buerger’s disease). (a) Superficial thrombophlebitis, dorsum of foot of 22-year-old man who smokes cigarettes. (b) Arteriogram showing typical segmental occlusions of arteries at the ankle level in a man with TAO. (From Spittell J.A. Jr (1990) Diagnosis and management of occlusive peripheral arterial disease. Curr Probl Cardiol 15: 7–35. By permission of Mosby, Inc.)

Acute arterial occlusion Acute embolic occlusion of a peripheral artery can be the initial or principal manifestation of a previously unknown cardiac lesion as well as a complication of known cardiac disease, aortic atherosclerosis or arterial aneurysm (Table 1.4). Acute thrombotic arterial occlusion, on the other hand, is a complication of an existing arterial disease – atherosclerotic or arteritic – or of an hypercoagulable state. Thus, embolic arterial occlusion is the type most likely seen in a cardiologic practice and its presentation is usually a dramatic event unless the artery occluded is quite small. The clinical manifestations may be the five Ps (Table 1.5) or a more subtle event such as a cold blue digit and/or livedo reticularis (see Figure 2.6) as seen in atheroembolism. Echocardiography has become an invaluable part of the evaluation of the patient with an acute arterial occlusion that is not clearly the thrombotic type. Many of the acute occlusions of uncertain etiology in the past are now known to be due to emboli arising from atherosclerotic lesions in the aorta identified by echo (Figure 1.17).


Figure 1.16 Popliteal artery entrapment. (a) Arteriogram demonstrating segmental occlusion of right popliteal artery due to entrapment by gastrocnemius muscle in a 34-year-old man with intermittent calf claudication. (From Juergens J.L. & Pluth J.R. (1980) Trauma and peripheral vascular disease. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.) (b) Demonstration by MRA with plantar flexion causing effacement of popliteal artery with plantar flexion in a man with symptomatic popliteal artery entrapment. (From Spittell J.A. Jr & Spittell P.C. (2000) Peripheral Vascular Disease in ACCSAP. American College of Cardiology Foundation, Bethesda, MA. By permission of the American College of Cardiology.)

20 Chapter 1

Thrombotic suggested by . Prior occlusive arterial disease . Occlusive disease, other extremities . Hypercoagulable states such as Myeloproliferative disease Neoplasm Inflammatory bowel disease Hereditary coagulation factor deficiency Embolic suggested by . Cardiac disease . Atrial fibrillation . Proximal arterial aneurysm . Proximal atherosclerosis

Table 1.4 Clinical clues in acute arterial occlusion

From Spittell J.A. Jr (1994) Peripheral arterial disease. Disease-A-Month 40: 644–704. By permission of Mosby, Inc.

The five Ps

Table 1.5 Acute arterial occlusion

Pain Pallor Pulseless Paresthesia Paralysis

Figure 1.17 Transesophageal echocardiogram showing a mobile thrombus in the ascending aorta near the origin of the left carotid artery in a woman having left carotid territory transient cerebral ischemic attacks.


Before discussing therapy of acute peripheral arterial occlusion, two conditions which mimic acute arterial occlusion warrant mention – acute aortic dissection (Figure 1.18) and ergotism (Figure 1.19). In the case of acute dissection, the artery occluded can be any of the branches of the aorta along the course of the dissection. Ergotism results from the excessive use of ergot-containing suppositories for the treatment of migraine and is frequently bilateral in the upper or lower extremity arteries. Treatment with sodium nitroprusside, after stopping the ergot, is effective in relieving the arterial spasm. While evaluating the patient with an acute arterial occlusion, heparinization to protect the compromised circulation (unless there is a possibility of ‘‘delayed-onset’’ heparin-induced thrombocytopenia25) and protection of the affected limb from injury (Table 1.6) are important. After arteriography, revascularization of limb-threatening arterial occlusion can be achieved surgically or by thrombolysis. The latter is the better choice as the initial therapy, followed by surgical or interventional treatment of arterial lesions unmasked by the thrombolysis.26

Carotid artery disease The clinical presentations of extracranial cerebrovascular disease include asymptomatic bruit, transient cerebral or ocular ischemia, and stroke. Localized carotid bruits are present in about 5% of persons over the age of 65 years, but not all are hemodynamically significant. A technique and precautions in examining the carotid artery is described in the section on physical examination for occlusive arterial disease (page 1). In addition to being an indicator of an increased risk of stroke, carotid bruits are an indication of likely coexistent coronary artery disease. Increased risk of stroke in the person with an asymptomatic carotid bruit is suggested by several findings – a bruit that extends into diastole (Figure 1.1), ipsilateral retinal artery emboli (Figure 1.20 p. 25), and/or demonstrated hemodynamic significance of the carotid stenosis. Cardiologists are often consulted when patients describe episodic attacks of cerebral ischemia because of the concern about cardiac rhythm disturbances. At times, of course, such attacks are due to extracranial cerebrovascular disease or cerebral arterial embolism. Transient cerebral ischemic attacks (TIAs) are characteristically spontaneous and brief with focal neurologic and/or visual symptoms without loss of consciousness. The features of TIA in the carotid territory are sufficiently different from those involving the vertebral-basilar artery circulation (Table 1.7 p. 25) so that clinical recognition is usually not difficult.

22 Chapter 1

Figure 1.18 (a) Drawing by Swaine in 1856 of aorta from a case of acute aortic dissection. Note re-entry into left common iliac artery and associated thrombus. (Reproduction of illustration of Swaine’s case reported by Latham P.M. (1856) Transactions of Pathological Society of London 7:106. Provided to author by Dr Howard B. Burchell.) (b) Aortic dissection extending into the wall of branches of the aortic arch. (From Spittell J.A. Jr & Wallace R.B. (1980) Dissecting aneurysm of the aorta. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)



Whether the patient with a TIA has a carotid bruit or not, the possibility of an embolic source needs to be kept in mind until significant carotid artery disease has been identified. If the carotid bruit is due to external carotid artery disease, or if internal carotid stenosis is mild, atherosclerosis of the thoracic aorta as a possible source of emboli warrants investigation.27 Carotid duplex ultrasonography28 and MRA provide the necessary information for decision making. Natural history studies have reported a twofold risk of stroke in persons with an asymptomatic pressure significant carotid stenosis compared to those with nonpressure significant stenoses and a sevenfold increased risk for stroke compared to persons without carotid stenosis. Management options for carotid artery stenosis include aspirin (or clopidogrel if the patient is allergic to aspirin), oral anticoagulant therapy, carotid endarterectomy, and percutaneous carotid angioplasty. Clinical factors for considering carotid endarterectomy, or carotid angioplasty, include the clinical reason (pressure significant stenosis, TIA, evolving stroke, or with coronary artery surgery), comorbidity, the risk of future stroke, as well as the surgeon’s or interventionalist’s experience. The latter criterion is well covered in the publication by the AHA Stroke Council.29

24 Chapter 1

Figure 1.19 Arteriogram of a 56-year-old woman with ergotism from excessive use of ergotcontaining suppositories for control of migraine headaches. Note the typical spasm of the distal popliteal artery, the cause of her acute limb ischemia. (From Fulton R.E., Stanson A.W., Forbes G.S., et al. (1980) Vascular imaging. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)

Don’t

Delay Elevate Heat Cool

Table 1.6 Acute arterial occlusion

Percutaneous carotid artery stenting can be performed with an acceptable 30-day complication rate30 and is a reasonable alternative to carotid endarterectomy in high-risk patients (e.g. patients undergoing combined or staged coronary and carotid intervention), those with prior carotid endarterectomy, and those with contralateral carotid artery occlusion.31


Figure 1.20 Cholesterol emboli in the retinal arterioles of a patient with atherosclerotic disease of the ipsilateral carotid artery.

Table 1.7 Symptoms and signs of transient ischemic attacks of the carotid arterial system of the vertebral-basilar arterial system Symptoms and signs Symptoms (attacks of) Weakness Numbness Aphasia Loss of vision Diplopia Other Physical signs (inconstant) Diminished pulsation Change of pressure in ophthalmic artery Retinal emboli Bruits

Carotid system

Vertebral-basilar system

One side of face or limbs One side, usually limbs If dominant hemisphere is involved In one eye on side of ischemia (amaurosis fugax) No —

Limbs in any combination One or both sides of body No Homonymous or bilateral hemianopsia Yes Dysarthria, dysphagia, vertigo, ataxia of gait or limbs

In involved carotid artery Decreased on involved side

In subclavian artery —

Retinal arterioles on involved side Over involved carotid artery bifurcation or over globe of eye on involved side, occasionally over opposite carotid artery

No Over subclavian artery or back of neck

Reproduced from Siekert R.G., Whisnant J.P. & Sundt T.M. Jr (1980) Ischemic cerebrovascular disease. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.

26 Chapter 1

Figure 1.21 Thoracic aortograms demonstrating buckling of (a) the right carotid artery and of (b) the innominate artery. (From Spittell J.A. Jr & Wallace R.B. (1980) Aneurysms. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)


The elongation and tortuosity of the aortic arch and its branches in older persons, particularly those with hypertension, may result in ‘‘buckling’’ of the carotid (Figure 1.21a) or innominate (Figure 1.21b) arteries. The presentation may be a pulsatile mass in the right cervical region; a useful maneuver to differentiate ‘‘buckling’’ from carotid artery aneurysm is to have the patient inspire and hold the breath; with ‘‘buckling’’ the prominent pulsation diminishes or even disappears while with carotid artery aneurysm it does not.

Renal artery stenosis Atherosclerotic renal artery stenosis32 is often present in persons with coronary and peripheral artery atherosclerotic disease. Clues to the possible existence of significant renal artery stenosis include the onset of diastolic hypertension after age 55, malignant hypertension, hypertension resistant to therapy, episodes of ‘‘flash’’ pulmonary edema, unexplained azotemia, and development of azotemia during ACE inhibitor therapy. Likewise, the presence of an epigastric and/ or flank bruit that extends into diastole (Figure 1.1) is a valuable clue to significant renal artery stenosis. When suspected, noninvasive diagnosis can be confirmed by renal artery duplex ultrasonography and MRA. Since progression of stenosis33 in patients with renal artery stenosis, though variable, can be rapid, close serial observation is warranted. The second most common cause of renal artery stenosis is fibromuscular dysplasia (FMD) which occurs at a younger age than ASO and affects women much more often than men. An appropriate algorithm for the diagnosis of FMD of the renal arteries is that suggested by Turi and Jaff 34 ‘‘a high clinical index of suspicion; a potentially abnormal renal artery duplex ultrasound, CT angiogram or MRA study and a confirmatory contrast arteriogram’’. Situations in which revascularization of the renal arteries (by surgical or percutaneous angioplasty techniques) with a 75% or greater stenosis that warrant consideration include severe hypertension resistant to antihypertensive medication, recurring ‘‘flash’’ pulmonary edema (particularly in a patient with normal left ventricular function), dialysis-dependent renal failure, and moderate chronic renal insufficiency. Reliable ways to identify those patients in whom revascularization of renal artery stenosis may not be of benefit have been lacking, though a prospective study of the renal resistance – index value determined by renal ultrasonography – has reported that a value of 80 will identify those who will not benefit.35

References 1 Burek K.A., Sutton-Tyrell K., Brooks M.M., et al. (1999) Prognostic importance of lower extremity arterial disease in patients undergoing coronary revascularization in the bypass angioplasty revascularization investigation (BARI). JACC 34: 716–721. 2 Hertzer N.R., Benen E.G., Young J.R., et al. (1984) Coronary artery disease in peripheral vascular patients. Ann Surg 199: 223–233.

28 Chapter 1 3 Criqui M.H., Fronlek A., Klauber M.R., et al. (1985) The sensitivity, specificity and predictive value of traditional clinical evaluation of peripheral arterial disease: Results from noninvasive testing in a defined population. Circulation 71: 516–522. 4 Barnhorst D.A. & Barner H.B. (1968) Prevalence of congenitally absent pedal pulses. N Engl J Med 278: 264–265. 5 McPhail I.R., Spittell P.C., Weston S.A., et al. (2001) Intermittent claudication: An officebased assessment. JACC 37: 1381–1385. 6 Patel A. & Toole J.F. (1965) Subclavian steal syndrome-reversal of cephalic blood flow. Medicine (Baltimore) 44: 289–303. 7 Valentine R.J., Frye R.E., Whelan K.R., et al. (1987) Coronary-subclavian steal from reversed flow in an internal mammary artery used for coronary bypass. Am J Cardiol 59: 719–720. 8 Allen E.V. (1929) Thromboangiitis obliterans: Methods of diagnosis of chronic occlusive arterial lesions distal to the wrist with illustrative cases. Am J Med Sci 78: 237–244. 9 Conklin L.D., Ferguson E.R. & Reardon M.J. (2001) The technical aspects of radial artery harvesting. Tex Heart Inst J 28: 129–131. 10 McDaniel M.D. & Cronenwett J.L. (1989) Basic data related to the natural history of intermittent claudication. Ann Vasc Surg 3: 273–277. 11 Rosenbloom M.S., Flanigan P., Schuler J.L., et al. (1988) Risk factors affecting the natural history of intermittent claudication. Arch Surg 123: 867–870. 12 Juergens J.L., Barker N.W. & Hines E.A. Jr (1960) Arteriosclerosis obliterans: Review of 520 cases with special reference to pathogenic and prognostic factors. Circulation 21: 188–195. 13 Schadt D.C., Hines E.A. Jr, Juergens J.L., et al. (1961) Chronic atherosclerotic occlusion of the femoral artery. JAMA 175: 937–940. 14 Coffman J.D. (1986) Intermittent claudication: Not so benign. Am Heart J 112: 1127–1128. 15 Caprie Steering Committee (1996) A randomized, blinded trial of clopidogrel versus aspirin in patients at risk of ischemic events (CAPRIE). Lancet 348: 1329–1339. 16 Weis A.J. & Fairbairn J.F. II (1968) Trauma, ischemic limbs and amputation. Postgrad Med 43: 111–115. 17 Cronenwett J.L., Warner K.G., Zelenock G.B., et al. (1984) Intermittent claudication: Current results of monoperative management. Arch Surg 119: 430–436. 18 Coffman J.D. (1979) Drug therapy: Vasodilator drugs in peripheral vascular disease. N Engl J Med 300: 713–717. 19 Lindgaarde F., Jelmes R., Bjorkman H., et al. (1989) Conservative drug treatment in patients with moderately severe chronic occlusive peripheral arterial disease. Circulation 80: 1549–1556. 20 Dawson D.L., Cutler B.S., Hiatt W.R., et al. (2000) A comparison of cilastazol and pentoxifylline for treating intermittent claudication. Am J Med 109: 523–530. 21 Montori V.M., Kavros S.J., Walsh E.E., et al. (2002) Intermittent compression pump for nonhealing wounds in patients with limb ischemia. The Mayo Clinic experience (1998– 2000). Int Angiol 21: 360–366. 22 Pantely G.A. & Porter J.M. (2000) Therapeutic angiogenesis: Time for the next phase. JACC 36: 1245–1247. 23 Spittell J.A. Jr (1994) Peripheral arterial disease. Disease-A-Month 40: 677–680. 24 Tak-Sun Tse, McBane R.D., Stanson A.W., et al. (2002) Secular trends and long-term survival in thromboangiitis obliterans. JACC 39 (Suppl A): 265A. 25 Rice L., Attisha W.K., Drexler A., et al. (2002) Delayed onset heparin-induced thrombocytopenia. Ann Int Med 136: 210–215.

Occlusive peripheral arterial disease 29 26 Ouriel K., Shortell C.K., DeWeese J.A., et al. (1994) A comparison of thrombolytic therapy with operative revascularization in the initial treatment of acute peripheral arterial ischemia. J Vasc Surg 19: 1021–1030. 27 Tunick P.A. & Kronzon I. (2000) Atheromas of the thoracic aorta: Clinical and therapeutic update. JACC 35: 545–554. 28 Howma S. & Di Tullo M. (2001) Cardiac sources of embolus: How to find it. ACC Curr J Rev 10: 45–48. 29 Ad Hoc Committee, American Heart Association (1995) Guidelines for carotid endarterectomy. Stroke 26: 188–201. 30 Roubin G.S., New G., Iyer S.S., et al. (2001) Immediate and late outcomes of carotid artery stenting in patients with symptomatic and asymptomatic carotid artery stenosis. Circulation 103: 532–537. 31 Shawl E., Kadro W., Domanski M.J., et al. (2000) Safety and efficacy of elective carotid artery stenting in high-risk patients. J Am Coll Cardiol 35: 1721–1728. 32 Vashest A., Heller E.N., Brown E.J. Jr, et al. (2002) Renal artery stenosis: A cardiovascular perspective. Am Heart J 143: 559–564. 33 Caps M.T., Perissinotto C., Zierler E., et al. (1998) Prospective study of atherosclerotic disease progression in the renal artery. Circulation 98: 2866–2872. 34 Turi Z.G., Jaff M.R. (2003) Renal artery stenosis: searching for algorithms for diagnosis and treatment. JACC 41: 1312–1315. 35 Radermacher J., Chavan A., Bleck J., et al. (2001) Use of Doppler ultrasonography to predict the outcome of therapy of renal artery stenosis. N Eng J Med 344: 410–417.

CHAPTER 2

Aneurysmal disease

Aneurysms of the aorta and peripheral arteries are almost always atherosclerotic, but some factors such as familial tendency, hypertension, male sex, and smoking are risk factors for aneurysm development, especially in the aorta. The similarity of the risk factors for aneurysmal disease and coronary artery disease no doubt accounts for their frequent comorbidity1 and emphasizes the need for cardiologists to be informed about aneurysmal disease and its management. It is interesting, and of some clinical importance, that atherosclerotic aneurysms tend to form where an artery is not supported by skeletal muscle or where the artery is subjected to frequent bending. Thus, the common locations of atherosclerotic aneurysms are the abdominal aorta, the femoral and popliteal arteries, the iliac arteries, and the thoracic aorta in about that order of frequency. Also for the cardiologist who performs echocardiograms, it is well to keep the risk factors for aneurysm in mind when studying patients over age 50 and taking a few minutes to screen the abdomen for possible abdominal aortic aneurysm.2 Screening for abdominal aortic aneurysm during transthoracic echocardiography is particularly desirable in persons over age 50 with a family history of abdominal aortic aneurysm.3 Progressive enlargement of aneurysms leads to the complications of pressure on surrounding structures or to rupture. The other complications of aneurysms are thromboembolic (from the laminated thrombus within the aneurysm (Figure 2.1)) and rarely infection. The frequency of different complications varies with different aneurysms and will be covered with each aneurysm. In addition to atherosclerosis, another important etiology of aneurysm in cardiovascular practice is the frequent puncture of the femoral artery for diagnostic angiograms and interventional procedures that may result in a pseudoaneurysm of the femoral artery (Figure 2.2). Ultrasound-guided compression is the initial therapeutic procedure of choice, but when it is not successful, surgical repair is indicated. A number of other therapeutic approaches have been proposed, the most recent being percutaneous injection of biodegradable collagen.4

Thoracic aortic aneurysm Of the two types of thoracic aortic aneurysm – atherosclerotic and posttraumatic – those due to atherosclerosis are by far the most common. There appear to be familial aggregations of thoracic aortic aneurysms and in such cases the aneurysms tend to occur at a younger age.5 These aneurysms are usually asymptomatic and are often discovered on chest roentgenograms. 30

Aneurysmal disease 31

Figure 2.1 Arteriosclerotic aneurysm of abdominal aorta containing laminated thrombus thick enough to produce a lumen of normal caliber. Note also point of rupture of proximal anterior wall of aneurysm. (Reproduced from Spittell J.A. Jr (1971) Aortic and peripheral arterial aneurysms. In Gifford R. (ed.) Peripheral Vascular Disease. Cardiovasc Clin 3: 113–126. By permission of F.A. Davis Co, Philadelphia.)

