Journal of Cardiology Cases (2010)

Journal of Cardiology Cases (2010) 1, e1—e5

available at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/jccase

Case Report

The lurking potential of tangential forces: A case of an arteriovenous shunt developed by percutaneous coronary intervention for the septal branch Hidetoshi Hashida (MD) ∗, Jun-ichi Funada (MD), Norikatsu Morioka (MD), Takeru Iwata (MD) Department of Cardiovascular Medicine, National Hospital Organization Ehime National Hospital, 366 Yokogawara, Toon, Ehime Prefecture 791-0203, Japan Received 30 January 2009; received in revised form 1 May 2009; accepted 18 May 2009

KEYWORDS Angioplasty; Perforation; Shunt; Size mismatch

Summary We describe the case of a 59-year-old male. His first percutaneous coronary intervention (PCI) using a bare metal stent was performed for a 90% stenosis in the mid portion of the left anterior descending artery (LAD). However, we performed re-PCI because in-stent restenosis developed during a chronic stage. After the first dilatation of the restenotic lesion, using a cutting balloon, the stenosis at the ostium of the septal branch, which takes off from the stent strut, became exacerbated. Therefore, after selective guidewire insertion to the septal branch, we performed balloon inflation. Unfortunately, a coronary dissection and perforation developed in the septal branch and a coronary arteriovenous shunt was also formed. Additional inflation for in-stent restenosis with a perfusion balloon provided successful occlusion of the ostium of the septal branch and the shunt flow disappeared. After careful re-selection of a guide wire into the septal branch, the perforated portion was then dilated using a small-sized conventional balloon. Finally, reperfusion of the septal branch was accomplished without any angiographic sign of coronary dissection, perforation or shunt. We herein report a rare case of coronary arteriovenous shunt formation due to the dissection and perforation of a coronary artery. © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.

Introduction Although a perforation of a coronary artery rarely develops during percutaneous coronary intervention (PCI), it can sometimes be fatal due to critical complications such as

∗

Corresponding author. E-mail address: [email protected] (H. Hashida).

cardiac tamponade [1]. We herein present a rare case of a coronary arteriovenous shunt associated with a coronary perforation during PCI.

Case report A 59-year-old male with symptoms of chest pain during exertion and drinking was admitted to our hospital. His coronary risk factors were smoking and diabetes mellitus. Coronary

1878-5409/$ — see front matter © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.jccase.2009.05.002

e2

H. Hashida et al.

Figure 1 Coronary angiogram revealed in-stent restenosis in mid portion of left anterior descending artery (indicated by the arrow).

Figure 2 The ostium of the septal branch was dilated after the guidewire selection into the septal branch through the stent strut.

angiography revealed a significant stenosis in the left circumflex artery (LCx) and the mid portion of the left anterior descending artery (LAD). After the first PCI for the LCx, the second PCI for a 90% stenosis in the LAD was successfully performed with implantation of bare metal stents (DuraflexTM 3.0/8-mm and 3.0/25-mm, Goodman, Nagoya, Japan). A follow-up coronary angiography performed 6 months later, however, revealed a significant restenosis in the proximal stent site (Fig. 1) and a third PCI was performed for that lesion. Pre-procedure medications included 100 mg/day of aspirin and 200 mg/day of cilostazol. After the sheath insertion, 5000 units of heparin was intravenously administered, followed by continuous infusion at 400 units/h. A right radial artery approach was taken using a 5F-guiding catheter (Heartrail JL3.5TM , Terumo, Tokyo, Japan). After two guide wires (Runthrough NSTM , Terumo) were placed in the septal branch diverging from the stent placement and the 2nd diagonal branch, a third guide wire (WhisperTM , Guidant, Santa Clara, CA, USA) was selected for the distal LAD. Then, the first dilatation of the culprit lesion was attempted using a cutting balloon (Cutting BalloonTM Ultra 2.5/10-mm, Boston Scientific, Maple Grove, MN, USA). However, the balloon did not advance to the stenosed lesion. After the removal of the two side branch guide wires, the balloon was then successfully advanced to the stenosed lesion and the lesion was dilated using the cutting balloon with nominal pressure. The stenosis at the ostium of the septal branch became exacerbated after dilatation, so the guide wire (Runthrough NSTM ) was again advanced through the stent strut and the septal branch was dilated using a conventional balloon (Maverick 2TM 2.25/20-mm, Boston Scientific) (Fig. 2). After 12 atm of balloon inflation was attempted due to indentation, which remained at the septal branch, the coronary flow suddenly became disrupted at the middle of the septal branch (Fig. 3). The angiogram performed after the removal of guide wires showed an expanded septal branch associated with the harsh blood flow running parallel to the LAD toward the

cardiac base (Fig. 4). These angiographic findings showed the complication of an arteriovenous shunt due to septal dilatation. First of all, we administered 18 mg of protamine sulfate intravenously. Next, in order to occlude the ostium of the septal branch, a perfusion balloon (ACS Rx EspritTM , 2.5/20-mm, Guidant) was inflated at the proximal stent site for approximately 5 min. Thereafter, the septal branch was occluded from the ostium and the abnormal blood flow disappeared (Fig. 5). In addition, to ensure the closure of the perforated portion in the middle of the septal branch, which was possibly related to the formation of an arteriovenous

Figure 3 Coronary angiogram, immediately after the dilatation of the septal branch, showed the septal branch dissection with the disruption of blood flow from the same portion (indicated by the arrow).

A coronary arteriovenous shunt

e3

Figure 4 Serial images of a coronary angiogram (1 and 2) revealed the novel blood flow (black arrow) from the dissected portion of the septal branch (white arrow) to the left anterior descending artery. This flow followed a course into the vessel running parallel to the left anterior descending artery toward the proximal portion (3 and 4; arrow heads).

Figure 5 Coronary angiogram after the dilatation using the perfusion balloon revealed successful occlusion of the septal branch and disappearance of shunt flow (indicated by the arrow).

Figure 6 Final angiogram revealed successful reperfusion of the septal branch without coronary dissection, perforation and/or shunt (indicated by the arrow).

e4

H. Hashida et al.

shunt, additional dilatation (at 4 atm) was performed using a small-sized balloon (Maverick 2TM , 1.5/20-mm) after a careful guide wire (Runthrough NSTM ) re-selection to the septal branch. Finally, the reperfusion of the septal branch was accomplished without any angiographic sign of either coronary dissection, perforation, or arteriovenous shunt (Fig. 6).

Discussion A coronary artery perforation is a relatively rare complication and the rate of occurrence is approximately 0.5%, according to previous reports [1—7]. With regard to classifications of coronary perforation, the classification reported by Ellis et al. has been widely used [1]. Namely, the perforations are divided into types 1, 2, and 3 and the existence of cavity filling, depending on the angiographic findings. Our case was classified as type 3 with cavity spilling. This group is reported to be very rare, comprising only 0.02% of all PCI patients and 5% of all perforation cases according to the report by Witzke et al. [2] or 0.01% of all cases that have undergone PCI and 2% of all cases of perforation according to the report by Fasseas et al. [3]. On the other hand, in Japan, Shirakabe et al. demonstrated that it accounts for 0.35% of all PCI cases and 17% of all perforations [6]. The main cause of coronary perforation is thought to be related to wire manipulation [2] during procedures. However, in this case, because we had no difficulty advancing the guide wire to the septal branch and noting that the perforation formed immediately after the balloon inflation in the septal branch, we believe that it is unlikely that guide wire manipulation was the cause. Regarding cases of perforation due to balloon dilatation, the main cause is reported to be the discrepancy between arterial diameter and balloon size [8]. The ratio of the balloon diameter to vascular diameter was significantly greater in the group with perforation than in the non-perforation group [1,9]. In this case, the balloon edge was carelessly advanced to a small septal branch and it caused a noticeable size difference. With regard to forming an arteriovenous shunt, simultaneous perforation occurring both in the artery and the vein was considered to be essential. Regarding the mechanism of the simultaneous perforation, the first possibility was the artery and the vein were penetrated concurrently with a pointed tip of a guidewire [3,6]. However, perforation by a guide wire was considered to be unlikely in our case since guide wire manipulation in the septal branch was technically simple. In addition, neither a shunt flow nor any extravasation of contrast medium was detected in a test shot after guide wire placement. In this case, therefore we may suggest another possibility of shunt formation: if the artery and the vein are anatomically located very closely together, the surplus tangential force, for instance, by an inappropriate balloon/vessel ratio, which was 1.76 in our case, can cause the simultaneous perforation of artery and adjacent vein wall. This microscopic section including the septal branch from autopsy heart after paraffin fixation and hematoxylin—eosin staining shows the septal branch and the vein running parallel and very close together (Fig. 7). With regard to the initial treatment, we believe that the administration of protamine to neutralize the effect of heparin was appropriate [8]. In order to close the shunt

Figure 7 A microscopic section including the septal branch (indicated by the arrow) from autopsy heart: the vein (arrowhead) is located closely to the septal branch.

flow, balloon dilatation of the LAD with a perfusion balloon to occlude the ostium of the septal branch might be the simplest option, but the possibility of recanalization could not be fully ruled out. Recent reports have indicated the effectiveness of a covered stent for the treatment of coronary perforation [10]. Therefore, the implantation of a covered stent to cover the ostium of the septal branch for the purpose of occluding the shunt flow could be a possible strategy. Moreover, when the covered stent is deployed, while reperfusion for the branches would be virtually impossible, the occlusion of the 2nd diagonal branch from the ostium, resulting in the occurrence of myocardial infarction, becomes inevitable due to the anatomical relationship in our case. The clinical course in the cavity spilling group of class 3 was reported to be good [1,7]. Shirakabe et al. [6] have reported that only balloon inflation or neutralization of heparin was performed for successful closure. Therefore, the balloon inflation of the perforated lesion should have been attempted first, but advancing the guide wire into the perforated septal branch was considered to involve the risk of exacerbating the perforation. As a result, a prudent guide wire manipulation into the perforated septal branch was essential. The direct closure of the perforated vessel using thrombin [11], adipose tissue [12], Gelfoam [13], coils [14], polyvinyl alcohol [15], and clots [16] have all been reported as possible strategies for coronary artery perforation. However, these procedures were generally used for perforation of a peripheral coronary artery caused by a guide wire and are not suitable for the treatment of the intermediate portion of relatively large vessels such as in this case. In our case, although only the ostium of the septal branch should have been dilated, the balloon was carelessly advanced into the small branch of the septal as well, thereby creating a size mismatch and thus causing a perforation and arteriovenous shunt. This case serves as a reminder that careless minor procedural mistakes can cause a serious complication.

A coronary arteriovenous shunt

References [1] Ellis SG, Ajluni S, Arnold AZ, Popma JJ, Bittl JA, Eigler NL, Cowley MJ, Raymond RE, Safian RD, Whitlow PL. Increased coronary perforation in the new device era. Incidence, classification, management, and outcome. Circulation 1994;90: 2725—30. [2] Witzke CF, Martin-Herrero F, Clarke SC, Pomerantzev E, Palacios IF. The changing pattern of coronary perforation during percutaneous coronary intervention in the new device era. J Invasive Cardiol 2004;16:257—301. [3] Fasseas P, Orford JL, Panetta CJ, Bell MR, Denktas AE, Lennon RJ, Holmes DR, Berger PB. Incidence, correlates, management, and clinical outcome of coronary perforation: analysis of 16,298 procedures. Am Heart J 2004;147:140—5. [4] Javaid A, Buch AN, Satler LF, Kent KM, Suddath WO, Lindsay Jr J, Pichard AD, Waksman R. Management and outcomes of coronary artery perforation during percutaneous coronary intervention. Am J Cardiol 2006;98:911—4. [5] Ramana RK, Arab D, Joyal D, Steen L, Cho L, Lewis B, Liu J, Loeb H, Leya F. Coronary artery perforation during percutaneous coronary intervention: incidence and outcomes in the new interventional era. J Invasive Cardiol 2005;17: 603—5. [6] Shirakabe A, Takano H, Nakamura S, Kikuchi A, Sasaki A, Yamamoto E, Kawashima S, Takagi G, Fujita N, Aoki S, Asai K, Yoshikawa M, Kato K, Yamamoto T, Takayama M, et al. Coronary perforation during percutaneous coronary intervention. Int Heart J 2007;48:1—9. [7] Dippel EJ, Kereiakes DJ, Tramuta DA, Broderick TM, Shimshak TM, Roth EM, Hattemer CR, Runyon JP, Whang DD, Schneider JF, Abbottsmith CW. Coronary perforation during percutaneous coronary intervention in the era of abciximab platelet glycoprotein IIb/IIIa blockade: an algorithm for percutaneous management. Catheter Cardiovasc Interv 2001;52: 279—86.

e5 [8] Rogers JH, Lasala JM. Coronary artery dissection and perforation complicating percutaneous coronary intervention. J Invasive Cardiol 2004;16:493—9. [9] Ajluni SC, Glazier S, Blankenship L, O’Neill WW, Safian RD. Perforations after percutaneous coronary interventions: clinical, angiographic, and therapeutic observations. Cathet Cardiovasc Diagn 1994;32:206—12. [10] Lansky AJ, Yang YM, Khan Y, Costa RA, Pietras C, Tsuchiya Y, Cristea E, Collins M, Mehran R, Dangas GD, Moses JW, Leon MB, Stone GW. Treatment of coronary artery perforations complicating percutaneous coronary intervention with a polytetrafluoroethylene-covered stent graft. Am J Cardiol 2006;98:370—4. [11] Jamali AH, Lee MS, Makkar RR. Coronary perforation after percutaneous coronary intervention successfully treated with local thrombin injection. J Invasive Cardiol 2006;18:E143—5. [12] Suzuki H, Maeno K, Nishikawa H. Novel Method to stop bleeding by using subcutaneous tissue embolization for guide wire induced coronary artery perforation. Jpn J Interv Cardiol 2007;22:444—50. [13] Dixon SR, Webster MW, Ormiston JA, Wattie WJ, Hammett CJ. Gelfoam embolization of a distal coronary artery guidewire perforation. Catheter Cardiovasc Interv 2000;49:214—7. [14] Gaxiola E, Browne KF. Coronary artery perforation repair using microcoil embolization. Cathet Cardiovasc Diagn 1998;43:474—6. [15] Yoo BS, Yoon J, Lee SH, Kim JY, Lee HH, Ko JY, Lee BK, Hwang SO, Choe KH. Guidewire-induced coronary artery perforation treated with transcatheter injection of polyvinyl alcohol form. Catheter Cardiovasc Interv 2001;52:231—4. [16] Tanaka S, Nishigaki K, Ojio S, Yasuda S, Okubo M, Yamaki T, Kubota T, Takasugi N, Ishihara Y, Kawasaki M, Minatoguchi S. Transcatheter embolization by autologous blood clot is useful management for small side branch perforation due to percutaneous coronary intervention guide wire. J Cardiol 2008;52:285—9.