However, when they are large, thoracic aortic aneurysms can cause chest pain or symptoms due to pressure on other structures in the mediastinum, e.g. dyspnea, dysphagia, hoarseness, or venous obstruction (Figure 2.3). The prognosis for untreated thoracic aortic aneurysm is poor due to rupture, especially in the hypertensive patient.6 Surgical resection is indicated when thoracic aortic aneurysms are causing symptoms, when they are 6 cm in diameter, or when enlarging (more than 1 cm per year) under serial observation. In patients with significant hypertension, serious consideration of surgical treatment, even in smaller thoracic aortic aneurysms, is warranted because of the frequency of rupture. A similar approach is appropriate in patients with Marfan syndrome where some have lowered the threshold to consider surgery for ascending aortic aneurysms to 4.5 cm in diameter. In persons subjected to blunt chest trauma and/or sudden deceleration, incomplete laceration of the thoracic aorta, usually at the isthmus, can result in an aneurysm (Figure 2.4, p. 35). Recognition of traumatic laceration of the

32 Chapter 2

Figure 2.2 (a) Longitudinal ultrasound scan of left femoral artery shows subcutaneous axillofemoral graft. Graft is surrounded anteriorly by pseudoaneurysm (false aneurysm). (b) Transverse ultrasound scan shows graft and pseudoaneurysm (false aneurysm). (From Fulton R.E., Stanson A.W., Forbes G.S., et al. (1980) Vascular imaging. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)

aorta in the acute setting requires a high index of suspicion (Table 2.1, p.36), prompt and appropriate evaluation to confirm the diagnosis, and urgent treatment7 because of the high rate of rupture in the first 3 weeks after injury, unless surgical treatment of other serious injury takes precedence. Traumatic pseudoaneurysm of the thoracic aorta may develop and when discovered later also warrants consideration of surgical resection, or endoluminal stent grafting, because many pseudoaneurysms are unpredictably unstable at some time in their course.8


Figure 2.3 Aneurysm of thoracic aorta in a 65-year-old woman. (a) Unilateral (left) jugular vein distention caused by compression of the left innominate vein by the aneurysm. (b) Chest roentgenogram showing the large aneurysm of the descending thoracic aorta. (c) Computed tomography with contrast enhancement demonstrating the aneurysm of the descending thoracic aorta. (Reprinted from Spittell J.A. Jr & Spittell P.C. (1991) Aneurysms. In Young G.R., Graor R.A., Olin G.O., et al. (eds) Peripheral Vascular Diseases. Copyright. By permission of Elsevier Science.)

34 Chapter 2


Abdominal aortic aneurysm Abdominal aortic aneurysms, common comorbid conditions in patients with coronary disease, are increasing in incidence. They present both clinical challenges and an important therapeutic opportunity. As such, the clinical features, new information on the natural history, and current management warrant discussion. About half of abdominal aortic aneurysms are asymptomatic so that recognition in the absence of symptoms, requires careful abdominal examination, keeping in mind the admonition of Osler9 in 1905: a mistake is not likely to occur if it is remembered that no pulsation, however forcible, no thrill, however intense, no bruit, however loud – singly or together, justify the diagnosis of an aneurysm of the abdominal aorta, only the presence of a palpable, expansile tumor.

Unless a patient is too obese, a diagnosis of abdominal aortic aneurysm can usually be made by careful physical examination. Tumors with transmitted pulsation and a tortuous atherosclerotic aorta (usually in elderly women) can mimic an abdominal aortic aneurysm on physical examination but can be differentiated readily by abdominal ultrasonography (Figure 2.5) which has nearly 100% sensitivity in detecting abdominal aortic aneurysm. Since virtually all abdominal aortic aneurysms are atherosclerotic in origin, and about half are asymptomatic, cardiologists performing transthoracic echocardiograms on persons (particularly men) over age 50 with risk factors for atherosclerosis would find it worthwhile to screen the abdomen to evaluate the abdominal aorta, as noted earlier.2,3 While serial ultrasound examination is valuable to follow small abdominal aortic aneurysms (the vast majority enlarge slowly at a rate of 0.2–0.3 cm per year10), CT scan (Figure 2.5), MRI, or aortography to provide anatomical


Figure 2.4 (a) Chest film of a 12-year-old girl involved in an auto accident 12 days earlier; mediastinum is widened and aortic arch is prominent. (b) Thoracic aortogram shows a false aneurysm at the site of the aortic laceration. (From Fulton R.E., Stanson A.W., Forbes G.S., et al. (1980) Vascular imaging. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)

36 Chapter 2

Decelerating injury Thrown from a vehicle Chest trauma Chest X-ray . Mediastinal widening . Left hemothorax Hypertension

Table 2.1 Clues to incomplete traumatic thoracic aortic rupture

Figure 2.5 (a) Transverse ultrasound scan of an abdominal aortic aneurysm extending to the left of the lumbar spine. (b) CT scan of the same abdominal aortic aneurysm at the same level as the ultrasound scan in (a). The dimensions of the aneurysm measured on the ultrasound scan and the CT scan differed by 2 mm. (From Fulton R.E., Stanson A.W., Forbes G.S., et al. (1980) Vascular imaging. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)

information on the renal, visceral, and iliac vessels, as well as the proximal and distal extent of the aneurysm, are needed if surgery or transfemoral intraluminal graft implantation is planned.11,12 Rupture, the most frequent complication of abdominal aortic aneurysm, may account for an overall mortality rate (prehospital deaths and surgical


mortality) of 90% or more.13 Rupture of aneurysms smaller than 4 cm in diameter is rare, but the risk of rupture becomes significant at 5 cm in diameter.10 The rupture rate of abdominal aortic aneurysms increases with diameter. In a study of large abdominal aortic aneurysms in patients refusing or unfit for elective surgical repair, the probable 1-year rupture rate increased from 9.4% for aneurysms 5.5–5.9 cm in diameter to 32.5% for aneurysms larger than 7 cm in diameter.14 Rupture most often is into the retroperitoneal, or intra-abdominal space, but rarely an aneurysm may rupture into the duodenum and present as gastrointestinal bleeding or into the inferior vena cava and present with protean clinical features due to the high flow into the vena cava. These protean features include venous hypertension in the lower extremities, heart failure, and/or renal failure – in addition to the local manifestations, such as abdominal pain, pulsatile abdominal mass, and a continuous multiple pitch bruit.15 Because surgical treatment of ruptured abdominal aortic aneurysm has a tenfold greater mortality rate than elective resection and graft placement, elective repair of symptomatic and asymptomatic aneurysms more than 5.5 cm in diameter is recommended for good-risk patients to avoid the complication of rupture. Advising the patient with an abdominal aortic aneurysm smaller than 5.5 cm in diameter is now somewhat controversial because of the recent reports of surveillance studies of patients with aneurysms smaller than 5.5 cm in diameter;16,17 these studies showed no improvement in the 5-year survival of the elective repair group compared to the surveillance group. In one of the studies, 61% of the patients in the surveillance group had an elective repair of their aneurysm because it had expanded to 5.5 cm or larger in the course of the study.16 Furthermore, in both studies aneurysm rupture occurred in the surveillance group at a rate of 0.6% per year in one study16 and 3% of patients randomized to surveillance died of ruptured abdominal aortic aneurysms in the other study.17 Considering the tenfold increased mortality for surgical treatment of ruptured abdominal aortic aneurysms compared to the mortality for elective repair, clinicians need to counsel their patients with abdominal aortic aneurysms smaller than 5.5 cm in diameter on an individual basis. Factors of patient preference and compliance, after being fully informed of the options, requirements, and risks of surveillance versus early surgical repair, should enter into the management decisions in good-risk patients. In the poor-risk patient, endovascular placement of a stented graft is an alternative to surgical treatment,11,12 but the need for lifelong surveillance and the uncertainty of clinical outcome of endovascular repair makes it less desirable than standard open surgical repair for the good-risk patient.18 Complications of abdominal aortic aneurysms other than rupture, though uncommon, produce clinically important syndromes. One is atheroembolism (Figure 2.6) from the laminated thrombus within the aneurysm (Figure 2.1).19 In addition to the livedo reticularis and blue toes, renal insufficiency and hypertension are a part of the clinical picture due to microembolism of the kidneys. Some refer to atheroembolism as the ‘‘blue toe syndrome,’’ but

38 Chapter 2

Figure 2.6 Atheroembolism from thrombus in an abdominal aortic aneurysm. (a) Livedo reticularis in skin over knees and multiple blue toes. (Reproduced from Spittell J.A. Jr (1980) Occlusive arterial disease. Cardiovasc Clin 10: 289–300. By permission of F.A. Davis Co, Philadelphia.) (b) Photomicrograph of biopsy showing evidence of cholesterol embolization (multiple clefts) of a dermal arteriole in a biopsy from an area of livedo reticularis. (Reproduced from Spittell J.A. Jr (1980) Clinical aspects of aneurysmal disease. Curr Prob Cardiol 5: 3–36. By permission of Mosby, Inc.)

Aneurysmal disease 39 Table 2.2 Causes of blue toe syndrome Thrombotic arterial occlusion Arteriosclerosis obliterans Thromboangiitis obliterans Hypercoagulable disorders Connective tissue disorders Trauma Embolic arterial occlusion Cardiac origin Atheroembolism from . Shaggy aorta . Abdominal aortic aneurysm . Femoropopliteal aneurysm Vasospastic disorders Raynaud’s phenomenon . Primary . Secondary Acrocyanosis Chronic pernio Cyanotic congenital heart disease Drug-induced Anticoagulant therapy (atheroembolism) Prednisone therapy of antiphospholipid antibody Modified from Spittell J.A. Jr & Spittell P.C. (1992) Chronic pernio: Another cause of blue toes. Int Angiol 11: 46–50.

it is only one cause of blue toes. A broader concept of the blue toe syndrome is more practical clinically because of a number of other important conditions that can present with blue toes, some even with associated livedo reticularis as well (Table 2.2). Attention to the kidneys, in addition to the cardiac status, of a person with an abdominal aortic aneurysm is important for several reasons besides atheroembolism. A rare renal complication of abdominal aortic aneurysm is hydronephrosis which causes impaired renal function, even anuria, due to pressure on the ureters by the aneurysm (Figure 2.7). Renal anomalies, particularly horseshoe kidney, take on special importance in the person with an abdominal aortic aneurysm since anomalous renal arteries and the symphysis of the horseshoe kidney warrant preoperative selective renal arteriography and special attention at the time of aneurysm surgery.20 Renal arteriography may also be indicated in the person with an abdominal aortic aneurysm and associated hypertension and bruits in the upper abdomen suggestive of renovascular disease that could be corrected at the time of aneurysm repair. An uncommon but important type of abdominal aortic aneurysm is the inflammatory type that occurs in 2–5% of patients. The clinical manifestations are abdominal and/or back pain, weight loss, and an elevated sedimentation rate. The perianeurysmal inflammatory process produces a characteristic halo

40 Chapter 2

Figure 2.7 Anteroposterior roentgenogram of the abdomen of a 69-year-old man, who was anuric for 1 day, shows displacement of ureteral catheters by what, at surgery, proved to be an 8 cm abdominal aortic aneurysm causing the ureteral obstruction. (Reproduced from Spittell J.A. Jr (1971) Aortic and peripheral arterial aneurysms. In Gifford R. (ed.) Peripheral Vascular Disease. Cardiovasc Clin 3: 113–126. By permission of F.A. Davis Co, Philadelphia.)

effect on imaging studies (Figure 2.8) and if extensive may involve the ureters. Treatment is surgical repair.21 When cardiologists are involved in the care of patients with infective endocarditis, peripheral arterial infections (termed mycotic aneurysms by Osler in 188522) need to be kept in mind. There have been, however, significant changes in the terminology, risk factors, and bacteriology of peripheral arterial and aortic infections. As far as terminology goes, most now use the terms infected aneurysm or infective endarteritis or infective aortitis to describe peripheral arterial infections. The role of infective endocarditis as a cause is now diminished and the risk factors now are arterial trauma (drug use and iatrogenic procedures), concurrent sepsis, and depressed immunocompetence (Figure 2.9). While streptococci and enterococci were the main infecting organisms in arterial infections in the preantibiotic era, now the major infecting organisms are staphylococci and salmonella, though there are recent reports of other organisms, chiefly pneumococci and campylobacter.23,24 Salmonella


Figure 2.8 Inflammatory abdominal aortic aneurysm. MRI of midabdomen of a 69-year-old man with a 4-month history of abdominal pain and an 8-pound weight loss. Sedimentation rate was 65 mm/hr. Note the typical perianeurysmal ‘‘halo’’ effect produced by the inflammatory process.

Figure 2.9 AP view of an aortogram showing a saccular (‘‘mycotic’’) abdominal aortic aneurysm in a 64-year-old woman with multiple myeloma who presented with a 1-month history of fever, abdominal pain, and blood cultures positive for S. pneumoniae. After treatment with penicillin the aneurysm, whose anterior wall was destroyed by infection, was resected and replaced by a dacron graft.

42 Chapter 2

organisms have a predilection for atherosclerotic and aneurysmal arteries; so they are the most common infecting organisms in aortic infections and they have a high risk of causing rupture.25 Thus, a high index of suspicion of aortic and arterial infections is essential in older persons, those with depressed immunocompetence, and drug users who have unexplained fever, if the diagnosis and successful treatment are to be timely. For diagnosis, CT scan and MRI are excellent. Treatment includes therapy with appropriate antibiotics and then surgical resection of the infected tissue, restoration of flow to the lower extremities if the aorta is resected, followed by antibiotic therapy for at least 6 weeks – longer in immunocompromised patients.

Iliac artery aneurysm Aneurysms of the common iliac artery usually are continuous or associated with abdominal aortic aneurysm but occasionally are solitary. Like abdominal aortic aneurysms, they occur most often in men more than 60 years of age. Since an aneurysm of the common iliac artery is not likely to be felt on physical examination, unusual symptoms of unexplained pain in the groin, unexplained obstruction of an iliac vein, or unexplained obstructive uropathy are the clues to the possibility of an iliac artery aneurysm. CT scan with contrast, or MRA, (Figure 2.10) can confirm the diagnosis.

Figure 2.10 Iliac artery aneurysms and occlusion of left common iliac artery on MRA. (From Spittell J.A. Jr (1998) Peripheral vascular disease. In Wenger N.K. (ed.) Cardiovascular Disease: Recognition and Management in the Octogenarian and Beyond. London: Martin Dunitz Ltd. By permission of Martin Dunitz Ltd.)


Figure 2.11 (a) Gangrene of the left foot as a result of thrombosis of a popliteal aneurysm. (From Spittell J.A. Jr (1980) Clinical aspects of aneurysmal disease. Curr Prob Cardiol 5: 3–36. By permission of Mosby, Inc.) (b) Thrombosed popliteal artery aneurysm; thrombus extends into the trifurcation. (From Spittell J.A. Jr & Wallace R.B. (1980) Aneurysms. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)

Symptomatic iliac artery aneurysms and those 2 cm or larger warrant surgical treatment.

Femoral and popliteal artery aneurysm Almost always seen in older men, these aneurysms, whose major complications are thromboembolic with a high risk of limb loss (Figure 2.11), may be overlooked unless the femoral artery in the groin, the femoropopliteal artery in the lower medial thigh (Figure 2.12), and the popliteal pulse just below the level of the knee joint are carefully examined. In persons who have had aortofemoral grafts placed, false aneurysm may develop at the distal anastomosis (Figure 2.2). In the groin and in the popliteal space, an enlarging aneurysm can obstruct the adjacent vein and cause edema of the leg (Figure 2.13). Popliteal aneurysms incidentally are bilateral more than half the time and are associated with aneurysms of other arteries in more than 40% of cases –

44 Chapter 2

Femur

Adductor magnus hiatus Proximal popliteal aneurysm Lat. head gastrocnemius Level of arcuate popliteal ligament Soleus Distal popliteal aneurysm

Figure 2.12 Photograph of popliteal fossa in dissected amputated limb of patient having proximal and distal popliteal aneurysms. (From Gedge S.W., Spittell J.A., Jr, Irvine, J.C., et al. (1961) Aneurysm of the digital popliteal artery and its relationship to the arcuate popliteal ligament. Circulation 24: 270–273. By permission of the American Heart Association.)

Figure 2.13 Edema and increased venous pattern of the right leg and thigh of a 70-yearold man with a common femoral artery aneurysm compressing the femoral vein (infrared photograph). The venous compression was relieved by resection of the aneurysm. Arterial continuity was restored with a prosthetic graft. (From Spittell J.A. Jr (1980) Clinical aspects of aneurysmal disease. Curr Prob Cardiol 5: 3–36. By permission of Mosby, Inc.)


Figure 2.14 (a) Longitudinal ultrasound scan of the left knee shows a popliteal aneurysm at about the level of the knee joint. (b) Left femoral arteriogram of same patient demonstrating the popliteal artery aneurysm just above the level of the knee joint. (From Spittell J.A. Jr (1980) Clinical aspects of aneurysmal disease. Curr Prob Cardiol 5: 3–36. By permission of Mosby, Inc.)

another reason to carefully examine the femoropopliteal and popliteal arteries. The diagnosis of femoral and popliteal aneurysm is readily made by ultrasound (Figure 2.14).

46 Chapter 2

Since these aneurysms, untreated, frequently lead to serious complications (distal embolization, acute arterial occlusion, and pressure on the adjacent vein or nerve), elective resection and restoration of arterial continuity prior to the development of complications present the best results.26

Upper extremity arterial aneurysm These aneurysms are far less common than lower extremity arterial aneurysms. Blunt, or penetrating trauma, in the arteries of the arm and hand, and compression of the subclavian artery (between the uppermost rib and the clavicle; if a cervical rib is present it can often be felt as a fullness in the supraclavicular area) in the thoracic outlet in addition to atherosclerosis may result in aneurysm formation. Complications are usually thromboembolic, often in the hand (Figure 2.15). When there is a thrombus overlying the compressed area of the subclavian artery (the source of the emboli to the hand), the damaged portion of the subclavian artery needs to be resected and the artery repaired.

Figure 2.15 (a) Painful ulcerations of left index and long fingers (present for 6 weeks) of a 55-year-old woman. On examination there was a palpable fullness in the left supraclavicular area, a positive Allen’s test in the left hand, and positive costoclavicular and hyperabduction thoracic outlet maneuvers on the left. (Reproduced from Spittell J.A. Jr (1994) Peripheral arterial disease. Disease-a-Month 40: 641–704. By permission of Mosby, Inc.) (b) X-ray of the cervical spine showing cervical ribs of patient in (a). (c) Arch aortogram of patient in (a) showing poststenotic dilatation of the left subclavian artery just beyond its compression by the uppermost ribs in the thoracic outlet. (d) Arteriogram of the left arm and hand of patient in (a) showing occlusion of several digital arteries. (e) Photograph of the compressed portion of the left subclavian artery with thrombus (the source of the emboli to the fingers of the left hand) overlying the injured area of the artery. The above was resected and the subclavian artery repaired after the left cervical and first ribs were resected and a left dorsal sympathectomy performed.



References 1 Hertzer N.R., Beven E.G., Young J.R., et al. (1984) Coronary artery disease in peripheral vascular patients: A classification of 1000 coronary angiograms and results of surgical management. Ann Surg 199: 223–233.

48 Chapter 2 2 Spittell P.C., Ehrsam J.E., Anderson L., et al. (1997) Screening for abdominal aortic aneurysm during transthoracic echocardiography in a hypertensive patient population. J Am Soc Echocardiogr 10: 722–727. 3 Davies M.J. (1998) Aortic aneurysm formation: Lesson from human studies and experimental models. Circulation 98: 193–195. 4 Hamraoni K., Sjef M., Ernst P.G., et al. (2002) Efficacy and safety of percutaneous treatment of iatrogenic femoral artery pseudoaneurysm by biodegradable collagen injection. JACC 39: 1297–1304. 5 Albomoz G.F., Tang P.C., Coady M.A., et al. (2003) Are thoracic aortic aneurysms a genetic disease?. JACC 41 (Suppl. A): 287A. 6 Bickerstaff L.K. (1982) Thoracic aortic aneurysms: A population-based study. Surgery 92: 1103–1108. 7 Merrill W.A., Lee R.B., Hamman J.W., et al. (1988) Surgical treatment of acute traumatic tear of the thoracic aorta. Ann Surg 207: 699–706. 8 Rousseau H., Soula P., Perrault P., et al. (1999) Delayed treatment of traumatic rupture of the thoracic aorta with endoluminal covered stent. Circulation 99: 498–504. 9 Osler W. (1905) Aneurysm of the abdominal aorta. Lancet 2: 1089–1096. 10 Nevitt M.P., Ballard D.J. & Hallett J.W. Jr (1989) Prognosis of abdominal aortic aneurysms. N Engl J Med 321: 1009–1014. 11 Parodi J.C., Palmay J.C. & Barone H.D. (1991) Transfemoral intraluminal graft implantation for abdominal aortic aneurysms. Ann Vasc Surg 5: 491–499. 12 Howell M.H., Strickman N., Mortazavi A., et al. (2001) Preliminary results of endovascular abdominal aortic aneurysm exclusion with the Aneurex-Stent Graft. JACC 38: 1040–1046. 13 Ernst C.B. (1993) Abdominal aortic aneurysm. N Engl J Med 328: 1167–1172. 14 Lederle F.A., Johnson G.R., Wilson S.E., et al. (2002) Rupture rate of large abdominal aortic aneurysms in patients refusing or unfit for elective repair. JAMA 287: 2968–2972. 15 Petetin L., Pelouze G.A., Mercier V., et al. (1987) Rupture of the abdominal aorta into the inferior vena cava: A study of seven cases. Ann Vasc Surg 1: 572–577. 16 Lederle F.A., Wilson S.E., Johnson G.R., et al. (2002) Immediate repair compared with surveillance of small abdominal aortic aneurysms. N Engl J Med 346: 1437–1444. 17 The UK Small Aneurysm Trial Participants (1998) Mortality results for randomized control trial of early elective surgery or ultrasonographic surveillance for small abdominal aortic aneurysms. Lancet 352: 1649–1655. 18 Chaikof E.L., Brinkman W.T., Dodson T.F., et al. (2002) Endovascular repair of abdominal aortic aneurysms: Risk stratified outcomes. Ann Surg 235: 833–841. 19 Kazmier F. Jr, Sheps S.G., Bernatz P.E., et al. (1966) Livedo reticularis and digital infarcts: A syndrome due to cholesterol emboli arising from atheromatous aortic aneurysms. Vasc Dis 3: 12–21. 20 Sidell P.M., Pairolero P.C., Payne W.S., et al. (1979) Horseshoe kidney associated with surgery of the abdominal aorta. Mayo Clin Proc 54: 97–103. 21 Nitechi S.S, Hallett J.W. Jr, Stanson A.W., et al. (1996) Inflammatory abdominal aortic aneurysms: A case-control study. J Vasc Surg 23: 860–868. 22 Osler W. (1885) The Gulstonian lectures on malignant endocarditis. Brit Med J 1: 467–470; 522–526; 577–579. 23 Gomes M.N., Choyke P.L. & Wallace R.B. (1992) Infected aortic aneurysms: A changing entity. Ann Surg 215: 435–442.

Aneurysmal disease 49 24 Bronze M.S., Shirwany A., Corbett C., et al. (1999) Infectious aortitis: An uncommon manifestation of infection with streptococcus pneumoniae. Am J Med 107: 627–630. 25 Katz S.G., Andros G. & Kohl R.D. (1992) Salmonella infections of the abdominal aorta. Surg Gyn Obst 175: 102–106. 26 Wychulis A.R., Spittell J.A. Jr & Wallace R.B. (1970) Popliteal aneurysms. Surgery 68: 942–951.

CHAPTER 3

Aortic dissection, penetrating atherosclerotic aortic ulcer, and intramural hematoma

Acute aortic dissection is an important condition for the cardiologist to keep in mind since it is one of the life-threatening disorders that presents with acute chest pain mimicking acute myocardial infarction. Despite its high early mortality (about 1% per hour in the first 48 hours), acute aortic dissection can be readily confirmed, if suspected, by one of several imaging procedures and then appropriately managed (Figure 3.1) when acute myocardial infarction and other causes of acute chest pain have been excluded. In regard to the above, awareness of the risk factors for acute aortic dissection – hypertension (70% of cases), the Marfan syndrome, and congenital aortic valve anomalies1 – and its occurrence twice as often in men as in women is helpful clinically. Other conditions which predispose patients of younger age to acute aortic dissection include coarctation of the aorta, Type IV Ehler–Danlos syndrome, Turner syndrome, cocaine abuse, and weight lifting.2 There are, in addition, reports of familial aortic dissection3 as well as a familial association of iris flocculi with aortic dissection,4 and a recently described familial association of aortic dissection with patent ductus arteriosus.5 In addition to the sudden chest pain, there are signs and symptoms of ischemia of various organ systems, such as the brain (stroke, syncope), the spinal cord (paraplegia), a kidney (acute hypertension), an extremity (acute peripheral arterial occlusion), due to involvement of the origin of the branches of the aorta by the dissecting column of blood (Figure 3.2). Also included are signs of cardiac disease, e.g. cardiac murmurs, pericardial friction rub, and cardiac tamponade (also a cause of syncope). Thus acute myocardial infarction, cerebrovascular accident, acute abdominal conditions, and acute peripheral arterial occlusion may be not only the initial clinical impression but an erroneous diagnosis in acute aortic dissection.6 The possibility that the dissecting column of blood may have narrowed or occluded one of the subclavian arteries and resulted in a lowered blood pressure in that arm emphasizes the need to determine the blood pressure in both arms lest existing hypertension be missed. Hypertension is not only the leading risk factor for aortic dissection but a clue to the diagnosis in younger persons. A recent review of acute Type A aortic dissection in persons more than 70 years of age found that in hospital hypotension occurred more frequently in 50


51

Chest pain History, physical exam CXR, ECG, cardiac enzymes Aortic dissection suspected Initiate beta-blocker Hemodynamically stable No TTE/TEE

Yes TEE, CT, angio, MRI Aortic dissection

Type III

Type I, II Surgery

Coronary care unit

Figure 3.1 Management of suspected acute aortic dissection.

Figure 3.2 Dissection into a branch of the aortic arch.

the elderly than in younger patients7– another important clinical point to take into account. Abnormalities in the region of the aortic knob on a chest roentgenogram – increased supracardiac aortic diameter, double density due to superimposed posterior aortic enlargement, and/or deviation of the tracheal shadow to the right – can be most helpful diagnostically, particularly if previous chest

52 Chapter 3

Figure 3.3 (a) Widening of supracardiac aortic shadow in patient with aortic dissection. (b) Same patient 11⁄2 years earlier. (From Spittell J.A. Jr & Wallace R.B. (1970) Aortic dissection. Am Fam Physician 1: 89–98. By permission of American Academy of General Practice.)

roentgenograms are available for comparison (Figure 3.3) or if serial roentgenograms show an increase in the aortic shadow.8 It is important, however, to remember that the chest roentgenogram may be normal in up to 20% of cases and thus a normal chest roentgenogram does not exclude acute aortic dissection. Clinical suspicion of acute aortic dissection should lead to initiation of betablocker therapy and prompt an accurate diagnosis by one or more of the noninvasive imaging modalities – transesophageal echocardiography (Figure 3.4), CT scan (Figure 3.5), or MRI (Figure 3.6) – each of which has advantages and disadvantages.9 Transesophageal echocardiography has become the procedure of choice in many centers because it can be performed rapidly and is portable. Regardless of the imaging procedure selected, in addition to confirmation of the diagnosis, locating the primary intimal tear is essential to plan definitive therapy even though the clinical features (Table 3.1, p. 55) are suggestive of its location. Because of the high incidence of early rupture and death with proximal (Types I and II) aortic dissections (Figure 3.7, p. 55), urgent surgical treatment is indicated as soon as the diagnosis is made. For distal (Type III) dissection, continued pharmacologic therapy (Table 3.2, p. 56) is appropriate for several weeks prior to surgical treatment (in suitable-risk patients). While some favor long-term pharmacologic therapy for distal (Type III) dissections, it appears that long-term survival is better in those treated surgically, particularly in those with hypertension and/or chronic obstructive lung disease whose rate of rupture is high. If there is continued pain, uncontrolled hypertension, or progression of the distal (Type III) dissection despite adequate pharmacologic treatment,


53

Figure 3.4 Echocardiographic images in a Type I aortic dissection. (a) The intimal tear. (b) False (FL) and true (TL) lumens.

more urgent surgical treatment is warranted. Compromise of a major arterial branch of the aorta by the dissection (Figure 3.2) also warrants more immediate treatment surgically or by a percutaneous procedure.10,11 The mention of compromise of a major arterial branch of the aorta brings up the renewed interest in, and utilization of, aortography if the noninvasive imaging does not provide the information needed for planning treatment of these complications. The review of acute Type A (proximal, Types I and II) aortic dissection in the elderly contributed several important points about management in these patients. First, it was noted that ‘‘medications known to reduce the risk and extension of aortic dissection, such as beta-blockers, were used less often in the elderly with Type A dissection.’’7 Second, in this cohort the mortality for those treated surgically was not prohibitive and was better than for those treated medically suggesting that ‘‘an aggressive surgical approach is not

54 Chapter 3

Figure 3.5 CT scan of Type I aortic dissection.

Figure 3.6 MRI of Type III aortic dissection.

Aortic dissection, penetrating atherosclerotic aortic ulcer, and intramural hematoma Table 3.1 Clinical features suggestive of type of aortic dissection

55

Proximal dissection (Type I & II; Type A) Marfan syndrome Coarctation of aorta Initial pain, anterior chest12 Decreased pulse(s), right arm and/or carotid Decreased blood pressure, right arm Syncope Aortic diastolic murmur Pericardial friction rub ECG changes of myocardial ischemia and/or infarction Cardiac tamponade Distal dissection (Type III; Type B) Initial pain, interscapular area and/or posterior chest12 Hypertension Left pleural effusion

Figure 3.7 Ruptured Type I aortic dissection.

unreasonable in selected elderly patients with acute Type A aortic dissection for improving survival. . . . ’’7 Another advantage of transesophageal echocardiography, and MRI, in acute aortic dissection is detection of pericardial effusion – an indication for surgical treatment of the dissection as soon as possible since this indicates leakage of blood into the pericardium from retrograde dissection of the aortic root and is the most common cause of death in the first 2 weeks. An additional point is whether the tamponade is associated with palpable pulses or not, as when pulses are not palpable there is a 16-fold increased risk of poor outcome, usually preoperative death.13,14 Percutaneous pericardiocentesis for

56 Chapter 3

Hypertensive patient: (Goal is systolic blood pressure in the range of 110 mm Hg (or lowest level maintaining a urine output of 25–30 ml/h) until oral medication can be started.) 1. Sodium nitroprusside IV (25–50 mg/min) but always with one of the following: IV propranolol (1 mg every 4–6 h) IV esmolol (1 mg/kg bolus over 30 sec, followed by 150–300 mg/kg/min) IV metoprolol (5 mg bolus over 2 min 1–3 times; then 50 mg orally every 6 h) IV atenolol (5 mg over 5 min, repeat in 10 min, then 50 mg orally 10 min after the last IV dose) OR 2. IV labetalol in 20 mg aliquots followed by infusion of 2 mg/min (in place of sodium nitroprusside and a b-blocker) OR 3. IV trimethaphan (0.3–6 ml/min) (in place of sodium nitroprusside and a b-blocker) Normotensive patient: IV b-blocker alone (see above)

Table 3.2 Pharmacologic therapy for acute aortic dissection

relief of cardiac tamponade complicating acute aortic dissection is best avoided since it may precipitate hemodynamic collapse and death.15 In addition to cardiac tamponade, the closer the entry site is to the aortic root, the worse the prognosis of Type A aortic dissection – another bit of important information available with echocardiography.16 The patient with a chronic proximal (Type A, Type I or II) dissection as a result of successful medical therapy (or one discovered incidentally on a chest roentgenogram) who has survived the risks of the acute phase may develop complications, e.g. aortic valve regurgitation or enlargement of the ascending aorta, or arch, to more than 5 cm in diameter, that warrant surgical treatment if the patient is an acceptable surgical risk. In the patient with a chronic distal (Type B, Type III) aortic dissection, the indication for surgical treatment is enlargement of the aorta to more than 5 cm in diameter, or rupture. Dissections beginning in the abdominal aorta are rare. They can be chronic and present as a pulsatile abdominal mass or be acute and present with abdominal pain. Based on a limited number of cases, surgical treatment (or perhaps stented graft placement) is advised for infrarenal dissection while pharmacologic treatment is recommended for suprarenal dissection.17 Although dissection may occur in any peripheral artery, dissection in the internal carotid artery may involve the cardiologist because in addition to


57

Figure 3.8 Pathology specimens of penetrating atherosclerotic aortic ulcers.

neck pain it may present as a cerebrovascular accident or as syncope.18 Fortunately, more than 85% have an excellent or complete recovery. Recurrence or rupture does not usually occur. Most cases of iatrogenic aortic dissection follow cardiac catheterization or cardiac surgery. Patients with iatrogenic dissection tend to be older and have more coronary disease than those with classic spontaneous aortic dissection but have essentially similar outcomes and complications. Management is the same as for classic aortic dissection.19

Atherosclerotic aortic ulcer and intramural hematoma Penetrating atherosclerotic ulcer (Figure 3.8) and localized intramural hematoma (IMH) of the aorta, while described much earlier from pathologic studies of cases of aortic dissection (penetrating atherosclerotic ulcer by Shennan in 193420 and Willius in 1941,21 and IMH as ‘‘dissection without a tear’’ by Krunkenberg in 192022), have become fairly common clinical problems with the evolution of noninvasive imaging techniques. Their distinguishing features can be conveniently summarized (Table 3.3). Clinical features that characterize patients with penetrating atherosclerotic ulcer and IMH include associated hypertension in more than 80% and advanced age. Both mimic classic aortic dissection in presenting with chest pain – anteriorly when the lesion is in the ascending aorta and posteriorly when the process is in the descending aorta (more frequent than in the ascending

58 Chapter 3 Table 3.3 Comparison of acute thoracic aortic disorders Acute aortic dissection

Feature

Proximal

Distal

Penetrating aortic ulcer

Intramural hematoma

Risk factors

Hypertension Marfan syndrome Aortic valve deformity Coarctation

Hypertension

Hypertension

Hypertension

Chest pain, frequent location

Anterior

Posterior

Posterior

Posterior

Clinical signs Aortic regurgitation Pulse changes, arms Blood pressure changes

+ + +

Occasional

Common

Common

þ þ +

þ þ

þ þ

þ +

Associated AAA* (past, present) Image findings Intimal flap False channel PAU{ IMHz Aortic regurgitation *Abdominal aortic aneurysm

{Penetrating aortic ulcer z

Intramural hematoma

aorta) as originally described by Burchell for acute classic aortic dissection.12 The rate of rupture is high at the time of initial presentation, and so is the rate of progression, so surgical treatment in good-risk patients is suggested by some.23 Associated symptoms due to ischemia of various organ systems are not a part of the clinical picture of these variants of aortic dissection since they do not compromise the branches of the aorta. One important clinical point is the frequent presence or occurrence of abdominal aortic aneurysms in these patients. Penetrating atherosclerotic ulcers may result in IMH or if they penetrate the media result in a pseudoaneurysm or perforate the adventitia and rupture.24 In their review of the literature, Movsowitz and associates25 have developed a practical treatment strategy for patients with symptomatic penetrating aortic ulcer; they recommend surgery for those patients who are hemodynamically unstable, those with penetrating ulcer in the ascending aorta or arch, and for those who develop pseudoaneurysm or rupture.


59

Patients with uncomplicated penetrating ulcer receive antihypertensive therapy reducing the blood pressure until pain is relieved and then monitoring while on continued antihypertensive therapy and serial imaging so long as they remain hemodynamically stable, pain-free, and show no change in the appearance of the aorta. For patients with asymptomatic aortic ulceration, careful blood pressure control and close follow-up is advised. For patients with penetrating atherosclerotic aortic ulcer for whom surgical repair is recommended, an alternative is endovascular repair with stents. This may become the treatment of choice in selected cases.26 Another variant, at times a precursor to acute aortic dissection, is aortic IMH. Like acute aortic dissection, aortic IMH presents with chest pain, the location of which depends on the portion of the thoracic aorta involved (ascending aorta, anterior chest pain; descending aorta, interscapular or posterior chest pain). Earlier experience suggested a strategy well summarized by Nienaber and associates:27 early surgical repair should be considered for all patients with ascending aortic involvement (Type A IMH) and for any patient with persistent or recurrent pain. Conversely, surgery may not be required in all patients with IMH of the descending thoracic aorta. In particular, older, high-risk individuals in whom both hematoma of the descending aorta and pain can be controlled with antihypertensive therapy may benefit from a conservative approach.

As experience with larger groups of patients with Type A aortic IMH has been gained, the risk of progression is high so that early surgical repair along with beta-blockade is indicated in good risk patients.28,29 Poor risk patients whose hypertension can be stabilized with beta-blockade can be managed medically with frequent follow-up imaging studies.30

References 1 Larson E.W. & Edwards W.D. (1984) Risk factors for aortic dissection: A necropsy study of 161 cases. Am J Cardiol 33: 849–855. 2 Fikar C.R. & Koch S. (2000) Etiologic factors of acute aortic dissection in children and young adults. Clin Pediatr 39: 71–80. 3 Nicod P., Bloor C., Godfrey M., et al. (1989) Familial aortic dissection. JACC 13: 811–819. 4 Lewis R.A. & Meriu L.M. (1995) Iris flocculi and familial aortic dissection. Arch Ophthalmol 113: 1330–1331. 5 VanKien P.K., Lalande A., Bonnet C., et al. (2002) Familial aortic dissection/aneurysm associated with patent ductus arteriosus: A new entity? JACC 39 (Suppl A): 264A. 6 Spittell J.A. Jr (1971) Differential diagnosis of dissecting aneurysm. Prog Cardiovasc Dis 14: 226–229. 7 Mehta R.H., O’Gara P.T., Bossone E., et al. (2002) Acute Type A aortic dissection in the elderly: Clinical characteristics, management and outcomes in the current era. JACC 40: 685–692. 8 Earnest F. IV, Muhm J.R. & Sheedy P.F. (1979) Roentgenographic findings in thoracic aortic dissection. Mayo Clin Proc 54: 43–50. 9 Goldstein S.A. & Lindsay J. Jr (2001) Aortic dissection: Noninvasive evaluation. ACC Curr J Rev 10: 18–20.