Journal of Cardiology Cases (2010) 1, e6—e8

available at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/jccase

Case Report

Congenital anomaly of the true double-lumen right coronary artery: An extremely rare case Shin Eun Lee (MD) a, Myung Ho Jeong (MD, PhD, FACC, FAHA, FESC, FSCAI, FAPSIC) b,∗, Jong Pil Park (MD) a, Sung Hee John (MD) a, Ji Hyun Lim (MD) a, Jay Young Rhew (MD) a a

Department of Cardiology, Presbyterian Medical Center, Jeonju, South Korea Cardiovascular Research Institute of Chonnam National University, The Heart Center of Chonnam National University Hospital, 671 Jaebongro, Donggu, Gwangju, South Korea

b

Received 31 March 2009; received in revised form 30 April 2009; accepted 28 May 2009

KEYWORDS Coronary artery; Right coronary artery; Congenital anomaly

Summary Double right coronary artery is a rare anomaly which is mostly identified by two separate pathways with a common ostium (one-two way). We report herein an extremely rare case of congenital true double-lumen right coronary artery, an anomaly, where a common pathway from the ostium to the proximal segment diverged into two separate pathways from the proximal to the mid-segment and then converged into one pathway (one-two-one way). © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.

Introduction The incidence of coronary artery anomalies varies from 0.95 to 2.00% in the adult population undergoing coronary angiography (CAG) [1—3]. The majority of these are reported to be anomalies of origin or distribution, with the occurrence of a separate ostium of the left anterior descending artery and left circumflex artery being the most common. However, double right coronary artery (RCA) is a rare anomaly which is mostly formed by two separate pathways with a common ostium (one-two way) or a common pathway with a separate ostium (two-one way) [4,5]. We present an extremely

∗ Corresponding author. Tel.: +82 62 220 6243; fax: +82 62 227 3105. E-mail address: [email protected] (M.H. Jeong).

rare case of congenital anomaly of the true double-lumen RCA where a common ostium separated into two pathways from the proximal to the mid-segment and then became a common pathway (one-two-one way).

Case report A 36-year-old man presented with exertional chest pain from 6 months ago. An electrocardiogram showed no ST-T abnormalities. He had no medical history of diabetes mellitus, hypertension, chest trauma, cigarette smoking, or family history of coronary artery disease. His lipid profile results were: total cholesterol, 187 mg/dL; triglycerides, 56 mg/dL; HDL cholesterol, 57 mg/dL; and LDL cholesterol, 119 mg/dL. A treadmill exercise test was performed on modified Bruce protocol, but terminated due to typical chest pain at 10 METs (15 min) without ST-T changes. The CAG showed no

1878-5409/$ — see front matter © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.jccase.2009.05.003

True double-lumen RCA

e7

Figure 1 The right coronary artery (RCA) angiography demonstrated mild stenosis and suggested a double-lumen from the proximal to the mid-RCA which developed from a common ostium. It is important to note that the true double-lumen has side branches arising from each lumen. One lumen’s side branchs are shown by two dotted arrows and the other is show by a straight arrow. (A) Right anterior oblique view; (B) left anterior oblique view.

Figure 2 Two guide wires were successfully inserted into the two different paths to identify the true double-lumen (A). The guide wires ran separate pathways at the proximal segment and then met at the mid-segment (B). The white line represents a schematic diagram of the true double-lumen.

significant stenosis in the left coronary artery. The RCA was cannulated with 5 Fr. Judkins right diagnostic catheter. The RCA angiography demonstrated mild stenosis and suggested a double-lumen from the proximal to the mid-RCA, which originated from a common ostium (Fig. 1). Two guide wires were inserted into the two different paths to identify the true double-lumen. Two guide wires ran separate pathways at the proximal segment and then became one at the mid-segment (Fig. 2). After the CAG, the patient received medications uneventfully during a 5-year clinical follow-up. We performed a coronary computed tomography (CT) angiography for a follow-up of the RCA lesion (Fig. 3).

Discussion A double RCA is a very rare anomaly with only a few cases being reported in the past. Most previously reported cases

were anomalies where a common ostium diverged into two separate pathways (one-two way). In one reported case, the separate pathways occurred due to separate ostia and then became one path at the mid-segment (two-one way) [4,5]. This is the first case in the literature of RCA anomalies where a common ostium diverges into two separate pathways from the proximal to the mid-segment and converges back to a common pathway (one-two-one way). The present case was initially considered a spontaneous or catheterinduced dissection. However, spontaneous dissections of the coronary artery mainly occur in young women with oral contraceptives or during the peripartum period [6—8]. Catheter-induced dissections usually begin at the catheter tip. In this case, the patient had no history of spontaneous severe chest pain or blunt chest trauma. The double-lumen began at the proximal segment of the RCA, not the catheter tip or the ostium. As well, there was a noticeable gap in between the double-lumen which was very tortuous. To con-

e8

S.E. Lee et al.

Figure 3 Volume-rendered images, without the myocardium, of the 64-slice computed tomography. The left coronary artery (LCA) showed no significant stenosis. The right coronary artery (RCA) appears to begin with one lumen (A of mid-inserts, arrow). It separates clearly into double-lumen (B and C of mid-inserts, double arrows), and then becomes one lumen again (D of mid-inserts, arrow).

firm if this case was a true double-lumen anomaly, two guide wires were inserted into the two different paths from the proximal to the mid-segment of the RCA. The guide wires ran separate pathways at the proximal segment and then converged at the mid-segment. The appearance of the patient’s RCA was similar to a previously reported case that had a dissection and healing post percutaneous transluminal coronary angioplasty [9]. However, in the present case, the patient had no history of coronary intervention. Also, double-lumens were long and more importantly, had side branches arising from each of them. The presence of the congenital true double-lumen RCA was conclusively confirmed and this was successfully managed with medical therapy.

References [1] Roberts WC. Major anomalies of coronary arterial origin seen in adulthood. Am Heart J 1986;111:941—63. [2] Greenburg MA, Fish BG, Spindola-Franco H. Congenital anomalies of coronary arteries: classification and significance. Radiol Clin North Am 1989;27:1127—46.

[3] Harikrishnan S, Jacob SP, Tharakan J, Titus T, Kumar VK, Bhat A, Sivasankaran S, Bimal F, Moorthy KM, Kumar RP. Congenital coronary anomalies of origin and distribution in adults: a coronary arteriographic study. Indian Heart J 2002;54: 271—5. [4] Rohit M, Bagga S, Talwar KK. Double right coronary artery with acute inferior wall myocardial infarction. J Invasive Cardiol 2008;20:E37—40. [5] Timurkaynak T, Ciftci H, Cengel A. Double right coronary artery with atherosclerosis: a rare coronary artery anomaly. J Invasive Cardiol 2002;14:337—9. [6] Dhawan R, Singh G, Fesniak H. Spontaneous coronary artery dissection: the clinical spectrum. Angiology 2002;53: 89—93. [7] Evangelou D, Letsas KP, Korantzopoulos P, Antonellis I, Sioras E, Kardaras F. Spontaneous coronary artery dissection associated with oral contraceptive use: a case report and review of the literature. Int J Cardiol 2006;112:380—2. [8] Ge JB, Huang ZY, Liu XB, Qian JY. Spontaneous coronary dissection associated with myocardial bridge causing acute myocardial infarction. Chin Med J 2008;121:2450—3. [9] Hsu PF, Lu TM, Leu HB, Chan WL. Double-barrel coronary artery dissection. J Chin Med Assoc 2005;68:383—5.

Journal of Cardiology Cases (2010) 1, e9—e11

available at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/jccase

Case Report

A case of right atrial tumor diagnosed by cardiac computed tomography Takuhiro Okuyama (MD) a, Shota Fukuda (MD, PhD) b,∗, Shoichi Ehara (MD, PhD) a, Daisuke Kaku (MD) b, Kenei Shimada (MD, PhD, FJCC) b, Junichi Yoshikawa (MD, PhD, FJCC) b, Minoru Yoshiyama (MD, PhD, FJCC) a a

Department of Internal Medicine and Cardiology, Osaka City University Graduate School of Medicine, 1-4-3 Asahi-machi, Abeno-ku, Osaka 545-8585, Japan b Department of Medicine, Cardiovascular Division, Osaka Ekisaikai Hospital, 2-1-10 Honden, Nishi-ku, Osaka 550-0022, Japan Received 30 January 2009; received in revised form 28 May 2009; accepted 28 May 2009

KEYWORDS Computed tomography; Echocardiography; Transesophageal; Ultrasonic diagnosis

Summary A 60-year-old woman was admitted with chief complaints of dyspnea for the previous 3 weeks. Transthoracic echocardiography (TTE) showed the amount of pericardial effusion and signs of cardiac tamponade. However, a tumor was not detected by TTE in any view. Multidetector computed tomography (MDCT) showed that the tumor protruded into the right atrium, invading to the outside of the heart. Transesophageal echocardiography (TEE) also showed the mobile tumor in the right atrium. In conclusion, this report may demonstrate that MDCT and TEE are required as complementary methods in the detection of cardiac tumors. © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.

Introduction Transthoracic echocardiography (TTE) is recognized as the primary imaging modality for the diagnosis of cardiac tumors [1]. However, previous studies have reported the difficulty of identifying right atrial (RA) tumors by TTE [2,3]. We present a case in which RA tumor failed to be detected by TTE. The tumor was detected by recently developed ECG-gated

∗ Corresponding author. Tel.: +81 6 6581 2881; fax: +81 6 6584 1807. E-mail address: [email protected] (S. Fukuda).

64-slice multidetector computed tomography (MDCT) and transesophageal echocardiography (TEE).

Case report A 60-year-old woman was admitted with chief complaints of dyspnea for the previous 3 weeks. At physical examination, a heart rate of 110 bpm and arterial systolic blood pressure of 90 mmHg with pulsus paradoxus were found. Chest radiography showed increased heart size, and electrocardiography presented sinus rhythm and a low voltage complex. The value of CA-125 was 1366 U/ml in the blood examination. TTE showed the amount of pericardial effusion and signs

1878-5409/$ — see front matter © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.jccase.2009.05.001

e10

T. Okuyama et al.

Figure 1 TTE images after pericardial drainage. The tumor was not detected in apical 4 chamber (left), parasternal short axis (middle), and RV inflow views (right). Middle picture is modified parasternal short-axis view at the level of aortic valve, tilting and sliding to visualize RA and RV. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle; TTE, transthoracic echocardiography.

Figure 2 MDCT images. The tumor (arrows) invaded to the outside of the heart (left), and protruded into the RA (right). Ao, aorta; LA, left atrium; LV, left ventricle; MDCT, multidetector computed tomography; RA, right atrium.

of cardiac tamponade. Pericardiocentesis demonstrated the bloody effusion without neoplastic cells at cytopathological examination. TTE was repeatedly performed before and after pericardiocentesis. However, the tumor was not detected in any views in these TTE examinations. In consideration of the possibility of malignant pericarditis from

other organs, the patient underwent chest and abdominal CT, suggesting the presence of an RA tumor. TTE images at the same day of CT examination are shown in Fig. 1. MDCT was then performed, clearly demonstrating that the tumor protruded into the RA, invading to the outside of the heart (Fig. 2). TEE also showed the mobile tumor in RA (Fig. 3).

Figure 3 TEE images at systole (left) and diastole (right). Mobile tumor was protruded into RA (arrows). LA, left atrium; RA, right atrium; TEE, transesophageal echocardiography.

A case of right atrial tumor diagnosed by cardiac computed tomography MDCT and TTE were performed 8 and 10 days after latest TTE examination, respectively. A surgical approach did not completely remove the tumor. Histological examination showed a vascular structure with malignant neoplasia, being defined as angiosarcoma. One month later, the patient had sudden cardiac death because of cardiac tamponade. Electrocardiographic monitoring did not show fatal arrhythmia, and there was the amount of pericardial effusion and no thrombus in pulmonary artery at autopsy.

Discussion Cardiac angiosarcoma is the most common primary malignant cardiac tumor and may occur in any part of the heart, being more common in the right heart chambers. Previous studies demonstrated the rapid growth of angiosarcoma, resulting in poor prognosis [4,5]. Rapid and accurate assessment of tumor location and its relationship to adjacent structures is therefore essential to consider the indication of medical or invasive care. Engberding et al. reported that TTE accurately diagnosed left-sided myxoma, although 25% of RA myxoma was missed [3]. In this case, TTE failed to detect the RA tumor, but was detected by MDCT and TEE. This report may claim that MDCT and TEE are required as complementary methods in the detection of cardiac tumors, especially when the tumor is not detected by TTE in patients with bloody pericardial effusion. Considering the rapid growth of angiosarcoma, the size of tumor might be different among TTE, TEE, and MDCT examinations (approximately 2 weeks). The tumor may be missed at early stage of clinical manifestation, even with TTE, MDCT, or TEE [6,7]. Second imaging tests, using less-invasive modality (cardiac MRI, TEE, or TTE with contrast media), may be useful

e11

after some interval, when the first imaging test missed the tumor.

Conflict of interest There is no conflict of interest or financial disclosure for our article.

References [1] Sun JP, Asher CR, Yang XS, Cheng GG, Scalia GM, Massed AG, Griffin BP, Ratliff NB, Stewart WJ, Thomas JD. Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients. Circulation 2001;103:2687—93. [2] Reeder GS, Khandheria BK, Seward JB, Tajik AJ. Transesophageal echocardiography and cardiac masses. Mayo Clinic Proc 1991;66:1101—9. [3] Engberding R, Daniel WG, Erbel R, Kasper W, Lestuzzi C, Curtius JM, Sutherland GR, Lambertz H, von Hehn A, Lesbre JP, Bogunovic N, Biasi S, Caruso A, Lengyel M, Iliceto S, et al., European Cooperative Study Group. Diagnosis of heart tumours by transoesophageal echocardiography: a multicentre study in 154 patients. Eur Heart J 1993;14:1223—8. [4] Shackell M, Mitko A, Williams PL, Sutton GC. Angiosarcoma of the heart. Br Heart J 1979;41:498—503. [5] Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas. A review and a case report. Cancer 1986;57:852—9. [6] Olsun A, Duzyol C, Gur AK, Kaplan M, Tosun R. Right atrial angiosarcoma: a case report. Heart Surg Forum 2007;10: E219—21. [7] El-Osta HE, Yammine YS, Chehab BM, Fields AS, Moore Jr DF, Mattar BI. Unexplained hemopericardium as a presenting feature of primary cardiac angiosarcoma: a case report and a review of the diagnostic dilemma. J Thorac Oncol 2008;3:800—2.

Journal of Cardiology Cases (2010) 1, e12—e16

available at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/jccase

Case Report

Efficacy of adaptive-servo ventilation (HEART PAPTM ) for an elderly patient with chronic heart failure who had Cheyne—Stokes respiration with central sleep apnea Yasuhiro Usui (MD) ∗, Yoshifumi Takata (MD), Kihiro Asano (MD), Yuki Hashimura (MD), Kota Kato (MD), Hirokazu Saruhara (MD), Kazuki Shiina (MD), Akira Yamashina (MD, FJCC) Department of Cardiology, Tokyo Medical University, 6-7-1 Nishi-Shinjuku, Shinjuku-ku, Tokyo 160-0023, Japan Received 16 January 2009; received in revised form 24 April 2009; accepted 3 June 2009

KEYWORDS Sleep apnea; Cheyne—Stokes respiration with central sleep apnea; Adaptive-servo ventilation

Summary An 82-year-old male patient, who had been diagnosed with chronic heart failure due to dilated cardiomyopathy and combined valvular disease and who had atrial fibrillation with complete atrioventricular block, was admitted to our hospital owing to the exacerbation of chronic heart failure. During admission, the patient became aware of drowsiness during daytime hours and had periodic apnea during sleep. Polysomnography (PSG) revealed Cheyne—Stokes respiration with severe central sleep apnea as evidenced by an apnea—hypopnea index (AHI) of 93.5/h. Nocturnal oxygen therapy failed to sufficiently suppress apnea, and arousal reactions occurred frequently. Therefore, we conducted titration by adaptive-servo ventilation (ASV; HEART PAPTM ). Consequently, subjective symptoms and respiratory sleep parameters improved. The patient showed excellent adherence to loading the device at home. PSG at 3 months after implementation of HEART PAPTM indicated improvement in the AHI to 13.5/h, and the patient exhibited marked improvements in breathlessness and awareness of drowsiness during daytime hours. HEART PAPTM was found to be a useful device for Cheyne—Stokes respiration with central sleep apnea that is associated with chronic heart failure even for very elderly patients. © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.