60 Chapter 3 10 Dake M.D., Kato N., Mitchell R.S., et al. (1999) Endovascular stent-graft placement for the treatment of acute aortic dissection. N Engl J Med 340: 1546–1562. 11 Beckman J.A., Mehta R.H., Bossane E., et al. (2003) Renal failure on presentation predicts morbidity and mortality in aortic dissection. JACC 41 (suppl. A): 235A. 12 Burchell H.B. (1955) Aortic dissection (dissecting hematoma; dissecting aneurysm of the aorta). Circulation 12: 1068–1079. 13 Bayegan K., Domanovits H., Schillinger M., et al. (2001) Acute type A aortic dissection: The prognostic impact of preoperative cardiac tamponade. Eur J Cardiothorac Surg 20: 1194–1198. 14 Nallamothu B.K., Mehta R.H., Saint S., et al. (2002) Syncope in aortic dissection: Diagnostic, prognostic and clinical implications. JACC 39 (Suppl A): 241A. 15 Isselbacher E.M., Cigarron J.E. & Eagle K.A. (1994) Cardiac tamponade complicating proximal acute aortic dissection: Is pericardiocentesis harmful? Circulation 90: 2375–2378. 16 Richartz B.M., Smith D.E., Cooper J.V., et al. (2002) New classification of aortic dissection with improved impact on prognosis. JACC 39 (Suppl A): 264A. 17 Becquemin J., Delenze P., Watelet J., et al. (1990) Acute and chronic dissections of the abdominal aorta: Clinical features and treatment. J Vasc Surg 11: 397–402. 18 Mokri B., Sundt T.M. Jr, Houser O.W., et al. (1986) Spontaneous dissection of the cervical internal carotid artery. Ann Neurol 19: 126–138. 19 Januzzi J.L., Evangelista A., Eagle K.A., et al. (2000) Iatrogenic aortic dissection: A distinct clinical entity – Results from the International Registry of Acute Aortic Dissection (IRAD). Circulation 102: 11–401(1952). 20 Shennan T. (1934) Dissecting aneurysms. Medical Research Council Special Report Series, No 193. London: His Majesty’s Stationery Office. 21 Willuis F.A. & Cragg R.W. (1941) A talk on dissecting aneurysm of the aorta. Staff Meet. Mayo Clin 16: 41–44. 22 Krunkenberg E. (2001) Quoted by Mohr-Kahaly S. Aortic intramural hematoma: From observation to therapeutic strategies. JACC 37: 1611–1613. 23 Elefteriades J.A. (2002) Thoracic aortic aneurysm: Current approach in surgical timing. ACC Curr J Rev 11: 82–88. 24 Stanson A.W., Kazmier F.J., Hollier L.H., et al. (1986) Penetrating atherosclerotic ulcers of the thoracic aorta: Natural history and clinicopathological correlations. Ann Vasc Surg 1: 15–23. 25 Movsowitz H.D., Lampert C., Jacobs L.E., et al. (1994) Penetrating atherosclerotic aortic ulcers. Am Heart J 128: 1210–1217. 26 Demers P., Miller D.C., Kee S.T., Chagonjean L., Dake M.D. (2003) JACC 41 (Suppl. A): 236A. 27 Nienaher C.A., Kodolitsch Y., Petersen B., et al. (1995) Intramural hemorrhage of the thoracic aorta: Diagnostic and therapeutic implications. Circulation 92: 1465–1472. 28 Mikidski K.J., McRae A.T., Penn M.S. et al. (2003) JACC 41 (Suppl. A): 235A. 29 Von Kodolitsch Y., Crosz S.K., Koschyk D.H. et al. (2003) Intramural hematoma of the aorta: predictors of progression to dissection and rupture. Circulation 107: 1158–63. 30 Mohr-Kahaly S. (2001) Aortic intramural hematoma: From observation to therapeutic strategies. JACC 37: 1611–1613.

CHAPTER 4

Arteritis

Since cardiac patients, like all other persons, can develop one of the arteritides, familiarity with their clinical manifestations by the attending cardiologist will avoid delays in their recognition, diagnosis, and management. Furthermore, cardiovascular manifestations may be the dominant or the presenting clinical feature of an arteritis. Involvement of the peripheral arteries usually small arteries or arterioles (by an arteritis) can present as an acute arterial occlusion (Figure 4.1) or as symptomatic chronic occlusive peripheral arterial disease (Figure 4.2). The most likely arteritis that the cardiologist may encounter is giant-cell arteritis which has been described as ‘‘a cardiological blind spot.’’1 The clinical manifestations can be quite variable from the rather classic case of temporal (cranial) arteritis, e.g. an elderly person complaining of polymyal-

Figure 4.1 Gangrene of toe due to digital artery thrombosis in a 56-year-old woman with a circulating anticoagulant and systemic lupus erythematosus (SLE).

61

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Figure 4.2 Angiograms demonstrating arterial involvement in giant-cell arteritis. (a) Smooth-walled segmental narrowing alternating with areas of normal caliber or poststenotic dilatation in subclavian and axillary arteries. (From Klein R.G., Hunder G.G., Stanson A.W., et al. (1975) Large artery involvement in giant-cell (temporal) arteritis. Ann Int Med 83: 806–812. By permission of American College of Physicians.) (b) Severe segmental narrowing of superficial femoral arteries in a 51-year-old woman with polymyalgia rheumatica and intermittent claudication; the left popliteal artery is occluded. (c) Same patient as in (b) after 6 months treatment with prednisone. Left popliteal artery remains occluded but superficial femoral arteries now appear normal. (From Hunder G.G. & Sheps S.C. (1967) Intermittent claudication and polymyalgia rheumatica associated with panarteritis. Arch Int Med 119: 638–643. By permission of American Medical Association.)

Arteritis 63

gia, malaise, anorexia, and headache with prominent reddened, nodular, and tender superficial temporal arteries (Figure 4.3), to a similar aged person with one or more of the protean manifestations (Table 4.1) of what is really a systemic arteritis that can involve the aorta and any of its branches.2 The principal laboratory findings in active giant-cell arteritis are an elevated sedimentation rate (often 100 mm in 1 hour or higher), a mild normochromic and normocytic anemia, and leukocytosis. In some cases there are thrombocytosis and erythrocyte casts in the urine.2 In the case of upper extremity arterial involvement by giant cell arteritis, an arch aortogram will show segmental stenoses and tapered arterial occlusions as seen in Figure 4.2. Faced with a patient over the age of 55 years and findings consistent with a diagnosis of giant-cell arteritis, angiography or temporal artery biopsy should be considered since prompt therapy with adequate doses of corticosteroids can relieve symptoms and prevent blindness and other complications, some of which (myocardial infarction, aortic dissection, stroke) can be fatal.3 Therapy with prednisone 1–2 mg per kg body weight per day initially (or an equivalent dose of a related steroid) is the usual practice. When symptoms abate in a few days, the dose of prednisone can be reduced to about 1 mg per kg per day for the next 4–6 weeks and then gradual tapering of the dose by 10% weekly as long as there is no recurrence of symptoms. Takayasu’s arteritis (idiopathic medial aortopathy, pulseless disease) should be included in the differential diagnosis of young women, particularly of Asian descent, who present with one of the many possible cardiovascular manifestations. Depending on the site of arterial involvement there may be cerebrovascular insufficiency, intermittent claudication in the arms or legs, hypertension, mesenteric insufficiency, angina, myocardial infarction, or aortic or mitral valve insufficiency. The hypertension may be overlooked if the brachial arteries are involved by the arteritis and the blood pressure is not checked in uninvolved arteries; mild anemia and an elevated sedimentation are seen when the arteritis is active.4 When Takayasu’s arteritis is suspected, angiographic study is indicated for confirmation. The symptoms of Takayasu’s arteritis include irregular segmental stenoses in large arteries near their origin from the aorta (Figure 4.4) and narrowing of the lumen due to the thickening of the wall of the aorta and its branches. Corticosteroid therapy is indicated when Takayasu’s arteritis is considered to be active and surgical or interventional treatment of occlusive arterial lesions is best deferred until the disease is inactive. Another widespread arteritis that involves chiefly small- and mediumsized arteries and arterioles is periarteritis nodosa. The signs and symptoms are often protean depending on the organs involved and therefore frequently simulate other diseases. The cardiovascular manifestations are less common than fever, neurologic (mononeuritis multiplex), cutaneous (rash and petechiae), and musculoskeletal (myalgia and arthralgia) but include hypertension, abnormal electrocardiogram (usually left ventricular hypertrophy), angina pectoris, and pericarditis. While angina is actually an infrequent

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Figure 4.3 Prominent beaded superficial temporal arteries in a man with active giant-cell (temporal, cranial) arteritis.

Cranial artery involvement Headache Visual symptoms Amaurosis fugax Diplopia Blindness Jaw ‘‘claudication’’ Aortic involvement Aortic arch syndrome Aortic valve incompetence Aortic aneurysm Aortic dissection Arterial involvement Coronary Angina Myocardial infarction Cerebral Transient cerebral ischemia Stroke Extremity Claudication – arms or legs Raynaud’s phenomenon Subclavian steal Renal Erythrocyte casts Mesenteric Abdominal angina

Table 4.1 Clinical manifestations of giant-cell (cranial, temporal) arteritis

Arteritis 65

Figure 4.4 Arch aortograms in a 28-year-old woman with Takayasu’s arteritis showing narrowing of both common carotid arteries (horizontal arrows) and both subclavian arteries (vertical arrows) and occlusion of left axillary artery. (From Sheps S.G. & McDuffie F.C. (1980) Vasculitis. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Disease, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)

complaint in patients with periarteritis nodosa, one autopsy study of 66 cases reported finding arteritis of the coronary arteries with myocardial infarctions in 41 but a clinical diagnosis of myocardial infarction had been made in only three of these cases.5 Peripheral vascular disorders (arterial occlusion and thrombophlebitis) are less common than cardiac disorders.6 In patients with obscure progressive illnesses and features of involvement of multiple organ systems, with an elevated sedimentation rate, periarteritis nodosa should come to mind, and in an effort to make the diagnosis biopsy of an accessible tissue (skin, testicle, nerve, or muscle) should be undertaken. At times the diagnosis can be made angiographically by demonstrating multiple intraparenchymal renal artery aneurysms (Figure 4.5) or similar aneurysms in other visceral arteries. The treatment of periarteritis is the same as described for giant-cell arteritis though tapering of the dose of steroids must be done slowly while closely observing for signs of recurrent activity of the arteritis. Pericarditis and valvular heart lesions may involve the cardiologist in the care of patients with one of the connective tissue disorders – specifically systemic lupus erythematosus (SLE) or scleroderma. Either of these diseases can have these disorders as clinical manifestations – either as complications or presenting manifestations. The arteritis seen with these connective tissue disorders usually involves small peripheral arteries, most often the digital arteries. The occlusive arterial disease can be chronic as seen in scleroderma (Figure 4.6) or acute as seen in SLE (Figure 4.1). Commonly both SLE and

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Figure 4.5 Selective left renal arteriogram showing aneurysm of small intrarenal artery in patient with periarteritis nodosa. (From Sheps S.G. & McDuffie F.C. (1980) Vasculitis. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Disease, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)

Figure 4.6 Gangrene on tip of index finger due to digital artery thrombosis in a patient with scleroderma. Note the typical telangiectatic spots on third finger.

scleroderma have Raynaud’s phenomenon (see Chapter 5) as the principal evidence of peripheral arterial involvement by the underlying disease. The management of the peripheral arterial involvement in arteritis is directed at the systemic disorder in addition to management of any ischemia resulting from the arteritis.

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References 1 How J., Strachan R.W. & Bresher P.O. (1980) Giant cell arteritis: A cardiological blind spot? Am Heart J 100: 405–407. 2 Klein R.G., Hunder G.G., Stanson A.W., et al. (1975) Large artery involvement in giant cell (temporal) arteritis. Ann Int Med 83: 806–812. 3 Sa¨ve-So¨derbergh J., Malmvall B., Andersson R., et al. (1986) Giant cell arteritis as a cause of death: Report of nine cases. JAMA 255: 493–496. 4 Hall S., Barr W., Lie J.T., et al. (1985) Takayasu arteritis: A study of 32 North American patients. Medicine 64: 89–99. 5 Holsinger D.R., Osmundson P.J. & Edwards J.E. (1962) The heart in periarteritis nodosa. Circulation 25: 610–618. 6 Frohnert P.P. & Sheps S.G. (1967) Long-term follow-up study of periarteritis nodosa. Am J Med 43: 8–14.

CHAPTER 5

Vasospastic disorders1

Vasospastic disorders (Table 5.1) involve the small arteries and arterioles of the skin and digits. Color changes of the digits or skin, aggravated by exposure to cold and/or stress, are the hallmark of these vasospastic disorders. Familiarity with the usual clinical features of the vasospastic disorders and their management is a useful part of cardiovascular practice. The reported association of Raynaud’s phenomenon with pulmonary hypertension in some patients2 and the occasional patient with Raynaud’s phenomenon, migraine headaches, and variant angina3 are specific, though rare, examples of value to the cardiologist having an interest in the vasospastic disorders.

Raynaud’s phenomenon Raynaud’s phenomenon, the best known and most common vasospastic disorder, is the one that a cardiologist is most likely to encounter since it may be experienced (as a side effect) by the person taking a beta-blocker drug as well as by a patient with a primary, or secondary, type of Raynaud’s phenomenon. An important therapeutic, or diagnostic, opportunity may be missed unless a question about color changes of the digits is included in the medical history, as patients may not volunteer this information since they may consider it only a minor nuisance or even a normal variant. Several clinically useful points can be elicited by asking some specific questions about Raynaud’s phenomenon (Table 5.2). Answers to these questions will often readily distinguish primary Raynaud’s phenomenon (Raynaud’s disease) from a secondary type of Raynaud’s phenomenon. The typical patient with Raynaud’s disease is a young woman (less than 40 years of age at onset of her vasospasm) experiencing intermittent color changes (frequently symmetrical) in one or more fingers and/or toes on exposure to cold. The color changes commonly are in three phases – pallor, cyanosis, and rubor – but occasionally are only pallor or cyanosis. The diagnosis of Raynaud’s disease is relatively straightforward if it is made only in patients whose Raynaud’s phenomenon is bilateral and present for at least 2 years and in whom none of the secondary causes of Raynaud’s phenomenon are evident. Physical examination of the involved digits in Raynaud’s disease reveals no abnormalities and the peripheral arterial circulation, including the Allen test (Figure 1.11), is normal. Laboratory studies, including noninvasive vascular laboratory studies are not necessary in the typical case but if there is some 68

Vasospastic disorders 69 Table 5.1 Vasospastic disorders Raynaud’s phenomenon Livedo reticularis Acrocyanosis Chronic pernio Reflex sympathetic dystrophy

Table 5.2 Raynaud’s phenomenon Some clinically useful questions: . When did it first occur? . Which fingers were involved? . Have the thumbs been involved? . Are the toes involved? . What medications are you taking?

symptom, such as a sedimentation rate, tests for antinuclear antibody and for cold proteins can be carried out to exclude the systemic causes of secondary Raynaud’s phenomenon. Management of Raynaud’s disease should include reassurance that loss of extremities (often a concern of these patients) never occurs. Instruction about protection from cold generally, as well as of the extremities, avoidance of tobacco and any medications that have vasoconstriction as their primary action or as a side effect, and protection of the fingers and toes from injury are basic. Pharmacologic treatment with calcium-channel blockers or an alpha-adrenergic blocker can be used to lessen the frequency or severity of the vasospasm though these agents should be avoided in women in the childbearing age if there is a possibility of pregnancy. In the latter situation as well as for other patients in whom the use of pharmacologic agents (e.g. pilots, truck drivers) is avoidable, instruction in biofeedback techniques may be helpful to lessen vasospasm. Sympathectomy can be used in the extreme case but is most beneficial when the Raynaud’s phenomenon involves mainly the toes. In the upper extremity the benefit from sympathectomy is less predictable and when effective often lasts for only a year or two. Secondary Raynaud’s phenomenon may occur in a variety of conditions (Table 5.3) – occlusive peripheral arterial disease, neurologic disorders, aftertrauma to the extremity, the connective tissue disorders, and as a side effect of drugs (ergot preparations, some beta-blockers, and certain types of chemotherapy). One clinical axiom to remember is that Raynaud’s phenomenon confined to one upper extremity is secondary and the cause is located in that extremity (Figure 5.1). Another axiom is that Raynaud’s phenomenon of all fingers and the thumbs can occur in myxedema and is relieved by treatment of the myxedema. For cardiologists, those cases secondary to occlusive arterial disease (particularly thromboangiitis obliterans (TAO), scleroderma (Figure 5.2), myxedema, and drugs) are the main ones of which to be aware since the management of secondary Raynaud’s phenomenon is that of the underlying cause as well as the general measures described for Raynaud’s disease.

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Occlusive arterial disease Arteriosclerosis obliterans Thromboangiitis obliterans Post-acute arterial occlusion Neurologic disorders Carpal tunnel syndrome Thoracic outlet compression syndrome Hand trauma Occupational Pneumatic hammer disease Connective tissue disorders Scleroderma Lupus erythematosus Rheumatoid arthritis Miscellaneous conditions Myxedema Primary pulmonary hypertension Pheochromocytoma Cold agglutinins Cryoglobulinemia Ergotism

Table 5.3 Causes of secondary Raynaud’s phenomenon

Figure 5.1 Unilateral (fourth left finger) Raynaud’s phenomenon in a 50-year-old right-handed man with left thoracic outlet compression syndrome. (a) Arteriogram showing compression of left subclavian artery as it crosses uppermost rib in thoracic outlet. (b) Arteriogram of left hand showing multiple digital artery occlusions in second through fifth fingers. (Reproduced from Spittell J.A. Jr & Spittell P.C. (2000) Peripheral Vascular Disease in ACCSAP. American College of Cardiology Foundation, Bethesda, MA. By permission of the American College of Cardiology.)

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Figure 5.2 Multiple ischemic ulcerations of tips of fingers due to digital artery occlusive disease in a 37-year-old woman with scleroderma and secondary Raynaud’s phenomenon.

Figure 5.3 Livedo reticularis of lower back and buttocks. (Reproduced from Spittell J.A. Jr (1984) Vasospastic disorders. Curr Prob Cardiol 8: 3–27. By permission of Mosby, Inc.)

Livedo reticularis Livedo reticularis (Figure 5.3), the bluish mottling of the skin produced by spasm or occlusion of the dermal arterioles, can be a primary disorder or a secondary manifestation of another disorder (Table 5.4). For cardiologists, several of the secondary types of livedo reticularis are of importance. The

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Table 5.4 Livedo reticularis – A classification Primary livedo reticularis Secondary livedo reticularis Myeloproliferative disorders Connective tissue disorders Dysproteinemias Atheroembolism (cholesterol emboli) Use of amantadine hydrochloride

primary type of livedo reticularis, usually seen in young women, will generally come to the attention of primary care physicians or dermatologists as a cosmetic problem. As a rule, the secondary types of livedo reticularis are of recent onset in the patient’s history. If the cause of the livedo is not evident from the history, physical examination and routine laboratory studies (the same investigation as for unclassified Raynaud’s phenomenon) are appropriate. The main secondary type of livedo reticularis of cardiovascular importance is atheroembolism (cholesterol embolization), which should be a first consideration when the livedo is of recent onset in a person over 50 years of age. The livedo reticularis and blue toes seen in atheroembolism are due to occlusion of arterioles and digital arteries by atheromatous debris (arising spontaneously or following surgery or an interventional procedure) from an aneurysm (Figures 2.1 and 2.7) or an atherosclerotic aorta (Figure 5.4). Occasionally, atheroembolism occurs at the time anticoagulant therapy is started,4,5 making its use a cause of concern, though there are sporadic reports of resolution of toe lesions with warfarin therapy.6 In the case of spontaneous atheroembolism from atherosclerotic plaques or aneurysms, the best approach is removal of the source of atheromatous debris when that is feasible. Since the incidence of atheroembolism complicating interventional and diagnostic procedures is increasing (with our elderly population), awareness of the major risk factors (age more than 60, female gender, peripheral occlusive arterial disease, and repeated procedures is important)7 and strategies to minimize the occurrence of atheroembolism (e.g. statins to decrease systemic inflammation) warrant investigation.8 The livedo reticularis seen in an occasional patient receiving amantadine hydrochloride for Parkinsonism is a benign side effect. This livedo reticularis will disappear 2–4 weeks after the drug is discontinued.

Acrocyanosis Although not a common clinical problem, acrocyanosis is important to the cardiologist since the question of a cardiac cause for the cyanosis may lead to referral of the patient for cardiac consultation. With no cardiac or pulmonary basis, acrocyanosis is a benign vasospastic disorder that occurs in some young


Figure 5.4 Atheromatous lesions of abdominal aorta.

women. The mechanism appears to be a local fault causing heightened tone of the small arterioles at ordinary environmental temperatures. The discoloration and coldness of the hands and fingers and occasionally the feet and toes is constant, distinguishing acrocyanosis from Raynaud’s phenomenon. Occasionally, the presence of erythermalgia (erythromelalgia) is suspected but the cold acral parts of acrocyanosis readily distinguish it from the discolored (red) warm acral parts in persons with erythermalgia (see Chapter 10). Treatment of acrocyanosis is reassurance and protection from cold. If the patient is concerned enough by the discoloration and coldness caused by the acrocyanosis, a trial of a small dose of an alpha-adrenergic blocker or a calcium-channel blocker is reasonable but, as noted for Raynaud’s phenomenon, pharmacologic therapy should be avoided in women of childbearing age who may become pregnant.

Chronic pernio Although chronic pernio is usually considered one form of the pernio syndrome (a group of disorders related to cold injury), it has a large vasospastic component that justifies its inclusion with other vasospastic disorders. Its inclusion in a presentation to cardiologists is warranted because it has such unique and classic symptoms that recognition is easy if one is aware of the disorder. Furthermore, treatment and prevention are just as easy.

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The typical case (Figure 5.5) is a woman, more often than a man, with symmetrical erythematous or bluish discoloration and blisters or ulcerations of the toes (Figures 5.5a & b) and sometimes the fingers (Figure 5.5c) that develop with the onset of cold weather and disappear in warm weather. (Usually a history of prior exposure to cold weather can be elicited if inquiry is made.) Treatment with an alpha-adrenergic blocking agent (e.g. doxasosin

Figure 5.5 Chronic pernio. (a) Feet of a 70-year-old woman who noted itching and blisters of the big toes with onset of cold weather in the previous 4 years. Toe lesions cleared with warm weather. The patient had frostbite as a child in northern Minnesota. (Reproduced from Spittell J.A. Jr (1984) Vasospastic disorders. Curr Prob Cardiol 8: 3–27. By permission of Mosby, Inc.) (b) Close-up of toes of patient in (a). (c) Hands of a veteran who had frostbite in his hands in World War II in Europe. Fifty years later began to have blistering and fissuring of third fingers with onset of cold weather but the lesions cleared in spring.



1 mg once or twice a day) will relieve the toe discoloration and lesions in a few days. The same drug can be used to prevent the manifestations of chronic pernio by beginning it in late summer (e.g. Labor Day – the first Monday of September) and continuing it through the winter until spring (e.g. Memorial Day – the last Monday of May) each year.

Reflex sympathetic dystrophy Reflex sympathetic dystrophy, a post-traumatic neurovascular disorder, is included here because the clinical features may include one or more vasospastic disturbances of the affected extremity – cyanosis, livedo reticularis, Raynaud’s phenomenon, and coldness. The onset of the symptoms and signs follows some type of injury, often trivial, to the involved extremity. Recognition and management of the troubling vasospastic component of reflex sympathetic dystrophy is appreciated by these persons and is a useful contribution to the management of this difficult disorder. Relief of the vasospastic component can be achieved by prescribing a small dose of an alpha-adrenergic blocking drug and this often will facilitate the patient’s compliance with the necessary rehabilitative program in the physical medicine department.

References 1 Spittell J.A. Jr (1984) The vasospastic disorders. Curr Prob Cardiol 8: 3–27. 2 Winters W.L. Jr, Joseph R.R. & Lerner N. (1964) ‘‘Primary’’ pulmonary hypertension and Raynaud’s phenomenon. Arch Int Med 114: 821–830. 3 Miller D., Waters D.D., Warnica W., et al. (1981) Is variant angina the coronary manifestation of a generalized vasospastic disorder? N Eng J Med 304: 763–766.

76 Chapter 5 4 Feder W. & Auerback R. (1961) ‘‘Purple toes’’: An uncommon sequela of oral coumarin drug therapy. Ann Int Med 55: 911–917. 5 Belenfont X., d’Auzac C., Bariety J., et al. (1997) Cholesterol crystal embolization during treatment with low-molecular-weight heparin. Presse Med 26: 1236–1237. 6 Blackshear J.L., Jahangir A., Oldenburg A.W., et al. (1993) Digital embolization from plaque-related thrombus in the thoracic aorta: Identification with transesophageal echocardiography and resolution with warfarin therapy. Mayo Clin Proc 68: 268–272. 7 Bashore T.M., Gehrig T. (2003) Cholesterol emboli after invasive procedures. JACC 42: 217–218. 8 Fukomoto Y., Tsutsui H., Tsuchihashi M. et al. (2003) The incidence and risk factors of cholesterol embolization syndrome, a complication of cardiac catheterization: a prospective study. JACC 42: 211–216.

CHAPTER 6

Venous disorders

Venous disorders are understandably not the area of primary concern for the cardiologist, but they do occur in cardiac patients and may be significant complications and comorbid problems. Furthermore, acute pulmonary embolism (PE) is one of the causes of dyspnea and/or acute chest pain that needs consideration once heart disease, acute myocardial infarction, and acute aortic dissection are excluded in the patient presenting with these symptoms. Also, when PE is massive, the cardiac findings of acute right ventricular dysfunction and/or syncope often involve the cardiologist to perform echocardiography and assist in management.

Venous thromboembolism Congestive heart failure (venous stasis) and the postoperative state, both wellknown risk factors (Table 6.1) for acute venous thromboembolism (VTE), are situations in which the cardiologist needs to keep this complication and its treatment, especially prophylaxis (Table 6.2),1 in mind. The recognition in recent years of hypercoagulable states predisposing to VTE has added to the body of knowledge of which the informed cardiologist needs to be aware (Table 6.3).2 The symptoms and signs of acute deep vein thrombosis (DVT), other than the rare phlegmasia cerulea dolens (Figure 6.1), are nonspecific and mimicked by numerous muscular (Figure 6.2), skeletal (Figure 6.3), and infectious conditions (Figure 6.4) and even acquired arteriovenous fistula (Figure 6.5). Acute thrombosis of the iliofemoral vein is usually not difficult to distinguish from other acute vascular problems in the lower extremity (Table 6.4). The use of objective testing following the history and physical examination is essential however, to confirm or exclude the diagnosis as shown in the algorithm for the management of the patient with suspected acute DVT (Figure 6.6). Duplex ultrasonography is currently the test of choice for the diagnosis of suspected DVT, but a recent study of magnetic resonance direct thrombus imaging (MRDTI) has shown it to be accurate and to have the advantage of diagnosing isolated calf, as well as proximal, DVT.3 Provided there are no contraindications to its use, anticoagulant therapy, initially with heparin and oral anticoagulant therapy, should be instituted and the latter should be continued long term for at least 6 months. In selected cases (extensive DVT of the lower extremity and axillary subclavian veins), thrombolytic therapy is used initially to restore venous patency and is followed by anticoagulant therapy to hopefully lessen postphlebitic venous insufficiency (Figure 6.7). In addition to preventing PE 77

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Advanced age Obesity Prior history of VTE Prolonged immobilization Malignancy Major surgery* Hypercoagulable states{ Congestive heart failure Fractures of pelvis and lower extremity Venous catheters Inflammatory bowel disease Nephrotic syndrome Pregnancy Oral contraceptives and estrogen therapy

Table 6.1 Risk factors for venous thromboembolism (VTE)

*Particularly operations involving the abdomen, pelvis, or lower extremities { See Table 6.3

and restoring deep venous patency to lessen the likelihood of postphlebitic deep venous insufficiency, the goal of antithrombotic therapy is prevention of recurrent VTE. To effect the latter, continued oral anticoagulant therapy with an international normalized ratio (INR) of 2:3 for 6 months is recommended.4,5 Persons with a hereditary hypercoagulable state (Table 6.3) who experience an episode of VTE present a special problem in management. Generally, with a single non-life-threatening episode related to an identifiable riskproducing factor, anticoagulant therapy for 6 months is appropriate if the risk-producing factor is no longer present. Long-term anticoagulant therapy is generally indicated for thrombophilic persons whose initial thromboembolic event was life threatening, for those with more than one allelic coagulation abnormality (e.g. homozygous Leiden mutation for factor V or a combination of heterozygous Leiden mutation for factor V combined with the prothrombin G20210A mutation), and for those who experience two or more spontaneous episodes of VTE.6 When caring for a postmenopausal woman who has a hypercoagulable disorder and has experienced acute venous thrombosis, the question of hormonal replacement therapy may come up and it is contraindicated because of the increased risk of venous thrombosis with its use.7 At this point postphlebitic deep venous insufficiency8 warrants consideration since it is a great cause of morbidity if not recognized and correctly managed. When not controlled with proper graded elastic support hose (30–40 mm Hg compression at the ankle), the elevated ambulatory venous pressure in the extremity results in dependent edema, followed later by changes of chronic venous stasis, such as stasis pigmentation, chronic indurated cellulitis (Figure 6.8), and ultimately venous stasis ulceration (Figure 6.9). All of the complications of chronic deep venous insufficiency can be prevented by the regular use of adequate elastic support on the affected extremity whenever the patient is ambulatory.

Venous disorders 79 Table 6.2 Levels of thromboembolism risk in surgical patients without prophylaxis Successful prevention strategies

Level of risk examples

Calf DVT (%)

Proximal DVT (%)

Clinical PE (%)

Fatal PE (%)

Low risk Minor surgery in patients < 40 yr with no additional risk factors

2

0.4

0.2

0.002

No special measures; Aggressive mobilization

Moderate risk Minor surgery in patients with additional risk factors; nonmajor surgery in patients aged 40–60 yr with no additional risk factors; major surgery in patients > 40 yr with no additional risk factors

10–20

2–4

1–2

0.1–0.4

LDUH q12h, LMWH, ES, or IPC

High risk Nonmajor surgery in patients > 60 yr or with additional risk factors; major surgery in patients < 40 yr or with additional risk factors

20–40

4–8

2–4

0.4–1

LDUH q8h, LMWH, or IPC

Highest risk Major surgery in patients > 40 yr plus prior VTE, cancer, or molecular hypercoagulable state; hip or knee arthroplasty, hip fracture surgery; major trauma; spinal cord injury

40–80

10–20

4–10

0.2–5

LMWH, oral anticoagulants, IPC/ES þ LDUH/ LMWH, or ADH

Modified from Geerts W.H., Heit J.A., Clagett C.P., et al. (2001) Prevention of venous thromboembolism. Chest 119 (Suppl): 132S–175S. With permission. LDUH, low dose unfractionated heparin; LMWH, low-molecular-weight heparin; ES, elastic (graduated compression) stockings; IPC, intermittent pneumatic compression; ADH, adjusted dose heparin.

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External venous compression (Figures 2.13 and 6.3) can mimic acute DVT in most respects except for the usual absence of tenderness to palpation. Unfortunately, not all deep venous obstruction mimicking DVT is due to a benign cause; the cause may be occult at onset (Figure 6.10) warranting careful investigation and this needs to be kept in mind. Another location of

Hereditary Leiden mutation of gene for factor V (resistance to activated protein C) Prothrombin G20210A mutation Antithrombin III deficiency Deficiency of protein C Deficiency of protein S Heparin cofactor II deficiency Dysfibrinogenemia Abnormal plasminogen Hyperhomocysteinemia Acquired Neoplastic disease Myeloproliferative disorders Circulating anticoagulant (antiphospholipid antibody syndrome) Ulcerative bowel disease Oral contraceptives

Table 6.3 Hypercoagulable disorders

Figure 6.1 Phlegmasia cerulea dolens in right lower extremity. Note discoloration of right foot. (Reproduced from Spittell J.A. Jr & Spittell P.C. Diseases of the peripheral arteries and veins. In Alpert J.S. (ed.) Cardiology for the Primary Care Physician, 3rd edn. By permission of Current Medicine, Inc, Philadelphia.)

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chronic venous obstruction that is of special interest to a cardiologist is the upper extremity where therapeutic devices (Figure 6.11) can be the cause. Probably the most common vascular disorder in the lower extremity is varicose veins. Common symptoms are swelling, aching, and cramps as well as cosmetic concerns, all of which, except the latter, can be relieved by

Figure 6.2 Plantaris muscle tear causing calf pain and ankle edema. Bleeding in the calf results in appearance of submalleolar ecchymosis with dependency. (a) Lateral view showing submalleolar ecchymosis. (b) Medical view showing submalleolar ecchymosis.

Figure 6.3 Popliteal vein obstruction by popliteal (Baker’s) cyst. (a) Right leg and ankle edema; note distended superficial veins. (b) Arthrogram demonstrating popliteal cyst. (c) Venogram showing displacement of popliteal vein by popliteal cyst.

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elevation of the legs and the use of elastic support when the patient is ambulatory. Injection therapy, or surgical treatment, is indicated when there are significant symptoms or complications, but before either of these a trial of regular use of elastic support (20–30 mm Hg at the ankle) is worthwhile. Also, since the coexistence of deep vein valvular incompetence is a relative contraindication to surgical treatment of varicose veins, the deep venous circulation should be evaluated in the vascular laboratory preoperatively if surgery is planned.

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Pulmonary embolism Like acute DVT (the source of most pulmonary emboli), the symptoms and signs of acute PE are variable and nonspecific. Since dyspnea is the most common presenting symptom of PE, it may be confused with, as well as accompanied by, congestive heart failure.9 In addition, the usual diagnostic tests – arterial blood gases, chest roentgenogram, and electrocardiogram – may be of little assistance in differential diagnosis. These clinical features make clinical awareness of the possibility of PE and prompt resort to diagnostic imaging techniques – ventilation perfusion lung scan, spiral CT scan with contrast, echocardiography,10 and pulmonary angiography – in likely settings essential if the poor diagnostic record for PE is to improve. Management depends on the promptness of diagnosis and severity of the hemodynamic consequences. In the case of major PE, time is of essence and echocardiography is ideal since it is portable and is capable of recognizing the characteristic features of PE and assessing right ventricular function.10,11 The finding of right heart thrombi in a patient hemodynamically compromised with acute pulmonary embolism indicates higher risk and may indicate the need for more aggressive therapy than heparin alone.12 In the management of acute major PE thrombolysis, provided there are no contraindications, is usually the treatment of choice with embolectomy – either catheter or surgical embolectomy reserved for patients in whom thrombolysis is contraindicated.11,13 Recently, a ‘‘contemporary’’ approach of acute pulmonary embolectomy, for patients with

Figure 6.4 Acute cellulitis in left lower extremity causing pain, fever, chills, and ankle edema. Note varicose veins in right lower extremity with normal ankle contour, i.e. no edema.

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extensive PE and liberalized criteria of moderate to severe right ventricular dysfunction despite preserved systemic arterial pressure, has been reported to provide 89% survival in 29 consecutive patients managed in this way.14 The question of inferior vena cava interruption may come up when the person with severe cardiopulmonary disease develops acute VTE. The usual indications for caval interruption are contraindication to, or failure of, anti-

Figure 6.5 Infrared photograph of right lower extremity showing edema and increased venous pattern. Cause is an arteriovenous fistula of iliac vessels created at time of subject’s lumbar disc surgery. Table 6.4 Differential diagnosis of acute vascular conditions in the lower extremity Acute arterial occlusion

Acute deep vein thrombosis

Acute lymphangitis and cellulitis

Edema Skin color Superficial venous pattern Arterial pulses Fever

Pale Normal

þ Normal or cyanotic Prominent

þ Red Normal

Absent Absent

Normal High (39.5–40.5 C)

Chills

Absent

Normal Mildly increased (less than 39 C) Absent

Clinical feature

Frequent

Venous disorders 85

coagulant therapy or prophylaxis when urgent surgery is indicated in the face of acute VTE.4 A vexing problem for all clinicians is the patient with recurring VTE (Table 6.5). While neoplasm is frequently suspected as the cause, it is important to first consider reversible causes such as medication (female hormones),

Venous Thromboembolism Clinically-Suspected DVT - No Sx for PE History and exam

Alternative diagnosis Appropriate treatment for Dx

Features still consistent Duplex or MRDTI

Pathognomic (phlegmasia) Treat

Positive

Positive Negative

? venogram Negative

Figure 6.6 Algorithm for the management of the patient with suspected acute DVT. MRDTI, magnetic resonance direct thrombus imaging.

Figure 6.7 Acute left axillary subclavian venous thrombosis. (From Kazmier F.J. & Juergens J.L. (1980) Venous thrombosis and obstructive disease of the veins. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)

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myeloproliferative disease, ulcerative bowel disease, thromboangiitis obliterans (TAO), and of course, the hypercoagulable states (Table 6.3). An appropriate evaluation in an undiagnosed case, after a history and physical exam, is outlined in Table 6.6. Pulmonary embolic disease also involves the cardiologist in the differential of pulmonary hypertension, one cause of which can be chronic pulmonary thromboembolism.15

Figure 6.8 Chronic indurated cellulitis (lipodermatosclerosis) of lower leg, medial aspect, in a patient with chronic deep venous insufficiency. (Reproduced from Spittell J.A. Jr & Spittell P.C. (2000) Peripheral Vascular Disease in ACCSAP. American College of Cardiology Foundation, Bethesda, MA. By permission of the American College of Cardiology.)

Figure 6.9 Bilateral venous stasis ulcerations. (From Spittell J.A. Jr (1998) Peripheral vascular disease. In Wenger N.K. (ed.) Cardiovascular Disease: Recognition in the Octogenarian and Beyond. London: Martin Dunitz Ltd. By permission of Martin Dunitz Ltd.)

Venous disorders 87

Figure 6.10 Iliac vein obstruction by sarcoma mimicking iliofemoral deep vein thrombosis (DVT) in a 62-year-old woman. (a) Duplex ultrasound scan. (b) CT scan of pelvis.

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Figure 6.11 Venogram showing an obstructive stenotic lesion in the superior vena cava resulting from two transvenous pacemaker placements.

Table 6.5 Recurrent venous thromboembolism Problems 1. 2. 3. 4.

Diagnosis Causes Extent of evaluation Anticoagulant therapy When? How long?

Table 6.6 Recurrent venous thromboembolism Evaluation if no evident cause 1. 2. 3. 4. 5. 6. 7.

Complete blood count Urinalysis Antinuclear antibody test Chest roentgenogram Sputum cytology (smokers) Sigmoidoscopic exam CT scan of abdomen and pelvis

Venous disorders 89

One additional point worth noting is the question of the use of postmenopausal therapy with estrogen plus progestin in women with coronary heart disease. Women who have a history of previous VTE, a hypercoagulable state, cancer, or lower extremity fracture should avoid postmenopausal hormone therapy because of the increased risk of VTE associated with its use.7 Another clinical problem that treatment of VTE and other cardiovascular diseases causes is heparin-induced thrombocytopenia (HIT).16 Of the two types of HIT, the more benign Type I usually occurs early (within 2–4 days of heparin exposure) and requires only close monitoring of the platelet count to be sure that thrombocytopenia is transient. The more severe Type II HIT occurs most often 5 days to 2 weeks after exposure to heparin therapy and is associated with thrombotic complications (Figure 6.12). The management of Type II HIT is prompt and complete cessation of heparin, and if continued

Figure 6.12 Arterial thrombosis and gangrenous change in Type II heparin-induced thrombocytopenia (HIT Type II). (a) Hand. (b) Foot – same patient as (a).