Introduction ∗

Corresponding author. Tel.: +81 3 3342 6111; fax: +81 3 5381 6652. E-mail address: [email protected] (Y. Usui).

Recent epidemiologic studies have clarified that sleepdisordered breathing (SDB) is a complication in approximately half of patients with chronic heart failure (HF), is involved in the pathogenesis and exacerbation of HF, and

1878-5409/$ — see front matter © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.jccase.2009.06.001

Efficacy of adaptive-servo ventilation is an important clinical entity that adversely affects life prognosis [1]. In addition, Cheyne—Stokes respiration with central sleep apnea (CSR-CSA) that includes central apnea and hyperventilation has been reported to be an important prognostic factor of chronic HF [2], and closely related to worsening quality of life (QOL) in HF patients [3]. Here we report the case of an elderly patient with severe CSRCSA that was associated with chronic HF for whom one of the interventions, adaptive-servo ventilation (ASV; HEART PAPTM , Respironics, Inc., Murrysville, PA, USA), was markedly effective.

Case report An 82-year-old male patient, who suffered from New York Heart Association class III chronic HF due to dilated cardiomyopathy, mitral regurgitation, and tricuspid regurgitation and who had been implanted with a permanent pacemaker for chronic atrial fibrillation with complete atrioventricular block, was being treated as an outpatient. The patient received torasemide (8 mg), furosemide (20 mg), spironolactone (50 mg), candesartan cilexetil (4 mg), and warfarin (3 mg) and had a history of several admissions to the hospital due to the exacerbation of HF. These medications did not change in this hospitalization. During the present admission due to the repeated acute exacerbation of HF,

e13 the patient presented with periodic apnea during sleep. We suspected that the patient’s complications included SDB because he complained of drowsiness during daytime hours and nocturia, although his Epworth Sleepiness Score (ESS) which represented objective sleepiness was 4. Therefore, we conducted polysomnography (PSG) after the improvement in HF. On admission, the patient (154.7 cm in height; 64.5 kg in body weight; 92/74 mmHg in blood pressure; and 80 bpm in pulse rate) showed jugular engorgement, systolic murmurs in the apical area, and marked edema of the lower extremities. Blood gas analysis (under atmosphere), that was performed prior to conducting PSG, revealed hypoxemia and hypocapnia as evidenced by 7.47 in pH, 35.1 torr in PCO2 , 68.7 torr in PO2, and 25 mEq/l in HCO3 − . The plasma brain natriuretic peptide (BNP) level was as high as 233 pg/ml. Plain chest X-ray indicated a cardiothoracic ratio of 72%, and echocardiography exhibited a left ventricular ejection fraction (LVEF) of 14%, a decrease in diffuse hypokinesis of the left ventricular wall, severe mitral regurgitation, and severe tricuspid regurgitation. A polysomnogram, that was obtained after the improvement in HF, is shown in Fig. 1. The patient showed severe SDB as indicated by an apnea—hypopnea index (AHI) of 93.5/h. Among the episodes, the central apnea index (CAI)—–mostly showing the waveforms of CSR-CSA—–accounted for 37.9/h. The minimum SpO2 level was 82%. Furthermore, the arousal index (ArI) was 94.2/h, indicating marked sleep fragmenta-

Figure 1 First polysomnogram. Air flow and entire respiratory movements are suspended (1), and so-called ‘‘crescendodecrescendo, fusiform hyperventilation’’ is repeated cyclically (2). This chart indicates severe sleep-disordered breathing as evidenced by an apnea—hypoxemia index of 93.5 episodes/h. Among the episodes, the central apnea index (CAI)—–mostly showing the waveforms of Cheyne—Stokes respiration with central sleep apnea (CSR-CSA)—–accounted for 37.9 episodes/h.

e14 tion associated with hyperventilation. Consequently, sleep quality was noticeably impaired. Furthermore, the patient had eight nocturnal micturitions, also indicating sleep fragmentation by these episodes. PSG indicated typical CSR-CSA. A beta-adrenergic receptor blocker was difficult to be used for our patient because he was hypotensive. Therefore, we considered nonpharmacotherapy for CSR-CSA. The patient presented possible difficulty in undergoing positive pressure ventilation therapy because of old age and underlying disease. First, we conducted titration by nocturnal oxygen therapy (3 L/min). Consequently, SDB improved as indicated by an AHI of 51.4/h, with a CAI of 3.1/h. However, hypopnea considered to be residual central and/or changing obstructive, and an elevated ArI (66.1/h) associated thereto persisted, and the number of nocturnal micturitions did not reduce. Oxygen therapy failed to sufficiently improve respiratory sleep parameters and sleep quality. Therefore, we conducted titration with HEART PAPTM (Fig. 2). Time was required to set initial pressures. Therefore, apnea persisted as evidenced by an AHI of 37.6/h and a CAI of 2.6/h. However, the ArI reduced to 38.2/h, and slow-wave sleep and REM sleep—–that had not been observed during oxygen therapy—–occurred. Furthermore, the patient had no nocturnal micturition and noticed, after the titration, a feeling of sound sleep and alleviated drowsiness during daytime hours. HEART PAPTM improved respiratory sleep parame-

Y. Usui et al. ters and sleep quality and was also well-tolerated by the patient. Therefore, the device was implemented under the following conditions: 24 cmH2 O in the maximum inspiratory positive airway pressure (IPAP max); 10 cmH2 O in the minimum inspiratory positive airway pressure (IPAP min); 10 cmH2 O in expiratory positive airway pressure (EPAP); and 15 breaths/min in respiratory rate. Subsequent follow-up on an outpatient basis revealed excellent adherence to loading the device. The mean duration of device use was 6.5 h/day. Edema of the lower extremities, nocturnal pollakiuria, a feeling of sound sleep, and exertional breathlessness during daytime hours improved. At 3 months after implementation of HEART PAPTM , PSG was conducted in the patient who loaded HEART PAPTM under the same conditions as those for outpatient care. Consequently, the AHI reduced to 16.3/h and the minimum SpO2 was 91%; hence, the patient showed improvement in hypoxemia. Furthermore, the ArI also showed a marked reduction to 13.0/h, and the improvement of both sleep depth and REM sleep persisted from the time of implementing HEART PAPTM . In addition, the patient showed no nocturnal micturition during the testing. During these 3 months, there was no change in medications for heart failure. LVEF evidenced by echocardiography slightly improved from 14% to 21%. However, the patient’s impression about usage is excellent, and he is under continuing treatment on an outpatient basis without readmission for 2 years.

Figure 2 Polysomnogram under HEART PAPTM titration. Ventilatory volume on a breath-by-breath basis is averaged by auto inspiratory positive airway pressure (IPAP) (1), desaturation is improved (2), and arousal reactions are reduced.

Efficacy of adaptive-servo ventilation

e15

Discussion CSR-CSA is concurrently associated with approximately 30—40% of patients with chronic HF, and the following events are considered to be involved in its pathogenesis: (1) hyperventilation due to the stimulation of lung stretch receptors induced by chronic pulmonary congestion associated with decreased cardiac output; (2) enhanced sensitivity of central chemoreceptors; and (3) a delay in circulation time associated with decreased cardiac output. Namely, CSRCSA is considered to be the consequence of HF. However, Lanfranchi et al. [2] have reported that the presence of CSR-CSA itself is an independent prognostic factor for HF. Moreover, Carmona-Bernal et al. [3] have demonstrated that HF patients with CSR-CSA had a worse QOL than those with HF alone. Consequently, CSR-CSA currently attracts attention as a new therapeutic target for chronic HF. To date, oxygen therapy [4], continuous positive airway pressure (CPAP) [5], bi-level positive airway pressure (BiPAP) [6], ASV [7], and other modalities have been reported as nonpharmacologic therapies for CSR-CSA. However, an improvement in long-term prognosis has never been reported, and no established therapeutic modality is currently available. However, beta-adrenergic receptor blockers and cardiac resynchronization therapy (CRT) have been reported to reduce the CAI [8,9]. Therefore, basic treatments for chronic HF are essential, i.e., sufficient pharmacotherapy principally consisting of beta-adrenergic receptor blockers and CRT (in patients for whom the modality is indicated). However, our patient could not receive these treatments for HF because of hypotension, severe tricuspid regurgitation, and high age, so we had to choose nonpharmacologic therapies for CSR-CSA. ASV (HEART PAPTM ), noninvasive positive pressure ventilation (NPPV) that was developed for the purpose of treating SDB, which is concurrently associated with chronic HF, is principally characterized by its function of assisting IPAP that automatically allows the sustainment of proper ventilation volume in proportion to the patient’s respiratory condition (auto IPAP). Furthermore, HEART PAPTM can set EPAP—–that deletes the components of OSA which is present frequently in a concurrent fashion—–and is theoretically capable of addressing all levels of SDB in HF. We surmised since the beginning of the present admission that NPPV was

Table 1

difficult to be implemented for our patient because of age, underlying disease, and other factors. First, we conducted nocturnal oxygen therapy. Although desaturation improved, sleep quality failed to be improved due to the frequent occurrence of arousal reactions induced by persisting CSRCSA. Nocturnal oxygen therapy (3 L/min) for outpatients with chronic HF, whose respiratory disturbance index (RDI) in arterial blood was ≥5 episodes/h and whose LVEF was ≤45%, improved their QOL [4]. However, this study examined principally patients with mild chronic HF and did not conduct nocturnal PSG in all the patients examined. Therefore, sleep quality was not assessed. Furthermore, they considered the improvement of oxygen saturation due to oxygen therapy as improvement in respiratory parameters; therefore, they possibly underestimated respiratory events involving arousal reactions. On the other hand, HEART PAPTM indicated increases in deep sleep and REM sleep and was tolerated by the patient rather better than oxygen therapy. Table 1 shows the results of PSG which was performed before treatment for CSR-CSA, under titration by nocturnal oxygen therapy, titration with HEART PAPTM , and after 3 months after implementation of HEART PAPTM . Our patient was a very elderly with low cardiac function, for whom NPPV is generally considered difficult to be implemented. Therefore, we considered that HEART PAPTM is a potentially effective therapeutic option also for patients for whom positive pressure therapy has been difficult to be implemented because of its low tolerability. Moreover, a study [5] reported that excessive preload reduced by CPAP might be one of the causes for the fact that the therapy had not improved prognosis. Our patients received extraordinary high IPAP pressure because obstructive component did not diminish even 10 cmH2 O of EPAP during titration. Avoiding the excessive high EPAP pressure, therefore, high IPAP max pressure was needed. During follow-up, however, our patient showed reduced edema of the lower extremities without a decrease in blood pressure. Therefore, we regard that ASV is an effective therapy when conducted at pressures that were set by titration under sufficient observation. Kasai et al. reported that HEART PAPTM significantly improved respiratory sleep parameters and sleep parameters for chronic HF patients with CSR-CSA which had persisted also under the use of CPAP and BiPAP [10]. However, they studied only one night and did not evaluate the long-term effectiveness of HEART

PSG findings of before and after treatment for CSR-CSA.

AHI (episodes/h) CAI OAI % of TST SpO2 31 J. An ICD with a transvenous right ventricular defibrillation lead alone required a high DFT. The decision was made to enhance the existing system with the addition

of a subcutaneous array lead (ENDOTAK SQ lead array model 0049; Cardiac Pacemakers, Inc., St. Paul, MN, USA) to lower the DFT. This lead consisted of three electrically common multifilar coil elements that comprised a single lead with an effective surface area of 39 cm2 . This procedure was performed as physician’s manual under general anesthesia in the operating room, after seven days. A short skin incision perpendicular to the ribs was made in the anterior-axillary line. The array lead was inserted through the left anterioraxillary incision and was positioned in the left lateral chest using a lead tunneler and sheaths (Fig. 2). The array lead terminal was tunneled subcutaneously to the ICD generator pocket and was attached to the ICD generator. The system was tested using the subcutaneous array lead and the proximal coil of the right ventricular lead as the anode and the distal coil of the right ventricular lead as the cathode. Both the first and the second induced VF were successfully converted with 21 J. The measured impedance was 40 ohms at each defibrillation. Defibrillation energy requirement was markedly reduced following the addition of the subcutaneous array lead. The ICD system with the subcutaneous array lead provided an adequate defibrillation safety margin of 10 J. Therefore, we did not attempt the lower defibrillation output below 21 J. The implantation procedure was well tolerated and involved only two incisions for the insertion of the entire lead system. There were no postoperative complications. The patient was followed up every three months, with no events noted.

Discussion ICDs terminate ventricular tachycardia (VT) and VF with high efficacy and reduce the rate of SCD in patients with fatal arrhythmias [4]. Following the introduction of transvenous defibrillation leads, implantation of ICDs was further simplified by the active can ICD concept. In a multicenter study, 98% of patients could be successfully implanted with the active can system using biphasic shocks [5]. Nevertheless, a sufficiently low DFT cannot be obtained in 2% of patients.

Efficacy of subcutaneous array lead

e23

Figure 2 Chest radiographs on admission (A) and after implantable cardioverter defibrillator (ICD) implantation using a transvenous lead system and a three-finger subcutaneous array lead (B). The transvenous right ventricular defibrillation lead is placed in the right ventricular apex. The subcutaneous array lead is positioned along the lateral aspect of the left chest to the posterior area.

Herein, we describe a case of BS in whom a subcutaneous array lead resulted in a marked reduction in defibrillation energy requirements and no complications. ICD implantation can be performed successfully using a transvenous lead system in the majority of patients with BS. Patients with BS are sometimes complicated with bradyarrhythmia, such as sick sinus syndrome or atrioventricular block [6]. Although we did not perform the genetic analysis of BS, a pilsicainide challenge test documented BS. Thus, sick sinus syndrome may be part of manifestation of genetic BS in the patient. The possible mechanisms of a high DFT in this case are old abandoned pacemaker leads and some characteristic abnormalities of BS. A transvenous right ventricular defibrillation lead might be placed only in the ineffective position of the right ventricle due to abandoned leads for an upgrade from a pacemaker to an ICD. In BS, electrophysiologic abnormalities: the area of conduction delay and the site of high inducibility of VF are believed to exist in the right ventricular outflow tract region [7]. The defibrillation shocks were uniformly delivered between the endocardial lead at the right ventricular apex and the active can in the left subclavicular region of the anterior chest. However, adequate current density in the right ventricular outflow tract may be necessary to produce adequate defibrillation. Watanabe et al. reported the characteristics of short ventricular fibrillation cycle length (VFCL) in BS [8]. Since previous studies have shown that DFT is negatively correlated with VFCL, short VFCL might be an alternative explanation for high DFT [9]. Therefore, an ICD with a transvenous right ventricular defibrillation lead alone might have a very high DFT and an unsafe device function. Options for the management of high DFTs include the use of a high-output device (at present a maximum of 36 J), addition of a subcutaneous array lead, or adjustment to the tilt of the biphasic waveform [10]. However, the use of a highoutput device alone was not enough to obtain an adequate DFT in 48% of patients who required modifications [11], and in this case it was unable to provide an adequate defibrillation safety margin (>10 J). Adjustment to the tilt of the biphasic waveform is a viable option for managing high DFTs in some patients. However, not all device manufacturers allow this option and head-to-head comparisons of specific tilt configurations have not demonstrated an ideal ‘‘one size fits all’’ configuration.