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antithrombotic therapy is indicated, the use of recombinant hirudin, is a safe and effective alternative.17 In patients with VTE the early administration of oral anticoagulant therapy can shorten the duration of heparin therapy and thus lessen the occurrence of Type II HIT. Another situation in which HIT can occur is in the recently hospitalized patient who had exposure to heparin and who returns after dismissal with an acute thrombotic (arterial or venous) complication. To avoid worsening the problem with heparin therapy, should such a patient represent ‘‘delayed heparin-induced thrombocytopenia,’’18 a platelet count should be checked before initiating antithrombotic therapy.

References 1 Geerts W.H., Heit J.A., Clagett G.P., et al. (2001) Prevention of venous thromboembolism. Chest 119: 132S–175S. 2 Thomas D.P. & Roberts H.R. (1997) Hypercoagulability in venous and arterial thrombosis. Ann Int Med 126: 638–644. 3 Fraser D.G., Moody A.R., Morgan P.S., et al. (2002) Diagnosis of lower limb deep venous thrombosis: A prospective blinded study of magnetic resonance direct thrombus imaging. Ann Int Med 136: 89–98. 4 Hyers T.H., Agnelli G., Hull R.D., et al. (2001) Antithrombotic therapy for venous thromboembolic disease. Chest 119: 176S–193S. 5 Kearon C. (2002) Commentary. ACP J Club 136: 10–11. 6 Bauer K.A. (2001) The thrombophilias: Well-defined risk factors with uncertain therapeutic implications. Ann Int Med 135: 367–373. 7 Grady D., Wenger N.K., Herrington D., et al. (2000) Postmenopausal hormone therapy increases the risk for venous thromboembolic disease. Ann Int Med 132: 689–696. 8 Mohr D.N., Silverstein M.D., Heit J.A., et al. (2000) The venous stasis syndrome after deep venous thrombosis or pulmonary embolism: A population-based study. Mayo Clin Proc 75: 1249–1256. 9 Belenkie I. (2000) The ten most commonly asked questions about the cardiac aspects of pulmonary embolism. Cardiol Rev 8: 79–81. 10 Goldhaber S.Z. (2002) Echocardiography in the management of pulmonary embolism. Ann Int Med 136: 691–700. 11 Wood K.E. (2002) Major pulmonary embolism: Review of a pathophysiologic approach to the golden hour of hemodynamically significant pulmonary embolism. Chest 121: 877–905. 12 Torbricki A., Galie´ N., Covezzli A. et al. (2003) Right heart thrombi in pulmonary embolism. Results from the International Cooperative Pulmonary Embolism Registry. JACC 41: 2245–2251. 13 Uflacker R. (2001) Interventional therapy for pulmonary embolism. J Vasc Interv Radiol 12: 147–164. 14 Aklog L., Williams C.S., Byrne J.G., et al. (2002) Acute pulmonary embolectomy: A contemporary approach. Circulation 105: 1416–1419. 15 Rich S., Levitsky S. & Brundage B.H. (1988) Pulmonary hypertension from chronic pulmonary thromboembolism. Ann Int Med 108: 425–434.

Venous disorders 91 16 Brieger D.B., Ma K.K., Kottke-Marchant K., et al. (1998) Heparin-induced thrombocytopenia. JACC 31: 1449–1459. 17 Greinacher A., Volpel H., Janssens U., et al. (1999) Recombinant hirudin (lepirudin) provides safe and effective anticoagulation in patients with heparin-induced thrombocytopenia: A prospective study. Circulation 99: 73–80. 18 Rice L., Attisha W.K., Drexler A., et al. (2002) Delayed onset heparin-induced thrombocytopenia. Ann Int Med 136: 210–215.

CHAPTER 7

Leg edema

While the differential diagnosis of leg edema is not a primary interest of most cardiovascular specialists, patients and some primary care providers often consult the cardiologist when edema occurs because they think first of cardiac disease as the possible cause. Unfortunately, the subject of leg edema does not receive much, if any, attention in medical school and many residency and cardiovascular training programs. This knowledge ‘‘gap’’ will hopefully be corrected by a review of a basic clinical approach to the patient with leg edema. Most cardiologists are comfortable in dealing with lower extremity edema of systemic origin (Table 7.1) and its management. The causes and clinical features of the various types of leg edema due to systemic disease are not the subject of this chapter except to note that all cause bilateral leg edema. However, two other types of bilateral leg edema – idiopathic edema1 (Table 7.2) and particularly drug-induced edema (Table 7.3) – need special mention. Idiopathic edema is not well understood and is often not recognized. These patients, virtually always women, complain of episodic leg edema and excessive weight gain during the day. They have oliguria during the day and nocturia at these times. Their management can be difficult and includes sodium restriction, diuretics, and sympathomimetic amines1. Drug-induced leg edema in this age of polypharmacy is very common, particularly in the elderly patient, and may be overlooked unless it is kept in mind; often some adjustments in medication will lessen this type of edema. Correctly categorizing leg edema into a systemic or regional type can usually be accomplished by the answers to eight questions: 1 When did the edema begin? 2 Is the swollen extremity painful? 3 Does the edema recede overnight? 4 Does the patient complain of dyspnea and/or orthopnea? 5 Is there evidence of renal disease? 6 Is there evidence of chronic hepatic disease? 7 Has there been any significant weight fluctuations or bowel changes? 8 Is the patient taking any medication or pills? In addition to systemic and regional types of leg edema, there is the gravitational type of leg edema that may be either bilateral or unilateral. This type of edema is readily recognized in patients whose disability causes prolonged sitting with their legs dependent. A vascular cause of this type of 92

Leg edema 93 Table 7.1 Systemic edema Features Bilateral Pitting Causes Cardiac Renal Hepatic Nutritional

Table 7.2 Idiopathic edema

. Women > men . Edema AM – face and hands PM – abdomen and lower extremities . Episodic excessive weight gain . Nocturia . Daytime oliguria

Table 7.3 Causes of drug-induced edema . . . .

NSAIDs* Antihypertensives Hormonal therapy Antidepressants

*nonsteroidal anti-inflammatory drug

edema is severe ischemia causing rest pain. To obtain relief from the pain, the affected limb is hung out of the bed for prolonged periods (Figure 1.4). Another type of edema that is unique in vascular practice is that which follows femoral or popliteal artery surgery. This is likely related to an interference with lymphatic drainage in the region of the incisions and is generally temporary, subsiding in a few days or weeks. A clinical point that many have forgotten, or perhaps not been taught, is that relatively minor degrees of leg edema are best seen at the back of the ankle with the patient standing (Figure 6.3). The regional types of leg edema – venous, lymphatic, and lipedema – are those which may be most difficult for the cardiologist to diagnose since they are infrequently seen and seldom discussed in cardiovascular training programs. The distinguishing features of the common types of regional leg edema can be conveniently shown in table form (Table 7.4). Venous and lymphatic edema can either be bilateral or unilateral while lipedema (and its associated orthostatic edema) is bilateral and symmetrical (Figure 7.1). Venous edema can involve the foot but not the toes while lymphedema involves both the foot and the toes.

94 Chapter 7 Table 7.4 Common regional types of leg edema Clinical feature Bilateral? Thickened skin? Stasis changes? Foot involved? Toes involved?

Venous

Lymphedema

Lipedema

+ þ þ

+ þ þ þ

þ

Figure 7.1 Lipedema (fat legs). Note sparing of the feet and toes.

A useful maneuver in differentiating lymphedema from other types of edema is to examine the texture of the skin, which in lymphedema is thickened. The examiner can compare the skin of a swollen limb with the skin on the back of his/her hand (Figure 7.2) which is normal skin texture. At times, however, longstanding venous and lymphedema are hard to confidently differentiate by physical examination and then the use of noninvasive vascular laboratory study and radionuclide lymphoscintigraphy can be most useful. Venous edema, whether acute (Figures 6.1 and 6.7) or chronic (Figures 2.13, 6.5, and 6.8), is characteristically soft, pitting readily, and is accompanied by an increased superficial venous pattern. The texture of the skin in venous edema is normal. The clinical impression can be confirmed by noninvasive vascular laboratory studies or duplex ultrasound, or by venography if there is an unresolved question of deep vein thrombosis (DVT).

Leg edema 95

Lymphedema is the type of regional edema that is most challenging diagnostically for many physicians. It can be primary or secondary (Table 7.5) and distinguishing one type from the other can also be clinically challenging (though sometimes quite easy, as discussed later in this chapter). Primary lymphedema, when not congenital (which is rare), is for practical clinical purposes lymphedema precox (Figure 7.3), affecting women nine to ten times more than men. Lymphedema precox begins before the age of 40 years, the onset most often around the time of the menarche or at the time of the first pregnancy. It becomes bilateral in about half the cases. The management of lymphedema precox, after preliminary edema reduction by pumping and massage (usually in a physical medicine facility) is the regular use of graded elastic support hose or garment with 40–50 mm Hg compression at the ankle whenever ambulatory. A point in convincing a young

Figure 7.2 Normal skin texture demonstrated on dorsum of examiner’s hand. Table 7.5 Lymphedema Clinical features . . . .

Firm edema Pits poorly Skin thickened Toes involved

Classification . Primary (idiopathic) Congenital Lymphedema precox . Secondary (acquired) Obstructive Postinflammatory

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Figure 7.3 Lymphedema precox of right lower extremity. Note edema of the foot and toes. (From Spittell J.A. Jr & Spittell P.C. (2000) Peripheral Vascular Disease in ACCSAP. American College of Cardiology Foundation, Bethesda, MA. By permission of the American College of Cardiology.)

woman to be compliant in using the elastic support is advising her that the most attractive part of a woman’s leg is the contour about the ankle and a near normal ankle contour in an elastic support is more attractive than a swollen ankle in regular stockings; failure to wear her stocking regularly will allow the edema to progress. A common clinical problem is confusing lymphedema precox with secondary obstructive lymphedema due to a neoplasm in the pelvis or abdomen. Obstructive lymphedema due to neoplasm generally has its onset after the age of 40, while lymphedema precox virtually always begins before the age of 40.2 When secondary lymphedema results from recurring infection, it can of course begin at any age. The practical clinical point of the differentiation of primary from secondary lymphedema is that an extensive search for an underlying neoplasm is not necessary in the case of lymphedema precox (onset before age 40) or in lymphedema secondary to recurring infections (most often associated with dermatophytosis or postmastectomy lymphedema). Obstructive lymphedema of a lower extremity, secondary to a neoplasm, is usually due to cancer of a pelvic organ (prostate, urinary bladder, ovary, or uterus) when not due to lymphoma.3 This type of lymphedema is typically painless and progressive and it may be the first clue to the underlying neoplasm.4 In fact, a useful clinical aphorism is that the occurrence of painless, progressive edema of the leg that does not resolve overnight in a man more than 60 years of age is cancer of the prostate until proven otherwise. Management of obstructive lymphedema of any type is the regular use of elastic hose or a garment with 40–50 mm Hg compression at the ankle, fitted

Leg edema 97

after preliminary edema reduction by elevation, massage, and pumping in addition to therapy directed at the underlying neoplasm. Secondary lymphedema can be due to a variety of infectious agents, but in clinical practice in the United States it is most often secondary to recurring acute lymphangitis (Figure 7.4) due to streptococcus. The two clinical settings in which this is most often seen are in cases secondary to dermatophytosis between the toes (Figure 7.5) and in postmastectomy lymphedema of the arm. In either type, the treatment is eradication of the portal of infection (e.g. treatment of the dermatophytosis) and long-term prophylactic antibiotic treatment with either penicillin (e.g. oral penicillin V potassium 500 mg q.i.d.), or for persons allergic to penicillin an erythromycin preparation or clarithromycin (in appropriate dosage) the first 7 days of every month.5 Failure to recognize and treat the infectious basis of this type of lymphedema can result in an elephantitic limb (Figure 7.6). In addition to the eight questions, several clinical points already mentioned deserve to be repeated and stressed in the evaluation of the patient with regional leg edema. 1 Checking the skin texture of the edematous limb. 2 Examining the patient while they are standing, looking at the posterior aspect of the legs as well as the anterior aspect. 3 Examining between the toes.

Figure 7.4 (a) Secondary lymphedema of right leg of a 74-year-old man due to recurring acute lymphangitis. (b) Lower legs and feet of patient in (a). Note edema of right foot.

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Figure 7.5 Interdigital fissure due to dermatophytosis, the portal of infection, in a 75-year-old man with lymphangitis of the leg. (From Spittell J.A. Jr & Spittell P.C. (2000) Peripheral Vascular Disease in ACCSAP. American College of Cardiology Foundation, Bethesda, MA. By permission of the American College of Cardiology.)

Figure 7.6 Elephantiasis resulting from untreated long-standing recurring lymphangitis due to untreated dermatophytosis.

Lipedema (fat legs, Figure 7.1) is not actually edema of the legs but patients with lipedema, usually women, often complain of a long-standing problem of the swelling of their legs. As a rule these patients have been conscious of their large legs and note their orthostatic edema after long sitting and premenstrually. The symmetrical enlargement of the legs, the normal size feet and toes, and the normal skin texture make recognition of lipedema easy. Management

Leg edema 99

of the patient with lipedema and orthostatic edema should emphasize the importance of weight control, the avoidance of salt, periodic leg elevation, and the use of comfortable support hosiery – patients with lipedema do not tolerate the discomfort of graded compression elastic stockings.

References 1 Streeter D.H.P. (1980) Understanding and treating idiopathic edema. Consultant March: 82–88. 2 Schirger A. & Peterson L.F.A. (1980) Lymphedema. In Juergens J.L., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co., pp. 823–851. 3 Smith R.D., Spittell J.A. Jr & Schirger A. (1963) Secondary lymphedema of the leg: Its characteristics and diagnostic implications. JAMA 185: 80–82. 4 Spittell J.A. Jr, Smith R.D., Harrison E.G. Jr, et al. (1963) Unilateral secondary lymphedema: A clue to malignant disease. Proc Staff Meet. Mayo Clin 38: 139–144. 5 Babb R.R., Spittell J.A. Jr, Martin W.J., et al. (1966) Prophylaxis of recurrent lymphangitis complicating lymphedema. JAMA 871–873.

CHAPTER 8

Leg and foot ulcers1

Ulceration of the leg, foot, or toes is not a primary cardiac problem but is the complication of one of several comorbid conditions – peripheral occlusive arterial disease, diabetes, neuropathy, hypertension, and chronic deep venous insufficiency – that commonly occur in adult persons. Their presence in a cardiovascular patient warrants attention and recognition in order to arrange proper management. The four commonly encountered types of leg and foot ulcers are ischemic arterial (Figures 1.6, 1.13 and 8.1), venous stasis (Figure 8.2), neurotrophic (Figure 8.3), and ischemic arteriolar (Figure 8.4). Their distinctive clinical features are listed in Table 8.1. Key clinical points in the differential diagnosis are the presence or absence of pain (ischemic ulcers are exquisitely painful while neurotrophic ulcers are painless) and location on the leg or foot. Table 8.1 can also be used to identify ulcerations that are not one of the four common types and worthy of referral to a dermatologist for identification and appropriate management.

Figure 8.1 Ischemic ulceration of the leg. (Reprinted with permission from Spittell J.A. Jr (1983) Diagnosis and management of leg ulcer. Geriatrics 38(6): 60. Copyright 2002 Advanstar Communications, Inc. Advanstar Communications, Inc retains all rights to this material.)

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Leg and foot ulcers 101

Figure 8.2 Venous stasis ulceration. (Reprinted with permission from Spittell J.A. Jr (1983) Diagnosis and management of leg ulcer. Geriatrics 38(6): 59. Copyright 2002 Advanstar Communications, Inc. Advanstar Communications, Inc retains all rights to this material.)

Figure 8.3 Neurotrophic ulcer of the foot.

The management of leg ulcers is not something that most cardiologists will undertake. However, the basic approach to the treatment of ischemic ulceration due to occlusive peripheral arterial disease or to arteriolar disease and venous stasis ulceration are covered in the chapters on arterial disease and venous disease respectively. It is important to remember that elevated ambulatory venous pressure, whatever its cause, can result in venous stasis ulceration. Thus, as seen in Figures 8.5a and b, congenital arteriovenous fistula can result in poorly healing venous stasis type ulceration. The management of this patient includes elevation of the limb, cleansing of the ulcer with local alternating saline and dry packs, and then skin grafting to effect healing

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Figure 8.4 (a) Cutaneous infarction of the leg in early stage of hypertensive ischemic (arteriolar) ulceration. (b) Later stage of hypertensive ischemic (arteriolar) ulceration. (Reprinted with permission from Spittell J.A. Jr (1983) Diagnosis and management of leg ulcer. Geriatrics 38(6): 65. Copyright 2002 Advanstar Communications, Inc. Advanstar Communications, Inc retains all rights to this material.)

Table 8.1 Leg ulcer – differential diagnosis

Stasis

Ischemic (arterial)

Neurotrophic

Onset Course Pain? Location

Trauma þ Chronic þ with infection Medial leg

Trauma Progressive Severe Toe, heel, foot

Spontaneous Progressive Absent Plantar

Skin Edges Base Arterial Venous Lymphatic

Stasis change Shaggy Healthy Normal Abnormal Normal

Atrophic Discrete Eschar; pale Abnormal Normal Normal

Callous Discrete Healthy or pale Normal or abnormal Normal Normal

Ischemic (arteriolar) Spontaneous Progressive Severe Lateral and posterior leg Normal Serpiginous Eschar; ischemic Normal Normal Normal

From Spittell J.A. Jr (1983) Diagnosis and management of leg ulcers. Geriatrics 38: 6. Copyright 2002 Advanstar Communications, Inc. Advanstar Communications, Inc retains all rights to this material. Reprinted with permission.


Figure 8.5 (a) ‘‘Hot’’ venous ulceration of foot. (b) Angiogram demonstrating congenital arteriovenous fistula of patient whose foot is shown in (a). (c) Healed state of patient in (a).

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Figure 8.6 Type of shoe created to control elevated venous pressure in foot of patient with congenital arteriovenous fistula of the foot (Figure 8.5).

Figure 8.7 Neurotrophic ulceration of medial side of foot in patient with Charcot joint causing weight to be borne on side of foot.

(Figure 8.5c) but the long-term healing requires the regular use of special footwear to control the elevated venous pressure (Figure 8.6). Neurotrophic ulceration, most common in the patient with diabetic neuropathy, also deserves some additional comment. As seen in Figure 8.3, these ulcers are commonly on the sole of the foot in a callus overlying the head of a metatarsal bone. However, neurotrophic ulceration should be remembered as occurring on any weight-bearing portion of the limb and always in a callus (Figure 8.7). Their recognition and referral to a podiatrist, or orthopedist, to


Figure 8.8 Ulcerations of foot in patient taking hydroxyurea as treatment for thrombocytosis.

facilitate healing and adjust footwear, or make other adjustments in weight bearing, is essential to prevent future ulcerations. Clinicians who care for patients with a myeloproliferative disorder, in addition to their cardiac disease, should be aware of one uncommon type of leg ulcer that is induced by hydroxyurea (Figure 8.8).2,3 Replacement of the hydroxyurea by another chemotherapeutic agent and local dressings will effect healing.

References 1 Spittell J.A. Jr (1983) Diagnosis and management of leg ulcer. Geriatrics 38: 57–65. 2 Roenigk H.H. Jr & Young J.R. (1991) Leg ulcers. In Young J.R., Graor R.A., Olin J.W., et al. (eds) Peripheral Vascular Diseases, 1st edn. St. Louis: Mosby Year Book, pp. 605–638. 3 Best P.J., Daoud M.S., Pittelkow M.R., et al. (1998) Hydroxyurea-induced leg ulceration in 14 patients. Ann Int Med 128: 29–32.