The addition of a subcutaneous array lead is a common strategy for lowering the DFT [10], and it has been shown in a previous study that the subcutaneous array lead can significantly lower the DFT when added to an existing transvenous configuration [3]. Therefore, we used the subcutaneous array lead, which improved defibrillation efficacy. It probably can be attributed to the larger defibrillation surface area and improved defibrillation field geometry due to more homogenous current within the heart [12]. Additionally, a reduced DFT offers the main advantage that the charge time will be markedly shortened if the shock necessary to terminate VT/VF is below 30—36 J. Hence, the incidence of syncopal events, which is as high as 15% of patients if the first shock is programmed to a maximum of 34 J, can be reduced [13]. Furthermore, the maximum output of the device can be reduced with a further decrease in its size and weight, or longevity can be improved. Thus, implantation of an extra subcutaneous array lead might be an option in patients with frequent syncopal events due to VT/VF despite ICD therapy to reduce the DFT and the charging time. In the present case, we did not perform the DFT testing just before the addition of a subcutaneous array lead. Even if we did and an ICD with a transvenous right ventricular defibrillation lead alone terminated VF, this ICD system cannot always terminate VF. Condition of the patient and medication were unchanged between implantation of a transvenous defibrillation lead system and addition of a subcutaneous array lead. Therefore, it is suggested that a subcutaneous array lead improved defibrillation efficacy. In conclusion, the novel subcutaneous array lead approach described in the present case facilitates implantation of an effective ICD lead system in patients requiring a high DFT. The subcutaneous array lead is a promising adjunct to a transvenous lead system in cases where the DFT with the transvenous lead alone is unacceptable.

References [1] Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome: a multicenter report. J Am Coll Cardiol 1992;20:1391—6. [2] Antzelevitch C, Brugada P, Borggrefe M, Brugada J, Brugada R, Corrado D, Gussak I, LeMarec H, Nademanee K, Perez Riera

e24

[3]

[4]

[5]

[6]

[7]

Y. Hadano et al. AR, Shimizu W, Schulze-Bahr E, Tan H, Wilde A. Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation 2005;111:659—70. Gradaus R, Block M, Seidl K, Brunn J, Isgro F, Hammel D, Hauer B, Breithardt G, Bocker D. Defibrillation efficacy comparing a subcutaneous array electrode versus an ‘‘active can’’ implantable cardioverter defibrillator and a subcutaneous array electrode in addition to an ‘‘active can’’ implantable cardioverter defibrillator: Results from active can versus array trials I and II. J Cardiovasc Electrophysiol 2001;12:921—7. The Antiarrhythmics versus Implantable Defibrillators (AVID) Investigators. A comparison of antiarrhythmic-drug therapy with implantable defibrillators in patients resuscitated from near-fatal ventricular arrhythmias. N Engl J Med 1997;337:1576—83. Bardy GH, Yee R, Jung W. Multicenter experience with a pectoral unipolar implantable cardioverter-defibrillator. Active Can Investigators. J Am Coll Cardiol 1996;28:400—10. Makiyama T, Akao M, Tsuji K, Doi T, Ohno S, Takenaka K, Kobori A, Ninomiya T, Yoshida H, Takano M, Makita N, Yanagisawa F, Higashi Y, Takeyama Y, Kita T, et al. High risk for bradyarrhythmic complications in patients with Brugada syndrome caused by SCN5A gene mutations. J Am Coll Cardiol 2005;46:2100—6. Morita H, Fukushima-Kusano K, Nagase S, Takenaka-Morita S, Nishii N, Kakishita M, Nakamura K, Emori T, Matsubara H, Ohe

[8]

[9]

[10] [11]

[12]

[13]

T. Site-specific arrhythmogenesis in patients with Brugada syndrome. J Cardiovasc Electrophysiol 2003;14:373—9. Watanabe H, Chinushi M, Sugiura H, Washizuka T, Komura S, Hosaka Y, Furushima H, Watanabe H, Hayashi J, Aizawa Y. Unsuccessful internal defibrillation in Brugada syndrome: focus on refractoriness and ventricular fibrillation cycle length. J Cardiovasc Electrophysiol 2005;16:262—6. Murakawa Y, Yamashita T, Kanese Y, Sezaki K, Omata M. Is ventricular fibrillation interval an indicator of electrical defibrillation threshold? Pacing Clin Electrophysiol 1999;22:302—6. Mainigi SK, Callans DJ. How to manage the patient with a high defibrillation threshold. Heart Rhythm 2006;3:492—5. Russo AM, Sauer W, Gerstenfeld EP, Hsia HH, Lin D, Cooper JM, Dixit S, Verdino RJ, Nayak HM, Callans DJ, Patel V, Marchlinski FE. Defibrillation threshold testing: Is it really necessary at the time of implantable cardioverter-defibrillator insertion? Heart Rhythm 2005;2:456—61. Chen PS, Wolf PD, Claydon FJ, Dixon EG, Vidaillet Jr HJ, Danieley ND, Pilkington TC, Ideker RE. The potential gradient field created by epicardial defibrillation electrodes in dogs. Circulation 1986;74:626—36. Bansch D, Brunn J, Castrucci M, Weber M, Gietzen F, Borggrefe M, Breithardt G, Block M. Syncope in patients with an implantable cardioverter-defibrillator: Incidence, prediction and implications for driving restrictions. J Am Coll Cardiol 1998;31:608—15.

Journal of Cardiology Cases (2010) 1, e25—e27

available at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/jccase

Case Report

Intravenous myocardial contrast echocardiography can predict recurrence of pressure gradient of left ventricular outflow tract in hypertrophic obstructive cardiomyopathy after percutaneous transluminal septal myocardial ablation Sinichiro Suna (MD, PhD), Masami Nishino (MD, PhD, FJCC) ∗, Tamaki Sawada (MD), Yasuyuki Egami (MD), Ryu Shutta (MD), Jun Tanouchi (MD, PhD, FJCC), Yoshio Yamada (MD, PhD, FJCC) Division of Cardiology, Osaka Rosai Hospital, 1179-3, Nagasone-cho, Kita-ku, Sakai-city, Osaka 591-8025, Japan Received 7 February 2009; received in revised form 9 June 2009; accepted 10 June 2009

KEYWORDS Myocardial contrast echocardiography; Hypertrophic obstructive cardiomyopathy; Percutaneous transluminal septal ablation; Levovist; Alcohol

Summary Percutaneous transluminal septal myocardial ablation (PTSMA) is appropriate in patients who are refractory to medical treatments for hypertrophic obstructive cardiomyopathy (HOCM). However, about 10% of patients show recurrence of left ventricular outflow tract pressure gradient (LVOG) after PTSMA and need repeat PTSMA. The authors describe two HOCM cases who underwent intravenous myocardial contrast echocardiography (IVMCE) at two weeks after PTSMA. IVMCE revealed the total defect of the PTSMA area in one patient, a 69-year-old woman. This patient has shown no recurrence of LVOG for 4 years. Faintly stained PTSMA area by IVMCE was seen in the other patient, an 83-year-old woman. This patient had a recurrence of LVOG 3 months later. Therefore, in conclusion, IVMCE might predict recurrence of LVOG after PTSMA. © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.

Introduction Percutaneous transluminal septal myocardial ablation (PTSMA) [1,2] is a recognized and promising non-surgical

∗

Corresponding author. Fax: +81 72 250 5492. E-mail address: [email protected] (M. Nishino).

treatment for symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM) who are refractory to medical treatment including ␤-blockers, calcium channel blockers, and class Ia antiarrhythmic drugs (e.g. cibenzoline and disopyramide). It has been reported that myocardial contrast echocardiography (MCE) during PTSMA is useful for selection of target vessels [3,4]; however, the usefulness of intravenous MCE (IVMCE) after PTSMA is unknown.

1878-5409/$ — see front matter © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.jccase.2009.06.005

e26

S. Suna et al.

Case report Two cases with HOCM refractory to medical treatment underwent PTSMA. Myocardial perfusion analysis by IVMCE with a slow-bolus injection of Levovist (Schering, Berlin, Germany; 200 mg/ml, 0.5 ml/s), followed by a 10-ml slow saline flush (over 10 s) and intermittent ultrasonic exposure (1:4 intermittent exposure using the ultraharmonic mode) [5] (Sonos 5500, Phillips Ultrasound, Andover, MA, USA) was performed two weeks after PTSMA for each patient. We depicted the apical 4 chamber view with ultraharmonic mode [high mechanical index (MI) imaging: MI > 1.0]. IVMCE imaging was obtained at a pulsing interval of 4 cardiac cycles with a phased-array transducer, using transmit/receive frequencies of 1.3/3.6 MHz. Case 1, a 69-year-old woman, whose chief complaint was dyspnea on effort, had a 3/6 cardiac systolic murmur at third left intercostal space, and her blood pressure was 130/70 mmHg. Transthoracic echocardiography showed thickening of basal interventricular septum (IVS) and existence of left ventricular outflow tract stenosis during systole, resulting in an increased left ventricular outflow tract pressure gradient (LVOG) of 83 mmHg. She was diagnosed as having hypertrophic obstructive cardiomyopathy (HOCM). She was intolerant of ␤-blockers and a Ia antiarrhythmic drug (disopyramide). Therefore, she was admitted to our hospital for PTSMA. Coronary angiography showed no significant stenosis. Guiding catheter was inserted in the ostium of the left anterior descending (LAD) coronary artery and balloon catheter was inserted to the first septal perforating branch. When Levovist (200 mg/ml, 2 ml) was administered in the first septal branch selectively, basal IVS, which was the cause of the stenosis during systole, was well opacified. Therefore, the first septal branch was diagnosed as the target vessel. Then, absolute ethanol was administered in the first septal branch selectively with careful observation not to inject to the LAD artery. Just after PTSMA, LVOG was reduced to 0 mmHg in the catheter laboratory. Two weeks after PTSMA, transthoracic Doppler-echocardiography showed basal IVS was akinetic and LVOG was not evident, and IVMCE visualized a total defect of basal IVS (Fig. 1). This was fully consistent with the opacified area seen after intracoronary (septal branch) injection of Levovist during PTSMA [4]. In this case, there has been no sign of recurrence of LVOG for 4 years after PTSMA. Case 2, an 83-year-old woman, experienced dyspnea on effort. She was incidentally found to have a cardiac murmur at medical check up and referred to our out-patient clinic. Her blood pressure was 90/50 mmHg, pulse was regular at 60 beats/min, and her heart sound showed a 3/6 systolic murmur at third left intercostal space. Transthoracic Doppler-echocardiography revealed that thickness of basal IVS was 20 mm and LVOG was 150 mmHg. She was considered as having HOCM and ␤-blockers and a Ia antiarrhythmic drug (cibenzoline) were administered orally. After 3 months of medications, no significant improvement was observed. Thus, she was diagnosed as being indicated for PTSMA. PTSMA was conducted with the same protocol as case 1. After PTSMA, the patient showed a reduction in LVOG (0 mmHg in catheter laboratory) and there was a dramatic improvement in her clinical condition. Two weeks

Figure 1 Case 1: Intravenous myocardial contrast echocardiography (IVMCE) at 3 months after percutaneous transluminal septal myocardial ablation (PTSMA). Basal interventricular septum (IVS) is visualized as a total defect (an arrow). LV, left ventricle; LA, left atrium; RV, right ventricle; RA, right atrium.

after PTSMA, IVMCE was performed using the same method as for case 1. IVMCE after two weeks of PTSMA showed faintly opacified basal IVS which was consistent with PTSMA area. This imaging denoted the residual blood flow and residual viable myocardium in the PTSMA area (Fig. 2). In case 2, at 3 months after PTSMA there was a sign of recurrence of LVOG (52 mmHg) and mild symptoms (dyspnea on effort) appeared. Peak creatine kinase (CK) and the amount of alcohol at PTSMA were similar in these two cases as follows: peak CK, 1178 IU/l in case 1 vs. 1473 IU/l in case 2 and alcohol, 1.5 ml in case 1 vs. 2 ml in case 2.

Figure 2 Case 2: Intravenous myocardial contrast echocardiography (IVMCE) at 3 months after percutaneous transluminal septal myocardial ablation (PTSMA). Faintly opacified area (arrows) was found in the basal interventricular septum (IVS) that corresponded with PTSMA area (an arrow). LV, left ventricle; LA, left atrium; RV, right ventricle; RA, right atrium.

MCE can predict recurrence after PTSMA

e27

Discussion PTSMA is an effective non-surgical technique for reduction of symptoms and LVOG in HOCM. Surgical myectomy is also effective for drug-refractory HOCM, however PTSMA is a less invasive procedure than myectomy and myectomy can be performed after unsuccessful PTSMA [6]. Therefore, in our two cases, we decided to perform PTSMA first. However, approximately 10% of patients need repeat PTSMA due to a recurrence of LVOG [7,8]. One reason for the recurrence may be an unsatisfactory initial necrosis and the development of collateral circulation. Experimental studies have demonstrated that the size of the contrast defect correlated well with the extent of myocardial necrosis [9,10]. Accordingly, contrast defect in IVMCE reveals non-viable myocardium. The mechanism of PTSMA is alcohol-induced necrosis in myocardium supplied by the target vessel. Thus, IVMCE is suitable to evaluate the area where alcohol induced necrosis. On the other hand, IVMCE is an ideal tool for measuring microcirculatory flow because it used tracers of red blood cells [11]. Therefore, the faintly stained area is the area that is supplied by microcirculatory flow even if it showed perfusion abnormality [12—14]. The faintly stained area was considered as the residual viable muscle in the PTSMA area. Some clinical studies have shown that IVMCE can predict functional recovery after reperfusion therapy in patients with acute myocardial infarction [13,15,16]. Moreover, it has been reported that the degree of MCE enhancement in the infarct area generally corresponded to the collateral grades assessed with coronary angiography [17]. These data suggested that IVMCE can be applied to predict recurrence after PTSMA. Actually, in case 1, whose IVMCE showed complete contrast defect in PTSMA area that corresponded with myocardial necrosis, no recurrence of LVOG was seen 3 months after PTSMA, while in case 2 whose IVMCE showed faintly the PTSMA area that denoted existence of residual viable muscle, recurrence of LVOG occurred at chronic phase. This is the first reported case in whom IVMCE showed the differences in perfusion pattern in PTSMA area that might predict the recurrence of LVOG. However, this is only a case report, therefore, further study should be required to confirm our speculation. We conclude that IVMCE at two weeks after PTSMA might predict the recurrence of LVOG at chronic phase in patients with HOCM.

References [1] Shamim W, Yousufuddin M, Wang D, Henein M, Seggewiss H, Flather M, Coats AJ, Sigwart U. Nonsurgical reduction of the interventricular septum in patients with hypertrophic cardiomyopathy. N Engl J Med 2002;347:1326—33. [2] Seggewiss H, Gleichmann U, Faber L, Fassbender D, Schmidt HK, Strick S. Percutaneous transluminal septal myocardial ablation in hypertrophic obstructive cardiomyopathy: acute results and 3-month follow-up in 25 patients. J Am Coll Cardiol 1998;31:252—8. [3] Okayama H, Sumimoto T, Morioka N, Yamamoto K, Kawada H. Usefulness of selective myocardial contrast echocardiography

[4]

[5]

[6]

[7]

[8]

[9]

[10]

[11]

[12]

[13]

[14]

[15]

[16] [17]

in percutaneous transluminal septal myocardial ablation: a case report. Jpn Circ J 2001;65:842—4. Faber L, Seggewiss H, Gleichmann U. Percutaneous transluminal septal myocardial ablation in hypertrophic obstructive cardiomyopathy: results with respect to intraprocedural myocardial contrast echocardiography. Circulation 1998;98: 2415—21. Lombardo A, Rizzello V, Galiuto L, Natale L, Giordano A, Rebuzzi A, Loperfido F, Crea F, Maseri A. Assessment of resting perfusion defects in patients with acute myocardial infarction: comparison of myocardial contrast echocardiography, combined first-pass/delayed contrast-enhanced magnetic resonance imaging and 99mTC-sestamibi SPECT. Int J Cardiovasc Imaging 2006;22:417—28. Nagueh SF, Buergler JM, Quinones MA, Spencer III WH, Lawrie GM. Outcome of surgical myectomy after unsuccessful alcohol septal ablation for the treatment of patients with hypertrophic obstructive cardiomyopathy. J Am Coll Cardiol 2007;50: 795—8. Faber L, Meissner A, Ziemssen P, Seggewiss H. Percutaneous transluminal septal myocardial ablation for hypertrophic obstructive cardiomyopathy: long term follow up of the first series of 25 patients. Heart 2000;83:326—31. Alam M, Dokainish H, Lakkis N. Alcohol septal ablation for hypertrophic obstructive cardiomyopathy: a systematic review of published studies. J Interv Cardiol 2006;19:319—27. Coggins MP, Sklenar J, Le DE, Wei K, Lindner JR, Kaul S. Noninvasive prediction of ultimate infarct size at the time of acute coronary occlusion based on the extent and magnitude of collateral-derived myocardial blood flow. Circulation 2001;104:2471—7. Lafitte S, Higashiyama A, Masugata H, Peters B, Strachan M, Kwan OL, DeMaria AN. Contrast echocardiography can assess risk area and infarct size during coronary occlusion and reperfusion: experimental validation. J Am Coll Cardiol 2002;39:1546—54. Lindner JR, Song J, Jayaweera AR, Lindner JR, Kaul S. Microvascular rheology of Definity microbubbles after intraarterial and intravenous administration. J Am Soc Echocardiogr 2002;15:396—403. Lindner JR, Villanueva FS, Dent JM, Wei K, Sklenar J, Kaul S. Assessment of resting perfusion with myocardial contrast echocardiography: theoretical and practical considerations. Am Heart J 2000;139:231—40. Leistad E, Ohmori K, Peterson TA, Christensen G, DeMaria AN. Quantitative assessment of myocardial perfusion during graded coronary artery stenoses by intravenous myocardial contrast echocardiography. J Am Coll Cardiol 2001;37:624—31. Masugata H, Peters B, Lafitte S, Strachan GM, Ohmori K, DeMaria AN. Quantitative assessment of myocardial perfusion during graded coronary stenosis by real-time myocardial contrast echo refilling curves. J Am Coll Cardiol 2001;37: 262—9. Lepper W, Hoffmann R, Kamp O, Franke A, de Cock CC, Kühl HP, Sieswerda GT, Dahl J, Janssens U, Voci P, Visser CA, Hanrath P. Assessment of myocardial reperfusion by intravenous myocardial contrast echocardiography and coronary flow reserve after primary percutaneous transluminal coronary angioplasty [correction of angiography] in patients with acute myocardial infarction. Circulation 2000;101:2368—74. Zoghbi WA. Evaluation of myocardial viability with contrast echocardiography. Am J Cardiol 2002;90:65J—71J. Lim YJ, Nanto S, Masuyama T, Kodama K, Kohama A, Kitabatake A, Kamada T. Coronary collaterals assessed with myocardial contrast echocardiography in healed myocardial infarction. Am J Cardiol 1990;66:556—61.

Journal of Cardiology Cases (2010) 1, e28—e32

available at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/jccase

Case Report

Infective endocarditis associated with acute myocardial infarction caused by septic emboli Iwao Okai (MD) a, Kenji Inoue (MD, PhD) a,∗, Naotaka Yamaguchi (MD) b, Haruka Makinae (MD) c, Sonomi Maruyama (MD) a, Kaoru Komatsu (MD) d, Yasunobu Kawano (MD) e, Shinya Okazaki (MD) a, Yasumasa Fujiwara (MD) a, Masataka Sumiyoshi (MD) a, Atsushi Amano (MD, FJCC) c, Hiroyuki Daida (MD, FJCC) d a

Department of Cardiology, Juntendo University Nerima Hospital, 3-1-10 Takanodai, Nerima-ku, Tokyo 177-8521, Japan Department of Emergency and Intensive Care, Juntendo University Nerima Hospital, Tokyo, Japan c Department of Cardiovascular Surgery, Juntendo University School of Medicine, Tokyo, Japan d Department of Cardiology, Juntendo University School of Medicine, Tokyo, Japan e Department of Cardiology, Juntendo University Urayasu Hospital, Chiba, Japan b

Received 15 December 2008; received in revised form 30 May 2009; accepted 10 June 2009

KEYWORDS Infective endocarditis; Myocardial infarction; Septic emboli

∗

Abstract A 53-year-old Japanese man presented with severe chest pain. He had suffered from persistent fever, muscle pain, arthralgia, and dyspnea on exertion (New York Heart Association class I) for two and half months prior to admission. He had been treated with several antibiotics for two months and prednisolone for almost one month prior to admission. On the day of admission, he had suffered from chest pain at rest, and had come to our hospital. Electrocardiography showed a normal sinus rhythm with significant ST segment elevation in leads V3—6 and abnormal Q waves in leads V4—6. Transthoracic echocardiography demonstrated left ventricular ejection fraction of 52% with severe mitral regurgitation and an 18-mm vegetation on the anterior mitral valve leaflet. Multiple blood cultures identified Streptococcus sanguis. The diagnosis was acute myocardial infarction and mitral regurgitation associated with infective endocarditis (IE). The incidence of acute coronary syndrome caused by IE is quite low in patients with native valves. After a 6-week course of antibiotics, mitral valve replacement and partial cardiomyotomy were performed. Two years after the surgery, follow-up echocardiography showed almost normal left ventricle function and no mitral regurgitation, and the patient has been living an active life without any complications. © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.

Corresponding author. Tel.: +81 3 5923 3111; fax: +81 3 5923 3217. E-mail addresses: [email protected], [email protected] (K. Inoue).

1878-5409/$ — see front matter © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.jccase.2009.06.003

Infective endocarditis associated with acute myocardial infarction caused by septic emboli

e29

1. Introduction Infective endocarditis (IE) is still associated with high inhospital mortality, ranging from 16% to 25%, and a high incidence of embolic events, ranging from 13% to 49%, despite recent improvements in diagnostic and therapeutic strategies [1]. Such wide ranges of incidence of mortality events emphasize the heterogeneity of the disease and the critical need for baseline risk stratification in order to focus aggressive management toward the high risk subsets of patients. IE is well known to cause many types of complication [2]. Systemic embolism is a common complication of IE, most frequently involving the central nervous system, spleen, kidney, liver, and iliac or mesenteric arteries, whereas acute coronary syndrome is infrequently encountered [3]. Acute myocardial infarction (AMI) complicated by septic coronary embolism from IE is a rare and fatal condition. The incidence of coronary septic embolism is difficult to estimate. Three series found that 11 (10.6%) of 104 Russian patients had AMI as a result of septic embolism [4], only 14 (2.9%) of 586 Spanish patients had acute coronary syndrome, and half were associated with prosthetic valves, 1.5% of cases of acute coronary syndrome occurred with native valves, and embolism was the cause in only 3 (0.51%) of 586 patients [5]. And only 2 (0.52%) of 384 patients had coronary embolisms in a multicenter prospective European study [1]. Therefore, the incidence of acute coronary syndrome is quite low in patients with native valves. Here, we report a case of AMI complicated by septic coronary embolism caused by IE of the mitral valve.

2. Case report A 53-year-old Japanese man presented with severe chest pain. He had suffered from persistent fever, muscle pain, arthralgia, and dyspnea on exertion (New York Heart Association class II) for two and a half months prior to admission. He had been treated with several antibiotic agents including cefditoren pivoxil, azithromycin, or cefpodoxime proxetil for two months at another clinic, but his fever had persisted. A primary physician had suspected vasculitis, and prescribed prednisolone (20 mg/day) for almost one month. He had suffered from chest pain at rest on the day of admission, and had come to our hospital. On admission, body temperature was 36.7 ◦ C, heart rate was 80 beats per minute, and blood pressure was 130/90 mmHg. Respiratory rate was 26 per minute with oxygen saturation of 95% breathing ambient air. Physical examination found coarse breath sounds and bilateral crackles, and a grade III systolic murmur at the left sternal border. The abdomen was normal. No peripheral edema was present in the legs. Skin examination revealed numerous petechiae, and multiple small, peripheral necrotic lesions on the feet (Fig. 1a). Conjunctival petechiae were identified without typical Roth’s spots (Fig. 1b). No other physical or neurological abnormalities were found, although he had dysphasia 1 week prior to admission which had improved. His coronary risk factors were hypertension, dyslipidemia, and current smoking. He had been treated with valsartan (80 mg/day) and fluvastatin (10 mg/day).

Fig. 1 (a) Painful papules were present at both extremities (arrows). (b) Petechiae were present on the palpebral conjunctiva (arrows). (c) Diffusion-weighted magnetic resonance images showed scattered regions of hyperintensity in the left middle cerebral artery territory (arrow). Intraluminal filling defects were not seen.

The results of laboratory tests are shown as follows. The white blood cell count was 17,000 mm−3 with 89% neutrophils. The level of creatine kinase had increased to 458 IU and troponin T test showed positive finding. Electrocardiography showed a normal sinus rhythm with significant ST segment elevation in leads V3—6 and abnormal Q waves in leads V4—6 (Fig. 2a). Chest radiography

e30

I. Okai et al.

Fig. 2 (a) Electrocardiogram on admission showed significant ST segmental elevation in leads V3—6, and abnormal Q waves in leads V4—6. (b) Coronary angiogram done 6 weeks after the onset of acute myocardial infarction (cranial view) showed total occlusion of the distal left anterior descending artery (arrow).

Fig. 3 Transthoracic echocardiographic images, four chamber view, showed highly mobile vegetations and hypokinesia of the left ventricular apex (left). The vegetation size was 18 mm. Severe mitral regurgitation was seen in the Doppler flowmetry image (right).

Infective endocarditis associated with acute myocardial infarction caused by septic emboli

e31

nificantly improved and repetitive blood cultures were negative. Postoperative echocardiography revealed normal function of the prosthetic mitral valve and hypokinesis of the apex of the left ventricle. One and a half years after the surgery, follow-up echocardiography showed no abnormalities in the regional wall motion of the left ventricle, and he has been living an active life without complications.

3. Discussion

Fig. 4 Macroscopic view of the mitral valve with vegetations. The vegetation is the large irregular, red swelling on the valve leaflet. Leaflet perforation is seen.

showed cardiomegaly and marked pulmonary edema. Bedside transthoracic echocardiography demonstrated left ventricular ejection fraction of 52% with severe mitral regurgitation and an 18-mm vegetation on the anterior mitral valve leaflet (Fig. 3). The periannular valve was intact. Hypokinesis was observed in the apical anterior of the left ventricle. Brain magnetic resonance imaging revealed cerebral infarctions in the distal portion of the left middle cerebral artery (Fig. 1c). Brain magnetic resonance angiography did not reveal any mycotic aneurysm. Six hours after the onset of chest pain, the electrocardiographic findings and serum enzyme measurements strongly suggested anterolateral AMI. However, we did not perform coronary angiography because of the presence of heart failure, and could not rule out the possibility of the vegetation in the aortic valve. We initiated conservative treatment with diuretics and planned an elective mitral valve operation because he had cerebral infarctions due to septic emboli. After obtaining two sets of blood samples, intravenous administration of ampicillin and gentamicin, and continuous infusion of carperitide at a dose of 0.1 ␮g/kg/min were begun. Multiple blood cultures identified Streptococcus sanguis. The course of intravenous antibiotics was continued for 6 weeks, with a good clinical response. He was then transferred to Juntendo University Hospital for mitral valve replacement. Before the operation, coronary angiography demonstrated that the distal left anterior descending artery was totally occluded (Fig. 2b). The diagnosis was embolic AMI and mitral regurgitation associated with IE. The surgical procedure is briefly described here. Standard cardiopulmonary bypass with mild hypothermia was used. After establishing complete cardiac arrest with blood cardioplegia, both atrial incisions were made, and the mitral and tricuspid valves were evaluated. The mitral valve had fragile, easily mobilized vegetations (Fig. 4). The mitral valve was completely excised and replaced with an ATS 27 mm mechanical valve. The multiple vegetations on the left atrial endocardium were also widely debrided and left ventricular aneurysmectomy was performed at the area of myocardial infarction. The postoperative course was uneventful. After the surgery, the patient was treated with ampicillin and gentamycin intravenously for 2 weeks. His symptoms sig-

Systemic embolism occurs in 22—50% of patients with IE, the majority (up to 65%) in the central nervous system, but other major arterial beds may be involved, including the coronary arteries. Most coronary embolisms occur in the left ascending coronary artery (13 of 14 cases) because of the downward course of the left ascending coronary artery compared with the right coronary artery or left circumflex artery, which originate at 90◦ to the aortic cusp [5]. Septic emboli are more frequent with mitral valve infection (25%) than with aortic valve infection (10%) [6]. We believe that the present case of AMI was caused by embolism from IE of the mitral valve. The excised myocardium did not involve the left coronary artery. Therefore we could not confirm the vegetation origin of the coronary embolism by pathological examination. However, we identified systemic embolization in the patient, and coronary angiography clearly showed the typical appearance of embolization in the left coronary artery (Fig. 2b). Coronary angiography can establish the diagnosis of septic emboli in the coronary artery. However, contact between the catheter and the valve surface with vegetation may release systemic emboli. Therefore coronary angiography in patients with IE was reported to be safe if no vegetation is observed on the aortic valve [7]. But percutaneous intervention is not the definitive therapeutic strategy. The indication mainly depends on the infarct size and the grade of congestive heart failure due to myocardial infarction itself because another embolic complication might be induced by the catheterization. Balloon or stent procedures may allow mycotic aneurysm to develop at the site, resulting in complications including coronary rupture or sudden death [8]. Furthermore, evaluation of the cerebral embolism is essential. Preoperative cerebral embolism requires modification of the timing of surgery. The rate of exacerbation of cerebral complications decreased to 10% in patients who underwent surgical treatment more than 15 days after cerebral infarction, and to 2.3% in those who underwent surgical treatment more than 4 weeks later [9]. In this case, we decided to continue medical treatment because the infarct area was relatively small [distal left anterior descending artery occlusion; ejection fraction 52% (Simpson method); and maximum creatine phosphokinase level, 890 IU]. Surgical embolectomy using direct coronary incision is possible if the patient is in unstable condition, and was successful in one case [10]. And a possible demerit of the conservative strategy might be an increased risk of bacterial myocarditis followed by cardiac rupture [11].

e32 However, surgical treatment carries high operative risk in this case. We reported the rare case of a patient with IE had developed AMI. If the patient with fever of unknown origin has chest pain, the possibility of IE in patients with AMI should be considered, and the dissemination of septic emboli should be evaluated including before coronary angiography. Especially, the evaluation of the cerebral embolism is essential. The therapeutic strategy depends on the clinical manifestations or signs of systemic embolism.