CHAPTER 9

Vascular clues to a diagnosis

Few things are more satisfying to clinicians than making an observation that provides a reliable ‘‘short cut’’ to the diagnosis of an important (sometimes even an occult) condition, which indeed at times is a clue to a cardiovascular disorder. The vascular system by virtue of its universal distribution may serve as almost as good an indication of underlying pathology as the skin itself. Without providing an exhaustive consideration of vascular clues, presentation of some vascular findings, evident on a cardiovascular examination, that can be diagnostic clues is the goal of this chapter. Like some other basic clinical skills, examination of the ocular fundus is frequently not included in a cardiovascular examination despite it being a unique opportunity to view arteries and veins. There are a number of classic findings and clues evident on ophthalmoscopic examination that can suggest or even clinch a diagnosis. A canoe-shaped para-arterial hemorrhagic lesion (Figure 9.1), the Roth spot, though rare, can support a clinical impression of

Figure 9.1 Roth spot in fundus of patient with infective endocarditis.

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Vascular clues to a diagnosis 107

infective endocarditis in a likely setting just like other nonspecific but suggestive clinical findings – a splinter hemorrhage in the nail bed of a finger or toe (Figure 9.2), Osler nodes in a finger, toe, or foot (Figure 9.3), or Janeway spots in the skin of the palm or soles (Figure 9.4). As noted, these findings are not specific for infective endocarditis but supportive when the clinical setting is appropriate for infective endocarditis. Another suggestive finding in the ocular fundus is very tortuous arterioles (Figure 9.5) that can be seen in some patients with coarctation of the aorta. When the fundus finding suggests coarctation, the diagnosis can be confirmed by noting the typical ‘‘delay’’ in the femoral artery pulsation compared to the radial artery pulsation (Figure 1.10). More common than the former ocular fundus findings is the cholesterol plaque (Figure 1.20) indicative of atherosclerosis of the ascending, or arch portions, of the

Figure 9.2 Sublingual splinter hemorrhages. (a) Finger. (b) Toe.

Figure 9.3 Osler’s nodes in skin of foot.

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Figure 9.4 Janeway lesions on sole of foot.

Figure 9.5 Tortuous retinal arterioles that can be seen in some patients with coarctation of the aorta.


thoracic aorta or of the ipsilateral carotid artery – the significance of this finding is the increased frequency of stroke seen in these patients compared to those with normal ocular fundi (Table 9.1). Two important hematologic disorders – polycythemia vera (Figure 9.6) and macroglobulinemia (Figure 9.7) – also have readily recognized changes in the ocular fundus. The rare heritable disorder of elastic tissue, pseudoxanthoma elasticum, in addition to the frequently associated occlusive peripheral, coronary, and cerebral arterial disease, has a typical ocular fundus finding of angioid streaks (Figure 9.8) as well as its yellowish confluent cutaneous papular lesions (like the skin of a ‘‘plucked’’ chicken) in the face, neck, axilla, and abdomen. The examination of the hand and fingers should include attention to the skin where telangiectatic spots may be the ‘‘give away’’ to scleroderma Table 9.1 Retinal cholesterol emboli (asymptomatic) outcome (mean follow-up of 3.4 years)

Patients Controls*

No.

No. of strokes

70 70

17 2

No. of ipsilateral carotid 12

*Controls matched for sex, age, " BP, diabetes, cholesterol, smoking, CAD. From Bruno A., Jones W.L., Austin J.K., et al. (1995) Vascular outcome in men with asymptomatic retinal cholesteral emboli. Ann Int Med 122: 249–253.

Figure 9.6 Engorged retinal veins in patient with polycythemia vera.

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Figure 9.7 ‘‘Box caring’’ or ‘‘link sausage’’ appearance of retinal veins plus hemorrhages and exudates in fundus of patient with macroglobulinemia.

Figure 9.8 Pseudoxanthoma elasticum. (a) Angioid streaks in the retina. (b) ‘‘Plucked chicken’’ appearance of the skin in the axilla.

(Figure 4.6) or to Osler–Weber–Rendu disease (Figure 9.9a), the only two causes of telangiectasia of the fingers. Recognizing Osler–Weber–Rendu disease (which may not present with epistaxis) is important because of the occurrence of pulmonary arteriovenous fistula (Figure 9.9b & c), in



Figure 9.9 Osler–Weber–Rendu disease. (a) Telangiectasia of skin of the thumb. (b) Chest x-rays of patient in (a) showing what proved on (c) pulmonary angiography to be a pulmonary arteriovenous fistula in the right lower lobe.

5–6% of these persons, which can be single or multiple. There is a familial incidence of this association in about 15%.1 The possibility of pulmonary hemorrhage or brain abscess as complications of pulmonary arteriovenous fistula warrants their treatment by selective embolization or surgical removal, which should be conservative because of the likelihood of additional fistulas developing in patients with Osler–Weber–Rendu disease. While on the subject of clues seen in the hand, some nonvascular clues include the well–known arachnodactyly and the clinically useful ‘‘thumb sign’’ (Figure 9.10) seen in the Marfan syndrome, as well as various other abnormalities in the

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hand and upper extremity – from minor thumb abnormalities to congenital absence of the hand – seen in the Holt–Oram syndrome with its congenital cardiac abnormalities, the most common being septal defects.


Figure 9.10 (a) Arachnodactyly in a patient with the Marfan syndrome. (b) Positive ‘‘thumb sign’’ in a patient with the Marfan syndrome.

The peripheral venous system provides a number of clues other than the well-known spontaneous and unexplained venous thromboembolism (VTE) that may signal various malignancies. As noted earlier, recurring superficial thrombophlebitis (Figure 1.15a, p. 18) is one of the clinical features of thromboangiitis obliterans (TAO) (Buerger’s disease) and may occasionally be the presenting symptom in a young smoker with the disease. Spontaneous thrombophlebitis, particularly with a family history of venous thrombosis, also raises the possibility of a hereditary hypercoagulable disorder (Table 6.3, p. 80), the most common of which are the Leiden mutation of the gene for factor V and the prothrombin G20210 A abnormality.2 Edema due to proximal venous compression by an expanding lesion can be a valuable clue to an important, otherwise asymptomatic, mass such as aneurysm (Figure 2.13, p. 44). Venous

114 Chapter 9

stars (Figure 9.11) (in chronic deep venous obstruction which can mimic deep vein thrombosis (DVT) except for the usual absence of pain) can be significant when they suddenly appear in the upper arm or upper anterior chest and indicate occlusion or obstruction of a major mediastinal vein.3 Another clue to compression of a major mediastinal vein is nonpulsatile distention of a jugular vein (Figure 2.3, p. 33). One other location where venous compression readily occurs is the popliteal space where either a popliteal aneurysm or a Baker’s cyst (Figure 6.3, p. 81) can obstruct the popliteal vein. Cardiologists are very aware of venous pulsation in the jugular veins but they need to remember that other

Figure 9.11 Venous ‘‘stars’’ on anterior chest and right arm in a patient with chronic superior vena cava obstruction.

Figure 9.12 Hands of a 42-year-old man who noted pain in his right hand for 7–8 months and a pulsatile mass in the midpalm and dorsum of his right hand for 3 months. He experienced a 10–15-pound weight loss as well. Urogram and later surgery revealed a grade 3 hypernephroma.


peripheral veins can be pulsatile (e.g. pulsating varicose veins4) due to tricuspid valve insufficiency or in the case of an acquired arteriovenous fistula (Figure 6.5, p. 84). Congenital arteriovenous fistula can result in the manifestation of elevation venous pressure – edema and stasis changes including skin ulceration – as a ‘‘hot ulcer’’ (Figure 8.5, p. 103). Arterial clues are generally well known and utilized by cardiologists as they relate the quality and timing of carotid artery pulsation to cardiac valvular disease and to myocardial contraction. A cardiac source, or even infective endocarditis, are usually the first considerations in patients with embolic peripheral arterial occlusion, but two other important conditions to keep in mind are ergotism (Figure 1.19, p. 24) and acute aortic dissection (Figure 1.18, p. 22), in either of which the acute peripheral arterial occlusion may be the presenting manifestation and a clue to the correct diagnosis. At times the diagnosis of a peripheral arterial aneurysm can be mimicked by a pulsating tumor metastasis (usually from hypernephroma (Figure 9.12) or a thyroid cancer) or by ‘‘buckling’’ of an atherosclerotic innominate, or carotid, artery (Figure 1.21, p. 26) that produces a prominent right carotid artery pulsation in the neck suggesting aneurysm. The latter can usually be clarified without needing ultrasound or arteriogram by having the patient, while sitting, take a deep breath, thus lowering the heart and ‘‘unbuckling’’ the buckled artery with disappearance of the prominent pulsation in the right side of the neck. The blue toe syndrome, while used by some as a descriptor for atheroembolism (Figure 2.6, p. 38), can actually be a clue to a number of other important conditions (Table 9.2), e.g. polycythemia vera (Figure 9.13). Table 9.2 Causes of blue toe(s)

Thrombotic arterial occlusion Arteriosclerosis obliterans Thromboangiitis obliterans Hypercoagulable states Myeloproliferative disorders Heritable coagulation disorders Heparin-induced thrombocytopenia Connective tissue disorders Trauma Embolic arterial occlusion Cardiac origin Atherosclerotic aorta* Aneurysmal disease* Aorta Femoropopliteal artery Vasospastic disorders Raynaud’s phenomenon Acrocyanosis Chronic pernio Cyanotic congenital heart disease *May occur with initiation of anticoagulant therapy or spontaneously Modified from Spittell J.A. Jr & Spittell P.C. (1992) Chronic pernio; another cause of blue toes. Int Angiol 11: 46–50.

116 Chapter 9

Figure 9.13 Blue toes in a man with polycythemia vera.

As mentioned earlier in Chapter 7, the onset of painless progressive swelling of a lower extremity in a patient more than 40 years of age is a clue that should bring to mind the possibility of a pelvic neoplasm or lymphoma causing obstructive lymphedema, if there is no supporting evidence for infection being the cause of the lymphedema.

References 1 Hodgson C.H. & Kaye R.L. (1963) Pulmonary arteriovenous fistula and hereditary telangiectasia: A review and report of 35 cases of fistula. Dis Chest 43: 449–455. 2 Bruno A., Jones W.L., Austin J.K., et al. (1995) Vascular outcome in men with asymptomatic retinal cholesterol emboli. Ann Int Med 122: 249–253. 3 Fred H.L., Castle C.H. & Cancilla P.A. (1962) Venous stars in mediastinal disease. AMA Arch Int Med 109: 290–296. 4 Brickner P.N., Scudder W.T. & Wernrib M. (1962) Pulsating varicose veins in functional tricuspid insufficiency. Circulation 25: 126–129.

CHAPTER 10

Some uncommon peripheral vascular disorders

There are several rather uncommon peripheral vascular disorders with which the informed cardiovascular specialist should be familiar – particularly when queried about one of these disorders by their patients or by a referring colleague.

Thoracic outlet compression Compression of the subclavian vessels in the thoracic outlet most often occurs in the costoclavicular space between the uppermost rib (first rib or cervical rib) and the clavicle (Figure 10.1). This is a difficult area of clinical practice because the symptoms can be so variable (Table 10.1) and natural history data are lacking. With performance of the thoracic outlet maneuvers (Figures 10.2, 10.3, and 10.4), compression of the subclavian artery can be demonstrated by

Anterior scalenus median Brachial plexus

Cla

vicl

e

1st

A

V

rib

Compression

Figure 10.1 Costoclavicular syndrome. Compression of the subclavian vessels very commonly occurs between the clavicle and the uppermost (first and/or cervical) rib. (From Fairbairn J.F. II, Campbell K. & Payne W.S. (1980) Neurovascular compression syndromes of the thoracic outlet. In Juergens J.F., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)

117

118 Chapter 10 Table 10.1 Thoracic outlet compression syndrome Symptoms None Palpable mass in neck (cervical rib) Raynaud’s phenomenon Arm ‘‘claudication’’ Ischemic ulceration of finger(s) Acute arterial occlusion in arm or hand Axillary subclavian deep vein thrombosis Vertebral-basilar transient cerebral ischemia

b

a Figure 10.2 (a) Costoclavicular maneuver, active. (Performed by patient forcibly throwing shoulders back.) Auscultation over subclavian artery, above or below midportion of clavicle, may reveal systolic bruit as artery is compressed. Radial pulse and bruit over subclavian artery disappear when compete compression of subclavian artery occurs. (b) Costoclavicular maneuver, passive. (Examiner raises arm and moves it posteriorly.) (From Fairbairn J.F. II, Campbell K. & Payne W.S. (1980) Neurovascular compression syndromes of the thoracic outlet. In Juergens J.F., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)

noting a change in the amplitude of the ipsilateral radial artery. A positive exercise can be documented by having the patient perform the maneuver during duplex ultrasound scanning of the subclavian artery. When there have been arterial complications in the ipsilateral hand, arteriography (Figure 10.5) is useful to both confirm the diagnosis and to identify any site of injury

Some uncommon peripheral vascular disorders 119

Figure 10.3 Hyperabduction maneuver. Axillary artery may be completely or incompletely compressed by maneuver. In latter case, bruit may be heard above or below clavicle or, on occasion, deep in the axilla. (From Fairbairn J.F. II, Campbell K. & Payne W.S. (1980) Neurovascular compression syndromes of the thoracic outlet. In Juergens J.F., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)

Figure 10.4 Scalene or Adson maneuver. This test is used in both cervical rib or anomalous first thoracic rib syndrome and scalenus anticus syndrome. (Patient tilts head to side and back; holds breath and performs a Valsalva maneuver while leaving the examined arm relaxed.) Auscultation over subclavian artery being tested may reveal bruit when artery is partially compressed. (From Fairbairn J.F. II, Campbell K. & Payne W.S. (1980) Neurovascular compression syndromes of the thoracic outlet. In Juergens J.F., Spittell J.A. Jr & Fairbairn J.F. II (eds) Peripheral Vascular Diseases, 5th edn. Philadelphia: W.B. Saunders Co. By permission of Mayo Foundation.)

to the subclavian artery that would warrant its repair at the time of definitive relief of the compression of the artery, i.e. removal of the uppermost rib. Before definitive therapy, careful correlation of symptoms and findings and exclusion of other causes of upper extremity symptoms (e.g. carpal tunnel syndrome and cervical disc disease) is essential. In addition to the arterial complications in the upper extremity, a rare complication of thoracic outlet compression of the subclavian artery is

120 Chapter 10

a

b Figure 10.5 (a) Ischemic ulceration of fingers of a 55-year-old woman with thoracic outlet compression of the left subclavian artery by a cervical rib. (Reproduced from Spittell J.A. Jr (1994) Peripheral arterial disease. Disease-a-Month 40: 641–704. By permission of Mosby, Inc.) (b) Arch aortogram demonstrating poststenotic dilatation of the left subclavian artery distal to its compression in the thoracic outlet. (c) Arteriogram of the left hand showing occlusions of multiple digital arteries.

vertebral-basilar ischemia as a result of proximal propagation of a thrombus in the subclavian artery to involve the vertebral artery. Compression of the subclavian vein in the thoracic outlet is probably the chief cause of what formerly was known as ‘‘effort thrombosis’’ of the axillary


C Figure 10.5 continued

a Figure 10.6 Venograms in a case of axillary subclavian vein thrombosis that occurred after vigorous weight lifting in a 30-year-old man. (a) Before thrombolysis. (b) After thrombolysis.

subclavian vein. In fact, it likely takes both factors – unusual effort of the arm and compression (and injury) of the subclavian vein in the thoracic outlet – to cause the venous thrombosis. Unusual home carpentry overhead, unusual throwing, and strenuous weight lifting are precipitating types of ‘‘effort’’ that are often seen as the cause in such cases. The treatment, after appropriate remediation of the deep vein thrombosis (DVT) (Figure 10.6), is removal of the uppermost rib if the compression of the subclavian vein in the thoracic outlet can be confirmed by maneuvers during ultrasound examination.

122 Chapter 10

b Figure 10.6 continued

Occlusive arterial disease in the hand due to blunt trauma1 The arterial circulation in the palm of the hand is rather superficial and when the hand is exposed to blunt trauma the arteries and palmar arch are easily compressed against the bones of the hand. Thus, with severe or repetitive blunt trauma to the palm, occlusive arterial disease in the hand sometimes results. In persons with various types of occupations or hobbies (Table 10.2), this type of occlusive arterial disease may be the cause of symptoms varying from none (evident only with a positive Allen test) to Raynaud’s phenomenon, a digital arterial occlusion (Figure 10.7), or even ischemic ulceration of a finger (Figure 10.8). Usually the problem is in the dominant hand unless the repetitive blunt trauma results from an occupation requiring the nondominant hand to be exposed to the trauma (e.g. chain saw operator). This type of occlusive arterial disease in the hand may be overlooked unless both the radial and ulnar artery pulsations and the Allen test are included in the examination of each patient. Arteriography (Figure 10.8) is usually needed to confirm the diagnosis (particularly if worker’s compensation is an issue) and determine if microvascular surgery is possible. Management of occlusive arterial disease in the hand needs to be directed at preventing further trauma to the hand along with the other measures for all types of occlusive arterial disease – interdiction of tobacco and avoidance of drugs with vasoconstriction in their side-effect profile. If Raynaud’s phenomenon is a problem to the patient, an alpha-adrenergic blocker or a

Some uncommon peripheral vascular disorders 123 Table 10.2 Some occupations and activities associated with arterial disease of the hands resulting from repetitive blunt trauma Occupation

Cause of trauma

Baseball catcher Butcher Carpenter Cash register operation Creamery worker Dentist Farmer

Repetitive impact of ball to the catcher’s hand Use of hand tools Use of hand tools, air hammer; ‘‘hammer hand’’ Use of hand as hammer on register Use of hand as hammer to loosen milk can lids Compressive trauma from gripping dental tools Use of hand tools, tractor steering wheel, gear shift handle Use of hand tools Use of hand as hammer on moulds Use of hand tools Hand trauma from gait-assistive devices Repetitive blunt palm trauma Striking of wrenches with palm Striking of wrenches, other tools with palm Trauma to palm of hand by outlet forceps Hand trauma from tools; ‘‘hammer hand’’ Use of palm to loosen bottle caps Striking of wrenches with palm Jamming of hand against throttle Trauma from unusual head of cane Trauma from stapler handle Striking of wrenches with palm of hand Repeated trauma to palm from levers, valves, steering wheel

Forestry worker Foundry worker Gardener Handicapped person Jackhammer operator Machinist/millwright Mechanic Obstetrician Packing house worker Pharmacist Plumber Railroad engineer Rhabdophile Secretary/office worker Steamfitter Truck driver

From Spittell P.C. & Spittell J.A. (1993) Occlusive arterial disease of the hand due to repetitive blunt trauma: A review with illustrative cases. Int J Cardiol 38: 282. Used by permission.

calcium-channel blocker can be prescribed. In the patient with ischemic ulceration of a finger, if the above measures do not effect healing, and if microvascular surgery is not feasible, ipsilateral sympathectomy to produce long-term vasodilatation to the hand is indicated.

Erythermalgia When a patient complains of burning feet or hands, many clinicians think first of this rare disorder without remembering that the clinical description includes ‘‘red and hot’’ in addition to ‘‘burning.’’ Without the three features, it is possible that one of the other causes of burning feet (Table 10.3) is the problem. Given a patient with the suspicion of erythermalgia, the diagnosis can be confirmed by the simple vascular laboratory procedure of determining the skin temperature of the toes (or fingers) and the knee (or elbow) when the patient is symptomatic. Normally, the skin temperature of the knee (or elbow) exceeds that of the toes (or fingers), but in true erythermalgia the reverse is the case when the patient is symptomatic (Table 10.4).