References [1] Thuny F, Di Salvo G, Belliard O, Avierinos JF, Pergola V, Rosenberg V, Casalta JP, Gouvernet J, Derumeaux G, Iarussi D, Ambrosi P, Calabró R, Riberi A, Collart F, Metras D, et al. Risk of embolism and death in infective endocarditis: prognostic value of echocardiography: a prospective multicenter study. Circulation 2005;112:69—75. [2] Takaya T, Takeuchi Y, Okamoto M, Hata K, Kojima Y, Nakajima K, Kita T, Ito M, Nakajima H, Takaoka R, Nomura T, Iwahashi K, Kawashima S, Seo T. Mitral regurgitation resulting from the consecutive multiple perforations by infective endocarditis mimicking the isolated anterior mitral cleft. J Cardiol 2008;52:159—62. [3] Herzog CA, Henry TD, Zimmer SD. Bacterial endocarditis presenting as acute myocardial infarction: a cautionary note for the era of reperfusion. Am J Med 1991;90:392—7.

I. Okai et al. [4] Tiurin VP, Korneev NV. The mechanisms of the development and diagnosis of myocardial infarct in septic endocarditis. Ter Arkh 1992;64:55—8 (in Russian). [5] Manzano MC, Vilacosta I, San Román JA, Aragoncillo P, Sarriá C, López D, López J, Revilla A, Manchado R, Hernández R, Rodríguez E. Acute coronary syndrome in infective endocarditis. Rev Esp Cardiol 2007;60:24—31 (in Spanish). [6] Bayer AS, Bolger AF, Taubert KA, Wilson W, Steckelberg J, Karchmer AW, Levison M, Chambers HF, Dajani AS, Gewitz MH, Newburger JW, Gerber MA, Shulman ST, Pallasch TJ, Gage TW, et al. Diagnosis and management of infective endocarditis and its complications. Circulation 1998;98:2936—48. [7] Welton DE, Young JB, Raizner AE, Ishimori T, Adyanthaya A, Mattox KL, Chahine RA, Miller RR. Value and safety of cardiac catheterization during active infective endocarditis. Am J Cardiol 1979;44:1306—10. [8] McGee MB, Khan MY. Ruptured mycotic aneurysm of a coronary artery. A fatal complication of Salmonella infection. Arch Intern Med 1980;140:1097—8. [9] Eishi K, Kawazoe K, Kuriyama Y, Kitoh Y, Kawashima Y, Omae T. Surgical management of infective endocarditis associated with cerebral complications. Multi-center retrospective study in Japan. J Thorac Cardiovasc Surg 1995;110:1745—55. [10] Beak MJ, Kim HK, Yu CW, Na CY. Mitral valve surgery with surgical embolectomy for mitral valve endocarditis complicated by septic coronary embolism. Eur J Cardiothorac Surg 2008;33:116—8. [11] LeLeiko RM, Bower DJ, Larsen CP. MRSA-associated bacterial myocarditis causing ruptured ventricle and tamponade. Cardiology 2008;111:188—90.

Journal of Cardiology Cases (2010) 1, e33—e36

available at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/jccase

Case Report

A case of newly demonstrated coronary spasm 4 months after paclitaxel-eluting stent implantation for in-stent restenosis Keisuke Watanabe (MD) ∗, Natsuki Nakamura (MD), Koichi Kikuta (MD, FJCC), Junichi Matsubara (MD), Eisaku Okuyama (MD), Tetsuji Katayama (MD) Division of Cardiology, Shinbeppu Hospital, 3898 Ooaza Tsurumi, Beppu City, Oita 874-0833, Japan Received 3 March 2009; received in revised form 8 June 2009; accepted 16 June 2009

KEYWORDS Drug-eluting stent; Coronary spasm; Endothelial dysfunction

Summary A 56-year-old woman with hypertension and hypercholesterolemia was admitted to our hospital with acute inferior myocardial infarction. The patient had total occlusion of the right coronary artery (RCA) segment 2, and bare-metal stents were placed. Four months later, plain old balloon angioplasty was performed for in-stent restenosis. Follow-up coronary angiography (CAG) 6 months later showed in-stent total occlusion, so a stent-in-stent procedure was performed using paclitaxel-eluting stents (PESs). Four months later, the patient began complaining of early morning chest pain at rest. CAG showed no in-stent restenosis, so coronary spastic angina was suspected. Intracoronary infusion of ergonovine to the right and left coronary arteries revealed spasm of the RCA with total occlusion just proximal to the PES in segment 1. Her chest pain was reproduced with ST-elevation in leads II, III, and aVF, so the diagnosis of coronary spastic angina was made. Treatment with a Ca-channel blocker and nitrates relieved the symptoms. The PES was the probable cause of the coronary spasm. © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.

Case report A 56-year-old woman with hypertension and hypercholesterolemia was admitted to our hospital in September 2006 with an acute inferior myocardial infarction. She had menopause when she was 43 years old. Coronary

∗ Corresponding author. Tel.: +81 9 7722 0391; fax: +81 9 7722 1151. E-mail address: [email protected] (K. Watanabe).

angiography (CAG) showed total occlusion of the right coronary artery (RCA) segment 2. A Multilink Vision stent (3.5 mm × 18 mm; Abbott, Tokyo, Japan) and a Driver stent (3.5 mm × 24 mm; Medtronic, Minneapolis, MN, USA) were overlapped and placed. The left circumflex artery (LCX) was also significantly stenosed, so sirolimus-eluting stents (SESs) (Cypher; Johnson & Johnson, Cordis Corporation, Miami, FL, USA) were placed in segment 11 (3.5 mm × 18 mm) and segment 13 (2.5 mm × 18 mm) before hospital discharge. Four months later, the patient was hospitalized for exertional chest pain. CAG showed in-stent restenosis of segment 2, so plain old balloon angioplasty (POBA) was performed. Six

1878-5409/$ — see front matter © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.jccase.2009.06.006

e34

K. Watanabe et al.

Figure 1 Third percutaneous coronary intervention. (A) Coronary angiography shows in-stent total occlusion in the right coronary artery. (B) Paclitaxel-eluting stents (PESs) were placed in the bare-metal stents (BMSs). The BMSs were fully covered by the PESs. A PES was also placed at the segment 3 90% new lesion (a: Multilink Vision stent; b: Driver stent; c, d, and e: PES).

months later, follow-up CAG revealed in-stent total occlusion of segment 2 (Fig. 1A). Because this was the second restenosis, a stent-in-stent procedure using a paclitaxeleluting stent (PES; Taxus, Boston Scientific, San Diego, CA, USA) was performed. Two PESs (3.5 mm × 24 mm) were overlapped and placed, completely covering the bare-metal stent (BMS). After reperfusion, a new 90% lesion in segment 3 was detected, so a PES (3.0 mm × 12 mm) was placed. The procedure was completed with good results (Fig. 1B).

However, 4 months later, the patient experienced early morning chest pain at rest for the first time. We performed CAG without an exercise test and without stopping oral vasoactive agents before CAG, because we were concerned about the possibility of unstable angina due to the presence of resting chest pain. CAG showed 75% stenosis just proximal to the PES in segment 1 (Fig. 2C). No other significant lesions were present to account for the resting chest pain. Thus, a spasm provocation test using ergonovine was per-

Figure 2 Left coronary angiograms both (A) pre- and (B) post-ergonovine infusion (a and b: sirolimus-eluting stent). (C) Right coronary angiogram pre-ergonovine infusion. (D) Total occlusion of segment 1 is observed after infusion. (E) Coronary spasm remains just proximal to the paclitaxel-eluting stent (PES) in segments 1 and 3 and just distal to the PES in segment 2 after infusion of isosorbide dinitrate 2.5 mg (arrows). (F) Following additional intracoronary infusion of isosorbide dinitrate, the coronary spasm has resolved completely.

A case of newly demonstrated coronary spasm associated with PES formed. First, ergonovine was infused into the left coronary artery at a rate of 8 ␮g/min for 5 min, but only slight vasoconstriction was noted (Fig. 2A and B). Then, ergonovine was similarly infused into the RCA, and within 4 min, the patient began to complain of her usual chest pain with ST-elevation in leads II, III, and aVF. CAG revealed that the stenotic lesion of segment 1 was totally occluded (Fig. 2D). Though intracoronary infusion of 2.5 mg of isosorbide dinitrate achieved reperfusion, coronary spasm remained just proximal to the PES in segment 1 and segment 3 and just distal to the PES in segment 2 (Fig. 2E). Again, with intracoronary infusion of 2.5 mg of isosorbide dinitrate, the coronary spasm, including the chest pain and the electrocardiogram changes, resolved completely (Fig. 2F). The patient had taken aspirin 200 mg/day, ticlopidine 200 mg/day, amlodipine 5 mg/day, bisoprolol 5 mg/day, lisinopril 10 mg/day, and atorvastatin 10 mg/day. Because coronary spasm was demonstrated, long-acting isosorbide dinitrate 40 mg/day and benidipine 8 mg/day were added. On follow-up, the patient has had no recurrence of chest pain at rest.

Discussion A case of newly demonstrated coronary spasm 4 months after PES implantation was described. Ergonovine did not provoke coronary spasm in the LCX with SES, but clearly provoked it with total occlusion just proximal to the PES in the RCA.

Table 1

Although drug-eluting stents (DES) effectively prevent restenosis and are now widely used in percutaneous coronary intervention (PCI), late stent thrombosis has been reported as a major concern of DES implantation [1]. As the cause, delayed endothelialization has been reported in DES cases as compared to BMS cases from a histopathological perspective [2]. Furthermore, as compared to BMS, endothelial dysfunction has been more often reported with DES [3,4]. The underlying mechanism of endothelial dysfunction due to DES includes: (1) direct endothelial injury and hypersensitivity reactions to the stent system (drug and polymer coating) and (2) delayed recovery of endothelial function due to delayed re-endothelialization [5]. Coronary spastic angina was first reported by Prinzmetal et al. [6] in 1959 as variant angina. During the 1970s, coronary artery spasm as the underlying etiology was documented by CAG [7]. A major cause of coronary artery spasm is endothelial cell dysfunction due to a variety of factors, such as oxidative stress, endothelial nitric-oxide synthase (e-NOS) gene polymorphism, and chronic inflammation [8]. Consequently, this has been raising concerns about coronary spasm due to endothelial injury associated with DES implantation, and there have actually been some reports of newly demonstrated coronary spasm after DES implantation. Based on our literature search, 13 cases of coronary spasm after SES or PES implantation, including our case, have been reported (Table 1) [5,9—11]. Coronary spasm in all 3 branches has been described, and time of onset has varied from during PCI to 6 months post-PCI. The site of coronary spasm also varies from diffuse to proximal or distal to the stent.

Case reports of coronary spasm associated with drug-eluting stent implantation.

Case

Type of stent

Indication for stent

Location of stent

Present report El-Bialy et al. [9]

PES PES

AP uAP

ISR in RCA LAD

Kim et al. [10]

PES

Acute MI

Maekawa et al. [11] SES Brott et al. [5] SES

Recent MI uAP

Brott et al. [5] Brott et al. [5] Brott et al. [5]

SES SES SES

AP Acute MI uAP

Brott Brott Brott Brott

[5] [5] [5] [5]

SES PES PES SES

N/A N/A N/A AP

Brott et al. [5]

SES

N/A

et et et et

e35

al. al. al. al.

Method of spasm identification

CAG Chest pain, ECG, CAG (patent stent) RCA, LAD, LCX Sudden cardiac arrest CAG LAD CAG LAD & diagonal Chest pain, ECG, CAG ISR in LAD Chest pain, CAG LAD CAG LCX distal Chest pain, ECG, CAG LAD via LITA CAG LAD CAG RCA, OM CAG LAD MI, chest pain, ECG, CAG (patent stents) RCA Chest pain, CAG (patent stents)

Location of spasm

Time to spasm (after PCI)

RCA stent proximal Unknown

4 months Same day

Diffuse narrowing of three coronary arteries LAD stent distal Diffuse, LAD&LCX

10 h 6 months 3h

Diffuse LAD&LCX Distal to stent Proximal to stent

1 week 4 weeks 12 h

Proximal and distal Entire LAD&LCX RCA, OM Unknown

3h During procedure During procedure 11 days

Unknown

11 days

AP, angina pectoris; CAG, coronary angiography; ECG, electrocardiogram; ISR, in-stent restenosis; LAD, left anterior descending coronary artery; LCX, left circumflex coronary artery; LITA, left internal thoracic artery; MI, myocardial infarction; N/A, not available; OM, obtuse marginal branch; PCI, percutaneous coronary intervention; PES, paclitaxel-eluting stent; RCA, right coronary artery; SES, sirolimus-eluting stent; uAP, unstable angina pectoris.

e36 Table 1 lists cases of coronary spasm associated with DES implantation, based on the absence of any prior symptoms suggesting coronary spastic angina. The present patient also first experienced early morning chest pain at rest after PES implantation, and, with the spasm provocation test, total occlusion just proximal to the PES in segment 1 was observed. Moreover, the spasm remained just proximal and distal to the PES after infusion of isosorbide dinitrate. These findings suggest coronary spasm related to PES implantation. However, objective evaluation of whether DES implantation indeed caused coronary spasm was limited, because the spasm provocation test (with negative results) was not performed beforehand in any of the cases, including the present case. In the present case, the presence of coronary spasm was confirmed by the spasm provocation test. Therefore, we consider that the spasm provocation test should be done in patients who complain of resting chest pain after DES implantation, although spontaneous attacks may not be proven to be the same as the induced attack. The present patient also had SES in the LCX, but coronary spasm did not develop at this site. There are more case reports of coronary spasm with SES than with PES, but this may be influenced by their period of availability and number used. It would be interesting to know whether SES and PES differ in their ability to provoke spasm. Individual differences in reactivity to drugs may exist. However, the details are unclear, because, unlike the present case, there are no reports that involved implantation of both SES and PES and that mentioned differences between the stents in their ability to provoke spasm. Brott et al. [5] reported, in addition to DES cases, 3 cases of coronary spasm after BMS implantation. Their findings suggest that PCI itself may also cause endothelial injury, leading to coronary spasm. In the present case, the patient had already undergone PCI 3 times for the PES implantation site in segment 2, so the proximal endothelium might have been injured by repeated PCI. In segment 3, however, PCI was performed only once, and PES was easily deployed after balloon predilation. Thus, we consider there was no significant endothelial injury due to PCI itself in segment 3. With the additional oral medications mentioned previously, further chest pain at rest did not develop, so drug therapy provided good control. In conclusion, coronary spasm must always be kept in mind as a potential cause of

K. Watanabe et al. resting chest pain after DES implantation. However, further investigation is necessary to determine whether drug therapy to prevent coronary spasm is required in all patients with DES implantation.

References [1] Sawada T, Shite J, Shinke T, Tanino Y, Ogasawara D, Kawamori H, Kato H, Miyoshi N, Yoshino N, Hirata K-I. Very late thrombosis of sirolimus-eluting stent due to late malapposition: serial observations with optical coherence tomography. J Cardiol 2008;52:290—5. [2] Joner M, Finn AV, Farb A, Mont EK, Kolodgie FD, Ladich E, Kutys R, Skorija K, Gold HK, Virmani R. Pathology of drug-eluting stents in humans: delayed healing and late thrombotic risk. J Am Coll Cardiol 2006;48:193—202. [3] Togni M, Windecker S, Cocchia R, Wenaweser P, Cook S, Billinger M, Meier B, Hess OM. Sirolimus-eluting stents associated with paradoxic coronary vasoconstriction. J Am Coll Cardiol 2005;46:231—6. [4] Hofma SH, van der Giessen WJ, van Dalen BM, Lemos PA, McFadden EP, Sianos G, Ligthart JMR, van Essen D, de Feyter PJ, Serruys PW. Indication of long-term endothelial dysfunction after sirolimus-eluting stent implantation. Eur Heart J 2006;27:166—70. [5] Brott BC, Anayiotos AS, Chapman GD, Anderson PG, Hillegass WB. Severe, diffuse coronary artery spasm after drug-eluting stent placement. J Invasive Cardiol 2006;18:584—92. [6] Prinzmetal M, Kennamer R, Merliss R, Wada T, Bor N. A variant form of angina pectoris. Preliminary report. Am Heart J 1959;27:375. [7] Oliva PB, Potts DE, Pluss RG. Coronary arterial spasm in Prinzmetal angina. Documentation by coronary angiography. N Engl J Med 1973;288:745—51. [8] Yasue H, Nakagawa H, Itoh T, Harada E, Mizuno Y. Coronary artery spasm—–clinical features, diagnosis, pathogenesis, and treatment. J Cardiol 2008;51:2—17. [9] El-Bialy A, Shenoda M, Caraang C. Refractory coronary vasospasm following drug-eluting stent placement treated with cyproheptadine. J Invasive Cardiol 2006;18: E95—8. [10] Kim JW, Park CG, Seo HS, Oh DJ. Delayed severe multivessel spasm and aborted sudden death after Taxus stent implantation. Heart 2005;91:e15. [11] Maekawa K, Kawamoto K, Fuke S, Yoshioka R, Saito H, Sato T, Hioka T. Severe endothelial dysfunction after sirolimus-eluting stent implantation. Circulation 2006;113:e850—1.

Journal of Cardiology Cases (2010) 1, e37—e41

available at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/jccase

Case Report

Pulmonary hemorrhage in a middle-aged woman as a complication of treatments for acute myocardial infarction Ryo Ishida (MD) a, Tetsuya Nomura (MD) a,∗, Akiteru Kojima (MD) a, Yota Urakabe (MD) a, Satoko Enomoto (MD) a, Susumu Nishikawa (MD) a, Natsuya Keira (MD) a, Hiroaki Matsubara (MD) b, Tetsuya Tatsumi (MD) a a b

Department of Cardiovascular Medicine, Nantan General Hospital, 25, Yagi-Ueno, Yagi-cho, Nantan City, Kyoto 629-0197, Japan Department of Cardiovascular Medicine, Kyoto Prefectural University School of Medicine, Kyoto, Japan

Received 14 May 2009; received in revised form 30 June 2009; accepted 6 July 2009

KEYWORDS Anticoagulants; Complications; Coronary artery disease

Summary A 49-year-old woman complaining of anterior chest pain underwent emergent coronary angiogram and thrombotic obstruction in the proximal left anterior descending artery was discovered. Deployment of a bare metal stent recovered good coronary flow and congestive heart failure was soon relieved. However, on day 3 of hospitalization, chest radiography suddenly showed newly emergent bilateral pulmonary infiltration shadow mimicking congestive heart failure. Chest computed tomography and clinical findings suggested bilateral alveolar hemorrhage. The patient received dual antiplatelet therapy, aspirin 100 mg/day and clopidogrel 75 mg/day and continuous 15,000 U/day heparin infusion, after percutaneous coronary intervention. Therapies that minimize bleeding risk while maintaining an antithrombotic effect are required for patients with acute coronary syndrome (ACS). Due to concern about the increased risk of early stent thrombosis induced by discontinuation of antiplatelet therapy, we continued to administer dual antiplatelet therapy. Pulmonary hemorrhage complicated with ACS without abciximab is a rare clinical entity, and we successfully overcame this potentially life-threatening complication with conservative therapy. © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.

Introduction

∗ Corresponding author. Tel.: +81 771 42 2510; fax: +81 771 42 2096. E-mail address: [email protected] (T. Nomura).

Currently percutaneous coronary intervention (PCI) is widely performed all over the world, and multiple antiplatelet drugs have become standard therapy for preventing thrombotic events after PCI. However, bleeding complications during the treatment for acute coronary syndrome (ACS) is a relatively frequent non-cardiac event and sometimes asso-

1878-5409/$ — see front matter © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.jccase.2009.07.003

e38 ciated with a poor hospital prognosis. Although pulmonary hemorrhage can be easily misdiagnosed as congestive heart failure on chest radiogram, we have to investigate the pathological entity as accurately as possible to achieve a better outcome for these patients.

Case report A 49-year-old medium-sized woman (body mass index = 21.4) who had been receiving medication therapy for diabetes mellitus (DM) consulted the emergency room complaining of sudden onset anterior chest pain in the early morning. Her only coronary risk factor was DM. Her blood pressure was 145/92 mmHg, and the pulse rate was 54/min with regular rhythm. A 12-lead electrocardiography demonstrated an elevated ST-segment in a wide range of anterior chest leads and ultrasound cardiography showed akinesis of broad apicoanterior wall of left ventricle. Laboratory study showed typical ACS findings of acute phase (Table 1). She was highly suspected of having acute apico-anterior myocardial infarction and underwent emergent coronary angiogram. Proximal left anterior descending artery (LAD) was totally occluded by thrombus (Fig. 1A) and PCI was continuously performed. During the PCI procedure, acute pulmonary congestion worsened rapidly and cardiogenic shock developed (Fig. 2A). The patient was intubated and artificial ventilation was started. Successful aspiration of red thrombus achieved LAD recanalization and demonstrated moderate LAD stenosis (Fig. 1B, Arrow). Then, bare metal stent deployment promptly achieved thrombolysis in myocardial infarction grade 3 flow under intra-aortic balloon pumping (IABP) support (Fig. 1C). The patient was admitted to the coronary care unit. She received aspirin 200 mg and clopidogrel 300 mg loading dose in the emergency room. After admission, she received aspirin 100 mg/day and clopidogrel 75 mg/day from the stomach tube. Unfractionated heparin 15,000 U/day was also continuously dripped. Medication for congestive heart failure effectively improved her respiratory state. Chest radiography on day 2 of hospitalization showed remarkable improvement of pulmonary congestion compared with that on admission (Fig. 2B). However, on day 3 of hospitalization her blood oxygenation suddenly decreased and chest radio-

R. Ishida et al. Table 1

Laboratory data on admission

WBC RBC Hgb Hct PLT BUN Cre Na K Cl Amy CPK AST ALT

10610/␮l 3.95 × 106 /␮l 11.7 g/dl 36.5% 25.3 × 104 /␮l 26.4 mg/dl 1.17 mg/dl 138 mequiv./l 3.9 mequiv./l 106 mequiv./l 78 IU/l 217 U/l 74 U/l 84 U/l

LDH ALP T-Bil CRP BS TP Alb TG HDL-C LDL-C TropT PT APTT Fibrinogen

297 U/l 655 U/l 0.2 mg/dl 0.1 mg/dl 195 mg/dl 6.8 g/di 3.6 g/di 52 mg/dl 39.4 mg/dl 104.0 mg/dl Positive 113% 24.9 s 440 mg/dl

Alb, albumin; Amy, amylase; AST, aspartate aminotransferase; ALP, alkaline phosphatase; ALT, alanine aminotransferase; APTT, activated partial thromboplastin time; BS, blood sugar; BUN, blood urea nitrogen; CPK, creatine phosphokinase; Cre, serum creatinine; CRP, c-reactive protein; Hct, hematocrit; HDL-C, high density lipoprotein cholesterol; Hgb, hemoglobin; LDH, lactate dehydrogenase; LDL-C, low density lipoprotein cholesterol; PLT, platelet count; PT, ptothrombin time; RBC, red blood cell count; T-Bil, total bilirubin; TG, triglyceride; TP, total protein; TropT, troponin T; WBC, white blood cell count.

graphy demonstrated newly emergent bilateral pulmonary infiltration shadow (Fig. 2C). Chest computed tomography showed a ground-glass appearance with partial consolidation at the bilateral posterior lung field (Fig. 3). At the same time, some fresh blood was aspirated through the intubated tube, and her hemoglobin decreased from 10.5 mg/dl to 8.4 mg/dl. Findings suggested that her clinical pathology was derived from pulmonary hemorrhage rather than worsening of congestive heart failure. The activated coagulation time was measured as 170 s and platelet number was decreased to 58,000/␮l. Therefore, we ceased continuous heparin infusion and discontinued IABP mechanical support. We then tried conservative treatment for pulmonary hemorrhage with continuous infusion of carbazochrome and tranexamic acid, and transfusion with 2 units of red cell concentrates mannitol adenine phosphate. Considering the patient’s cardiac state, we continued dual antiplatelet

Figure 1 Emergent coronary angiogram showed obstruction of proximal left anterior descending artery (A). After aspiration of thrombus, stenotic lesion was shown in the mid-left anterior descending artery (B, arrow). Deployment of bare metal stent recovered thrombolysis in myocardial infarction grade 3 coronary flow and the procedure was successfully finished (C).

Pulmonary hemorrhage complicated with acute myocardial infarction

e39

Figure 2 Transition of images on chest radiogram. Severe pulmonary congestion was seen on coronary care unit admission (A). Pulmonary congestion quickly recovered on day 2 of hospitalization (B). Bilateral infiltration shadow appeared on day 3 (C).

Figure 3 Chest computed tomography demonstrated bilateral ground-glass appearance with partial consolidation predominantly at the lower posterior lung field on day 3 of hospitalization. Images show the horizontal section just above the carina (A), below the carina (B), and at the level of mid-left ventricle (C).

therapy. Because of the persistence of a low PaO2 /FiO2 (P/F ratio) score, we started sivelestat infusion and her respiratory state dramatically improved. She was extubated on day 8 of hospitalization (Fig. 4). Thereafter, she progressed

well without relapse of either pulmonary hemorrhage or congestive heart failure. Cardiac rehabilitation after acute myocardial infarction gradually progressed, and she was finally discharged on day 30 of hospitalization.

Figure 4 Changes in the medical treatment and clinical course during the early stage of hospitalization. CRP, C-reactive protein; IABP, intra-aortic balloon pumping; MAP, mannitol adenine phosphate; P/F ratio, PaO2 /FiO2 ratio; WBC, white blood cells.

e40

Discussion In most cases of PCI in the modern era, stent deployment is a fundamental and very useful strategy. However, once stent thrombosis occurs, catastrophic results often follow. As reported by Schomig et al. [1] and Leon et al. [2], potent antiplatelet therapy is very useful for preventing stent thrombosis. Dual antiplatelet therapy has been reported to reduce the incidence of stent thrombosis to less than 1%. Currently dual antiplatelet therapy is a common strategy for interventional cardiologists after stent deployment, but we must remember that hemorrhagic complications caused by dual antiplatelet therapy occur as frequently as stent thrombosis. Also in cases of ACS, a strategy of clopidogrel pretreatment followed by long-term therapy is reported to be beneficial in reducing major cardiovascular events [3]. That study also reported that there was less need for glycoprotein IIb/IIIa inhibitor in the clopidogrel group and there was no increase in major or life-threatening bleeding with clopidogrel use. However, when PCI is performed, we must always consider the risk of hemorrhagic complication. Major bleeding is a relatively frequent non-cardiac complication of contemporary therapy for ACS and it is associated with a poor hospital prognosis. Advanced age, female sex, history of bleeding, and renal insufficiency were independently associated with a higher risk of bleeding [4]. The GUSTO (Global Use of Strategies To Open Occluded Coronary Arteries) bleeding classification identifies patients who are at risk for short- and long-term adverse events [5]. The reason for the increased occurrence of cardiovascular events related to bleeding complication is that these patients show common risk factors such as advanced age or renal insufficiency as indicated above. Furthermore, to cope with those bleeding complications, we sometimes have to discontinue antiplatelet or anticoagulant drugs, which consequently lead to more frequent thrombotic events. Therapies that minimize bleeding risk while maintaining an anticoagulant effect may improve the outcomes of patients with ACS. Bleeding problems from the gastrointestinal and genitourinary tract or arterial access are easily recognized. However, we must carefully monitor other possible but infrequent hemorrhagic complications. Pulmonary hemorrhage appears as a bilateral alveolar infiltration shadow on chest radiography and can be easily mistaken for acute pulmonary edema in the context of ACS [6]. When the infiltrates do not resolve with administration of drugs for congestive heart failure, cardiologists must be aware of the possibility of pulmonary hemorrhage because early treatment can increase the probability of patient survival. Diffuse pulmonary hemorrhage is a rare event, which has mostly been reported in patients receiving glycogen IIb/IIIa inhibitors in combination with other antiplatelet drugs, such as aspirin, ticlopidine, and clopidogrel. Kalra et al. reported that severe pulmonary hemorrhage was identified in 0.27% patients who received abciximab, while there were no cases of pulmonary hemorrhage among 5412 patients who underwent coronary procedures without abciximab infusion [7]. Clopidogrel use related to alveolar hemorrhage without glycogen IIb/IIIa inhibitors has been infrequently reported

R. Ishida et al. to date. In the first report of this type of pathology, alveolar hemorrhage was controlled by ceasing clopidogrel use [8]. In the other case, the patient developed severe pulmonary hemorrhage and could not be resuscitated [9]. We successfully used the elastase inhibitor sivelestat that is usually applied for the treatment of acute lung injury (ALI). Although our patient did not strictly correspond to the criteria of ALI [10], she showed lower P/F ratio which is often applied for an index of respiratory dysfunction. From that point of view, we hoped the effect of this drug to improve her respiratory state. We presented a case of pulmonary hemorrhage complicated with ACS. Multifactorial causes such as dual antiplatelet therapy, heparin infusion, thrombocytopenia, increased alveolar pressure induced by mechanical ventilation, and assumed pulmonary hypertension due to left ventricular dysfunction, were thought to be involved in the occurrence of pulmonary hemorrhage in our patient. Thrombocytopenia may be related to the pathophysiology of heparin-induced thrombocytopenia (HIT). However, no more information is available because further examination about HIT was not performed. Because our patient overcame her hemorrhagic complication without stopping antiplatelet use, strictly speaking, we cannot determine the direct correlation between antiplatelet therapy and the hemorrhagic event. However, there is no doubt that the use of multiple antiplatelet and anticoagulant drugs is involved in the increased risk of hemorrhagic events. Our patient is a case of relatively broader anterior myocardial infarction with lower cardiac function, which indicated that recurrent infarction caused by early stent thrombosis is quite capable of being fatal. Deciding to stop antiplatelet medicines in such a clinical setting could be fatal. Therefore, we continued dual antiplatelet therapy and were still able to achieve a better outcome. Making a quick and accurate diagnosis of the clinical pathology is of course important, and moreover, we have to fully consider the patient’s background and current condition to determine an appropriate treatment policy.