124 Chapter 10

Erythermalgia may also be secondary as in the case of an 83-year-old man who developed polycythemia vera and burning of the hands and fingers, partially relieved by phenacetin but totally relieved by treatment with hydroxyurea.

a

b Figure 10.7 Occlusive arterial disease in the hand due to trauma from a hobby. (a) (b) Hand of a 60-year-old man who collects walking sticks (a rhabdophile). Note discoloration of tip of fifth finger and the positive Allen test. (c) Arteriogram demonstrating occlusion of the ulnar artery and superficial palmar arch as well as the impaired arterial circulation to the fourth and fifth fingers. (d) The offending walking stick.


d

c Figure 10.7 continued

Treatment of the primary form is very difficult since few patients experience relief with (the usual textbook recommendation) aspirin. Some patients get relief with propranolol or atenolol. Treatment of the underlying disorder causing secondary erythermalgia may give complete relief as in the case described.

Mesenteric vascular disease Like other uncommon types of vascular disease, mesenteric vascular disease, either arterial or venous, needs to be deliberated upon in likely clinical a

Figrue 10.8 An ischemic ulcer of one of the fingers of the right hand of a right-handed 34-year-old heavy equipment mechanic who frequently uses his right hand as a hammer in his work. (a) Ischemic ulceration of the finger. (b) Arteriogram showing narrowed ulnar artery and occlusion of the ulnar portion of the superficial palmar arch. (From Spittell J.A. Jr & Spittell P.C. (2001) Diseases of peripheral arteries and veins. In Alpert JS (ed.) Cardiology for the Primary Care Physician, 3rd edn. Philadelphia: Current Medicine, Inc. Used by permission.)

126 Chapter 10

b Figure 10.8 continued

Table 10.3 Burning feet Erythermalgia – ‘‘red, hot, burning feet’’ Primary Nonfamilial Familial Secondary Myeloproliferative disorders Diabetes Venous insufficiency Connective tissue disorders Peripheral neuropathy – ‘‘burning feet’’ Ischemic – painful, red, cold burning feet Arteriosclerosis obliterans Thromboangiitis obliterans Sensory neuropathy Hereditary Diabetes Post-traumatic

settings. The key symptom, of course, is abdominal pain in all types of mesenteric vascular disease. Occlusion of the major mesenteric arteries may be acute (usually embolic from a cardiac source) or chronic in the patient with extensive atherosclerotic occlusive arterial disease. The abdominal pain in the acute type is sudden in onset and severe in the patient with congestive failure and/or atrial fibrillation. In the chronic type of major mesenteric arterial occlusion, the abdominal pain (‘‘intestinal angina’’) occurs after eating and often leads to weight loss as

Some uncommon peripheral vascular disorders 127 Table 10.4 Skin temperature studies in a 43-year-old woman with intermittent red, hot, burning feet and hands relieved by cold water – primary erythermalgia Skin temp. ( C) 11:00

4:00

AM

PM

Symptoms

None

Feet red and hot

Toes

R

L

R

L

1 2 3 4 5 Ankle Patella

27.1 26.5 26.7 27.9 27.8 35.6 32.8

26.7 26.3 26.2 26.3 26.5 34.5 32.2

36.7 35.8 36.6 36.3 35.9 35.3 35.3

37.8 37.3 37.7 37.5 37.0 36.9 34.9

Table 10.5 Less common causes of mesenteric arterial occlusive disease

Thromboangiitis obliterans Arteritides Acute aortic dissection Iatrogenic – after abdominal aortic surgery Hypercoagulable states Fibromuscular dysplasia Drugs . Vasopressors . Digitalis . Oral contraceptives

the patient avoids eating to avoid the pain. There are a number of less common causes of occlusive disease of the mesenteric arteries (Table 10.5) to keep in mind. When either type of major mesenteric arterial occlusion is suspected, arteriography is indicated to confirm the diagnosis. Treatment of the acute type of major mesenteric arterial occlusion is prompt restoration of arterial flow to prevent bowel infarction; if infarction of the bowel has occurred, the affected bowel needs to be resected. In the chronic type of major mesenteric arterial occlusion, restoration of pulsatile flow either interventionally or surgically is indicated.3 Occlusion of the small mesenteric arteries or the mesenteric veins can occur in giant-cell arteritis, connective tissue diseases, hypercoagulable disorders, and with certain medications (Table 10.5). In the types of disorders listed, the occurrence of unexplained abdominal pain should bring this possibility into consideration. Treatment is that of the underlying disorder and anticoagulant therapy in the hypercoagulable disorders, provided that intestinal infarction (which requires surgical resection) has not occurred. When mesenteric

128 Chapter 10

ischemia is due to, or aggravated by, medication, replacing the suspected medication is indicated.

Congenital arteriovenous malformation A very diverse group of lesions, varying from simple hemangioma and the dysplastic angiopathies2 to congenital arteriovenous fistulas, are included under this rubric. The cardiologist is likely to be consulted only in respect to congenital arteriovenous fistula, which can occur in almost every organ of the body but they are most evident when they occur in the extremities (more often in the lower extremity). Clues to a congenital arteriovenous fistula in an extremity are the presence of a birthmark (Figure 10.9), particularly when it is associated with increased size or length of the extremity, and/or varicose veins in the extremity. The usual complaints of the patient are cosmetic. It is extremely rare for congestive heart failure to result from a congenital arteriovenous fistula in adults though it has been reported in the newborn.4 The reason to include congenital arteriovenous fistulas of the extremity in this presentation is to caution against considering surgical or interventional treatment unless it is absolutely necessary. Even though the lesion appears to be localized on arteriography, the arteriovenous communications are usually so numerous and extensive that complete excision is not possible and more extensive recurrence is likely if surgical removal or embolization is attempted. Conservative treatment is best, if feasible. Consideration of surgical, or interventional treatment, of congenital arteriovenous malformations in various organs or the face and neck needs to be

Figure 10.9 Hemangioma of the first and second toes.


determined on an individual basis, again recognizing the potential for recurrence of these lesions.

Fabry disease Unfortunately, the diagnosis of Fabry disease is often missed, but its early diagnosis is important because of the availability of enzyme replacement therapy.5 Fabry disease (angiokeratoma corporis diffusum), a sex-linked recessive storage disorder, may come to the cardiologist’s attention because affected persons may develop cardiovascular, and cerebrovascular, disorders due to accumulation of the lipid ceramide trihexoside in the vascular endothelium in the heart, brain, and kidney. The purplish cutaneous lesions (Figure 10.10) usually occur in groups on the lower trunk, upper portion of the lower extremities, and genitalia. Fabry disease should not be confused with the angiokeratoma of the scrotal skin of elderly men. Cardiac involvement in Fabry disease is frequent. Clinical manifestations vary from those of coronary artery disease to valvular disease to unexplained cardiomyopathy. Involvement of cerebral vessels may lead to transient cerebral ischemia or stroke. The later stage of Fabry disease occurs in adults and is characterized by hypertension and progressive renal failure. If Fabry disease is suspected, there is an enzyme test (a-Gal A enzyme level) that is available for diagnosis.5

Figure 10.10 Deep red–purple papules in the skin of the crural fold of a 46-year-old man with Fabry disease. (From Pittlekow R.B., Kierland R.R. & Montgomery H. (1956) Angiokeratomoa corporis diffusum. AMA Arch Derm 72: 556–561. Copyright 1955. By permission of American Medical Association.)

130 Chapter 10

References 1 Spittell P.C. & Spittell J.A. Jr (1993) Occlusive arterial disease of the hand due to repetitive blunt trauma: A review with illustrative cases. Int J Cardiol 38: 282–292. 2 Bean W.A. (1958) Vascular Spiders and Related Lesions of the Skin. Springfield, IL: Charles C. Thomas. 3 Barkhordarian S. & Gusberg R. (2003) Mesenteric ischemia: Identification and treatment. ACC Curr J Rev 12: 19–21. 4 Murray D.E., Myerowitz B.R. & Hutter J.J. (1969) Congenital arteriovenous fistula causing congestive heart failure in the newborn. JAMA 209: 770–771. 5 Desnick R.J., Brady R., Barranger J., et al. (2003) Fabry disease, an under-recognized multisystem disorder: Expert recommendations for diagnosis, management and enzyme replacement therapy. Ann Int Med 138: 338–346.

Index

Note: Page numbers in italic refer to tables and/or figures separate from the text. abdominal pain 126, 127 ABI 1, 4–8 acrocyanosis 72–3 acute arterial occlusion 18–21, 24 Adson maneuver 119 Allen test 11, 13, 122 amantadine hydrochloride 72 aneurysm 30, 113 abdominal aortic 34, 36–42 complications 30, 31 femoral artery 43–6 iliac artery 42–3 infected (mycotic) 40, 41, 42 peripheral arterial 115 popliteal artery 43–6, 114 renal artery 65, 66 thoracic aorta 30–2, 33–4, 35, 36 upper extremity arterial 46, 47 angina, intestinal 126–7 angiography, occlusive peripheral arterial disease 8 angioid streaks, retina 109, 110 angiokeratoma, scrotal 129 angiokeratoma corporis diffusum 129 ankle–brachial index 1, 4–8 anticoagulant therapy complications 89–90 deep vein thrombosis 77–8 hypercoagulable states 78 anuria 39, 40 aorta abdominal, aneurysm 34, 36–42 acute dissection 21, 22–3, 115 atherosclerotic ulcer 57–9 coarctation 11, 12, 50, 107, 108 comparison of acute thoracic disorders 58 dissection 50–7, 58 intramural hematoma 57–9 thoracic, aneurysm 30–2, 33–4, 35, 36 traumatic laceration 31–2, 35, 36 traumatic pseudoaneurysm 32 aortic valve, congenital anomalies 50 arachnodactyly 111, 113 arm see upper extremity arteries, peripheral occlusive disease 1–29 arteriovenous fistula congenital 100, 103–4, 128–9 lower extremity 77, 84, 101, 103–4, 115

pulmonary 110–11, 112 arteritis 61–7 ASO see atherosclerotic occlusion aspirin 14, 23 atheroembolism 72, 73 and abdominal aortic aneurysm 37–9 atherosclerotic occlusion (ASO) 1–2 chronic lower extremity 2–9, 7, 11, 12–14 chronic upper extremity 9–10, 9–11, 12, 12, 13 and coronary artery disease 1, 14 and diabetes mellitus 5, 14 management 14, 15–17 natural history and prognosis 11–14, 15 Baker’s (popliteal) cyst 81–2, 114 birthmark 128 blue toe syndrome 37, 38, 39, 115, 116 bruits carotid 21, 23 in occlusive arterial disease 1, 2 Buerger’s disease (thromboangiitis obliterans) 11, 17, 18, 69, 86, 113 carotid artery angioplasty 23 buckling 26, 27, 115 disease 21–7 duplex ultrasonography 23 endarterectomy 23 examination in occlusive arterial disease 1–2 percutaneous stenting 24 pulsation 115 cellulitis 78, 83, 84, 86 cholesterol embolism, retinal artery 21, 25, 107–9, 108 chronic pernio 73–5 cilastazol 15 clopidogrel 14, 23 cocaine abuse 50 common iliac artery, aneurysm 42–3 computed tomography abdominal aortic aneurysm 34, 36 aortic dissection 52, 54 congestive heart failure 77, 83 coronary artery disease and atherosclerotic occlusion 1, 14

131

132 Index costoclavicular maneuver 118 costoclavicular syndrome 117 cranial (giant cell) arteritis 14, 15, 61, 62, 64 deep vein thrombosis 77–82, 84, 85 dependent rubor 4, 5 diabetes mellitus and atherosclerotic occlusion 5, 14 and neurotrophic ulcers 100, 101, 104 diagnosis, vascular clues 106–16 dorsal pedis pulse 1 drug-induced leg edema 92, 93 duplex ultrasound carotid artery 23 deep vein thrombosis 77 DVT 77–82, 84, 85 dyspnea 83 echocardiography acute arterial occlusion 18, 20 and aortic aneurysm 30, 34 aortic dissection 52, 53, 55–6 pulmonary embolism 83 edema, lower extremity 92–9, 116 Ehlers–Danlos syndrome, type IV 50 elephantiasis 97, 98 elevation-dependency test 3–4, 5 embolectomy, pulmonary embolism 83–4 ergotism 21, 24, 115 erythermalgia 73, 123–5, 126, 127 exercise study, intermittent claudication 2, 3 Fabry disease 129 factor V, Leiden mutation 113 femoral artery aneurysm 43–6 palpation 11, 12 pseudoaneurysm 30, 32 foot see lower extremity footcare in atherosclerotic occlusion 14, 15, 16 footwear in atherosclerotic occlusion 14, 16 in congenital arteriovenous fistula of the foot 100, 104 gangrene 16, 43, 61, 66 giant cell arteritis 14, 15, 61, 62, 64 hand see upper extremity hemangioma 128 heparin-induced thrombocytopenia 89–90 hip pain 8 hirudin 90 HIT 89–90 Holt–Oram syndrome 112 hormone replacement therapy 89 hydronephrosis and abdominal aortic aneurysm 39, 40

hydroxyurea 105, 105 hyperabduction maneuver 119 hypercoagulable states 77, 78, 80, 86, 113 hypernephroma 114, 115 hypertension and aortic dissection 50 and thoracic aortic aneurysm 31 hypogastric artery, occlusive disease 8 idiopathic medial aortopathy (Takayasu’s arteritis) 61–2, 65 iliac artery see common iliac artery; internal iliac artery iliac vein obstruction 80, 87 IMH 57–9 infective aortitis 40, 41, 42 infective endocarditis 40, 41, 42, 106, 107, 115 inferior vena cava, interruption in pulmonary embolism 84 innominate artery, buckling 26, 27, 115 intermittent claudication 1, 2, 3, 8, 11, 14, 15 intermittent compression pump treatment 17 internal iliac artery, occlusive disease 8 internal mammary artery 10, 14 intestinal angina 126–7 Janeway lesion 107, 108 jugular vein distention 33, 114 pulsation 114 kidney, horseshoe 39 leg see lower extremity Leiden mutation, factor V 113 lipedema, lower extremity 93, 94, 98–9 livedo reticularis 37, 38, 71–2 lower extremity arteriovenous fistula 77, 84, 100, 103, 115 cellulitis 78, 83, 84, 86 chronic atherosclerotic occlusion 2–9, 11, 14 chronic venous obstruction 80, 87 differential diagnosis of acute vascular conditions 84 edema 92–9, 116 lipedema 93, 94, 98–9 lymphoedema 93–4, 95–7, 98, 116 ulcers 100–5, 115 venous stasis ulceration 78, 86 lymphangitis 97, 98 lymphedema lower extremity 93–4, 95–7, 98, 116 obstructive 96–7, 116 lymphedema precox 95–6

Index 133 macroglobulinema 109, 110 magnetic resonance angiography (MRA) carotid artery disease 23 iliac artery aneurysm 42 occlusive peripheral arterial disease 8, 9 popliteal artery entrapment 17, 19 magnetic resonance direct thrombus imaging 77 magnetic resonance imaging, aortic dissection 52, 54, 55 Marfan syndrome 111, 113 and aortic dissection 50 and thoracic aortic aneurysm 31 medial calcinosis 5 mesenteric vascular disease 125–8 MRA see magnetic resonance angiography MRDTI 77 myxedema 69 occlusive peripheral artery disease 1–29 occupation and arterial disease of the hands 122, 123 ocular fundus examination 21, 25, 106, 106, 108, 109, 110 Osler’s nodes 107, 107 Osler–Weber–Rendu disease 110–11, 111–12 PE 77, 83–90 pentoxifylline 15 periarteritis nodosa 63, 65, 66 pernio, chronic 73–5 pernio syndrome 74 phlegmasia cerulea dolens 77, 80 plantaris muscle, tear 81 polycythemia vera 109, 109, 115, 116, 124 popliteal artery aneurysm 43–6, 111 entrapment 17, 19 popliteal (Baker’s) cyst 81–2, 114 posterior tibial artery 1 posterior tibial pulse 1 postmenopausal hormone therapy 89 postphlebitic venous insufficiency 77, 78, 85, 86 prothrombin G20210 A abnormality 113 pseudoaneurysm femoral artery 30, 32 traumatic, thoracic aorta 32 pseudoclaudication 2, 3, 8 pseudoxanthoma elasticum 109, 110–11 pulmonary embolism 77, 83–90 pulmonary hypertension 86 pulseless disease (Takayasu’s arteritis) 61–2, 65 radial artery palpation 9, 9, 12 Raynaud’s phenomenon/disease 66, 68–9, 70, 71, 122 reflex sympathetic dystrophy 75 renal arteriography 39

renal artery aneurysm 65, 66 stenosis 27 retina, angioid streaks 109, 110 retinal artery, cholesterol embolism 21, 25, 107–9, 108 Roth spot 106–7, 106 scalene maneuver 119 scleroderma 65–6, 69, 109 scrotal angiokeratoma 129 skin, ‘plucked chicken’ 109, 111 SLE 61, 65–6 smoker’s lesion 9, 10 spine, lumbar stenosis 2 splinter hemorrhage 107, 107 stroke and carotid artery disease 23 and retinal cholesterol embolism 108, 109 subclavian artery atherosclerotic occlusion 9, 9–10, 14 compression 117–18, 118, 120–1 subclavian steal 9–10 subclavian vein thrombosis 117–21, 121–2 superior vena cava obstruction 88 surgery, risk of thromboembolism following 77, 79 systemic lupus erythematosus 61, 65–6 Takayasu’s arteritis 63, 65 TAO 11, 17, 18, 69, 86, 113 telangiectasia, hands and fingers 66, 109, 111 temporal (giant cell) arteritis 14, 15, 62, 63, 64 thoracic outlet compression 117–21, 119, 120–1 thoracic outlet maneuvers 117, 119 thromboangiitis obliterans 11, 17, 18, 69, 86, 113 thrombocytopenia, heparin-induced 89–90 thrombolytic therapy deep vein thrombosis 77–8 pulmonary embolism 83 thumb sign 111, 113 TIA 21–3, 25 transcutaneous oximetry 5, 7 transient ischaemic attack 21–3, 25 trauma, occlusive arterial disease in the hand due to 122, 123, 124–5 Turner syndrome 50 ulcerative bowel disease 86 ulcers atherosclerotic aortic 57–9 ischemic 5, 7, 16, 71, 100, 100, 122, 125 ischemic arteriolar 100, 102 lower extremity 100–5, 115 neurotrophic 100, 101, 104 venous stasis 100, 101

134 Index ultrasound abdominal aortic aneurysm 34, 36 popliteal aneurysm 45 upper extremity arterial aneurysm 46, 47 chronic atherosclerotic occlusion 9–11, 9–10, 12, 13, 14 chronic venous obstruction 80–1, 88 post-traumatic occlusive arterial disease in the hand 122, 123, 124–5 varicose veins 81–2 pulsation 115

vasospastic disorders 68–76 venous disorders 77–91 venous stars 114, 114 venous stasis ulceration, lower extremity 78, 86 venous thromboembolism 77–82, 84, 85, 113 vertebral–basilar ischemia 119–20 VTE 77–82, 84, 85, 113 walking and intermittent claudication 15 weight lifting 50

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Mechanisms of Vascular Disease: A Textbook for Vascular Surgeons

Peripheral Vascular Stenting: Second Edition

Vascular embolotherapy: a comprehensive approach

Pulmonary Vascular Disease

Retinal Vascular Disease

Pulmonary Vascular Disease

Fifty Cases of Peripheral Vascular Interventions

Complex Cases in Peripheral Vascular Interventions

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