References [1] Schomig A, Neumann FJ, Kastrati A, Schuhlen H, Blasini R, Hadamitzky M, Walter H, Zitzmann-Roth EM, Richardt G, Alt E, Schmitt C, Ulm K. A randomized comparison of antiplatelet and anticoagulant therapy after the placement of coronary-artery stents. N Engl J Med 1996;334:1084—9. [2] Leon MB, Baim DS, Popma JJ, Gordon PC, Cutlip DE, Ho KK, Giambartolomei A, Diver DJ, Lasorda DM, Williams DO, Pocock SJ, Kuntz RE. A clinical trial comparing three antithrombotic-drug regimens after coronary-artery stenting. Stent Anticoagulation Restenosis Study Investigators. N Engl J Med 1998;339:1665—71. [3] Mehta SR, Yusuf S, Peters RJ, Bertrand ME, Lewis BS, Natarajan MK, Malmberg K, Rupprecht H, Zhao F, Chrolavicius S, Copland I, Fox KA, Clopidogrel in Unstable angina to prevent Recurrent Events trial (CURE) Investigators. Effects of pretreatment with clopidogrel and aspirin followed by long-term therapy in patients undergoing percutaneous coronary intervention: the PCI-CURE study. Lancet 2001;358:527—33. [4] Moscucci M, Fox KA, Cannon CP, Klein W, Lopez-Sendon J, Montalescot G, White K, Goldberg RJ. Predictors of major bleeding in acute coronary syndromes: the Global Registry of Acute Coronary Events (GRACE). Eur Heart J 2003;24:1815—23.

Pulmonary hemorrhage complicated with acute myocardial infarction [5] Rao SV, O’Grady K, Pieper KS, Granger CB, Newby LK, Van de Werf F, Mahaffey KW, Califf RM, Harrington RA. Impact of bleeding severity on clinical outcomes among patients with acute coronary syndromes. Am J Cardiol 2005;96: 1200—6. [6] Fernandez-Perez GC, Vazquez M, Delgado C, Velasco M, Vazquez-Lima A, Rodriguez-Perez J. Pulmonary hemorrhage in a patient with acute coronary syndrome. Am J Roentgenol 2007;189:W135—7. [7] Kalra S, Bell MR, Rihal CS. Alveolar hemorrhage as a complication of treatment with abciximab. Chest 2001;120:126—31.

e41

[8] Kilaru PK, Schweiger MJ, Kozman HA, Weil TR. Diffuse alveolar hemorrhage after clopidogrel use. J Invasive Cardiol 2001;13:535—7. [9] Gill DS, Ng K, Ng KS. Massive pulmonary haemorrhage complicating the treatment of acute coronary syndrome. Heart 2004;90:e15. [10] Bernard GR, Artigas A, Brigham KL, Carlet J, Falke K, Hudson L, Lamy M, Legall JR, Morris A, Spragg R. The American-European Consensus Conference on ARDS. Definitions, mechanisms, relevant outcomes, and clinical trial coordination. Am J Respir Crit Care Med 1994;149:818—24.

Journal of Cardiology Cases (2010) 1, e42—e44

available at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/jccase

Case Report

Right ventricular rupture induced by cardiopulmonary resuscitation Masahiro Natsuaki (MD) a,∗, Ayumu Yamasaki (MD) a, Noritsugu Morishige (MD) b, Masaru Nishimi (MD) b, Masanori Okabe (MD) a, Yusuke Yamamoto (MD) a a b

Department of Cardiology, Saiseikai Fukuoka General Hospital, 1-3-46 Tenjin Chuo-ku, Fukuoka 810-0001, Japan Department of Cardiovascular Surgery, Fukuoka University Hospital, Fukuoka, Japan

Received 13 March 2009; received in revised form 30 June 2009; accepted 3 July 2009

KEYWORDS Right ventricular rupture; Cardiac tamponade; Cardiopulmonary resuscitation

Summary Right ventricular rupture is a rare complication of cardiopulmonary resuscitation and could be fatal. We report a survival case of right ventricular rupture induced by cardiopulmonary resuscitation in a patient with acute myocardial infarction. A 57-year-old man was admitted to our hospital with ventricular fibrillation. Although chest compression and defibrillation were performed, ventricular fibrillation continued. We inserted a percutaneous cardiopulmonary system and performed coronary angiography, which revealed occlusion of the left anterior descending artery. After coronary stenting and intra-aortic balloon pumping, we succeeded in defibrillation and vital signs became stable. Twenty hours after the intervention, systolic blood pressure dropped to 60 mmHg. Ultrasonic cardiogram at that time revealed massive pericardial effusion. We diagnosed cardiac tamponade, and 8Fr drainage tube was placed in the pericardial space. We determined that emergent operation was necessary because we suspected left ventricular rupture due to acute myocardial infarction or coronary rupture induced by percutaneous coronary intervention. However, operative findings revealed right ventricular free wall rupture, which could have been induced by chest compression. In these cases, we should consider the possibility of not only the rupture of left ventricle and coronary artery but also the rupture of right ventricle induced by cardiopulmonary resuscitation. © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.

Introduction

∗

Corresponding author. Tel.: +81 92 771 8151 fax: +81 92 716 0185. E-mail address: [email protected] (M. Natsuaki).

Right ventricular (RV) rupture is one of the rare complications of cardiopulmonary resuscitation (CPR), and could be fatal. We report a survival case of RV rupture induced by CPR in a patient with acute myocardial infarction (AMI).

1878-5409/$ — see front matter © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.jccase.2009.07.006

Right ventricular rupture induced by cardiopulmonary resuscitation

e43

Case report A 57-year-old man (height: 177 cm; body weight: 61 kg) experienced a ventricular fibrillation (VF) attack in his car while waiting for a traffic signal. An ambulance was called and emergency services arrived about 5 min later. Although chest compression and defibrillation were performed, VF continued. The ambulance arrived at our hospital 20 min after cardiopulmonary arrest. We continued CPR along with the advanced cardiopulmonary life support protocol, but VF persisted. We promptly inserted a percutaneous cardiopulmonary system (PCPS) under the guidance of fluoroscopy (total CPR time was about 35 min) and performed coronary angiography (CAG) which revealed occlusion of the left anterior descending artery (LAD). After successful reperfusion by coronary stenting to the LAD and intra-aortic balloon pumping (IABP), we tried defibrillation again. Then, the rhythm came back to sinus rhythm. Under the support of PCPS, IABP, and intravenous dopamine (5 ␮g/kg/min), vital signs became stable (systolic blood pressure was approximately 100 mmHg). Ultrasonic cardiogram (UCG) just after percutaneous coronary intervention (PCI) revealed wall motion abnormality in the anteroseptal—apical area, and slight pericardial effusion. However, systolic blood pressure was gradually decreasing from 15 h after PCI. Twenty hours after PCI, systolic blood pressure dropped to 60 mmHg and heart rate rose to 120 beats/min. UCG at that time revealed massive pericardial effusion, so we diagnosed cardiac tamponade. However, there were not any apparent leak flows. Pericardial effusion could have accumulated gradually after the recovery of hemodynamics. An 8Fr drainage tube was placed in the pericardial space, and 330 ml of bloody fluid was withdrawn. Discharge from the drainage tube continued and approximately 100 ml was withdrawn during the next 1 h. We determined that emergent operation was necessary because we suspected left ventricular oozing rupture due to AMI or coronary rupture induced by PCI. However, operative findings revealed subcutaneous and pericardial hematoma induced by CPR and RV free wall rupture (Figs. 1 and 2). No apparent sternum and rib fractures were found. The laceration was about 1.5 cm and placed at the anterior wall of RV. It was sutured by 4-0 PROLENETM (Ethicon, Inc., Somerville, NJ) (Fig. 3). Two days after the operation, cardiac function ameliorated (cardiac index was 3.6 L/min/m2 and pulmonary artery wedge pressure was 13 mmHg), and PCPS was removed. IABP and pericardial drainage tube were also removed at post-operative days 3 and 6, respectively.

Figure 1 ation.

Right ventricular rupture was observed at the oper-

fluoroscopy. The site of RV rupture was under the sternum and placed between the sternum and vertebra. For these reasons, traumatic injury induced by CPR was the most suspicious cause of RV rupture. The causes of cardiac rupture by CPR are reported to be direct compression of the chest, compression between the sternum and vertebral column, injuries induced by sternum or rib fractures [4], and so on. In this case, no apparent fractures were found. Therefore,

Discussion The most frequent complication of CPR is skeletal injury, especially fracture of the rib and sternum. But severe lifethreatening complications are rare. Krischer et al. reported the prevalence of life-threatening complications as less than 0.5% [1]. RV rupture is exceedingly rare and fatal [2,3]. The causes of RV rupture are as follows: RV infarction due to right coronary artery occlusion; perforation induced by catheter placed in RV; and trauma. Emergent CAG showed that neither right coronary artery nor RV branch perfusion was hampered at all. Furthermore, neither guide wire nor catheter was inserted into the RV under the guidance of

Figure 2

Enlarged image of the laceration.

e44

M. Natsuaki et al. with the flow of blood from the right ventricle [5]. In this case, there was no apparent cause for obstruction of RV outflow. We suppose that the position of chest compression was higher than recommended which closed the right ventricular outflow tract, and at the same time, the tricuspid valve was closed by the pressure. This situation might have caused RV rupture. Optimal position of chest compression could have prevented this critically hard complication of CPR. In AMI cases with acute cardiac tamponade, LV rupture usually comes as the first cause and the next might be coronary rupture due to PCI. There was no apparent extravasation just after PCI, so the coronary rupture was not so suspicious. We considered performing CAG or left ventriculography (LVG) for diagnosis, but we judged that operation should be taken immediately. Operative findings unexpectedly revealed RV rupture as a cause of cardiac tamponade. If we had performed only CAG or LVG before the operation, we could have failed to diagnose and delayed the operation. In these cases, we should consider the possibility of not only the rupture of LV and coronary artery but also the rupture of RV induced by CPR.

References

Figure 3

The laceration was sutured.

compression due to CPR might be the cause of RV rupture. The mechanism of RV rupture without fractures might be the result from trapping of blood in the right ventricle at levels of systemic arterial pressure. Massive bilateral pulmonary embolism is reported to be one of the causes of RV rupture induced by chest compression because of reducing the caliber of the pulmonary artery and thereby interfering

[1] Krischer JP, Fine EG, Davis JH, Nagel EL. Complications of cardiac resuscitation. Chest 1987;92:287—91. [2] Stefan J, Gregora Z, Krumulova V. Traumatic complications of indirect cardiac massage. Soud Lek 1989;34:49—53. [3] Sokolove PE, Willis-Shore J, Panacek EA. Exsanguination due to right ventricular rupture during closed-chest cardiopulmonary resuscitation. J Emerg Med 2002;23:161—4. [4] Machii M, Inaba H, Nakae H, Suzuki I, Tanaka H. Cardiac rupture by penetration of fractured sternum: a rare complication of cardiopulmonary resuscitation. Resuscitation 2000;43: 151—3. [5] Baldwin JJ, Edwards JE. Rupture of right ventricle complicating closed chest cardiac massage. Circulation 1976;53: 562—4.

Journal of Cardiology Cases (2010) 1, e45—e48

available at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/jccase

Case Report

Preservation of neurological function following therapeutic hypothermia in a patient of in-hospital cardiac arrest with non-ventricular fibrillation Yuichiro Arima (MD) a, Sunao Kojima (MD, PhD) a,∗, Kenichi Kusuhara (MD) a, Yasuhiro Nagayoshi (MD, PhD) a, Hiroaki Kawano (MD, PhD) b, Koichi Kaikita (MD, PhD) a, Seigo Sugiyama (MD, PhD) a, Yoshihiro Kinoshita (MD, PhD) b, Hisao Ogawa (MD, PhD, FJCC) a a b

Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan Department of Intensive Care Unit, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

Received 10 March 2009; received in revised form 5 July 2009; accepted 7 July 2009

KEYWORDS Cardiac arrest; Cardiopulmonary resuscitation; Heart failure; Hypothermia; Pulseless electrical activity; Return of spontaneous circulation

Summary A 76-year-old woman with a diagnosis of dilated-phase hypertrophic cardiomyopathy was admitted to our hospital for exacerbation of congestive heart failure. After admission, she developed cardiac arrest and the electrocardiogram showed pulseless electrical activity. Cardiopulmonary resuscitation was started immediately; however, return of spontaneous circulation was achieved 56 min after cardiopulmonary arrest. Therapeutic hypothermia was considered as an adjunct therapy, together with intensive treatment. The target temperature of 33.0 ◦ C was achieved 10 h after cardiopulmonary arrest. Core temperature was maintained between 33.0 and 35.0 ◦ C for 72 h with no cardiac arrhythmia detected during this period. Rewarming was initiated at a rate of 1 ◦ C/day. On day 6, the core temperature returned to 37 ◦ C and recovery of consciousness was achieved on day 9. No impairment of neurological function was noted. She had no heart failure-related symptoms and B-type natriuretic peptide level decreased from 4174 pg/mL on admission to 450 pg/mL at discharge. Therapeutic hypothermia may be a promising post-resuscitation therapy for comatose survivors of in-hospital cardiac arrest with non-ventricular fibrillation leading to improvement in neurological outcome. © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.

Introduction ∗ Corresponding author at: Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto City 860-8556, Japan. Tel.: +81 96 373 5175; fax: +81 96 362 3256. E-mail address: [email protected] (S. Kojima).

Recent reports have demonstrated that paramedics carry out electrical cardioversion by automated external defibrillator and that successful defibrillation can be achieved outside the hospital [1]. The 2005 American Heart

1878-5409/$ — see front matter © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.jccase.2009.07.001

e46

Y. Arima et al.

Figure 1

Electrocardiogram on hospital admission.

Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care recommend mild therapeutic hypothermia for patients with return of spontaneous circulation (ROSC) after out-of-hospital cardiac arrest [2]. However, the initial cardiac rhythm of comatose patients is limited to ventricular fibrillation (VF). We present a patient who developed in-hospital cardiac arrest with non-VF rhythm and achieved ROSC after 1-h standard cardiopulmonary resuscitation (CPR). Therapeutic hypothermia over 6 days, in combination with a series of therapeutic interventions, resulted in full recovery without any neurological deficits.

Case report A 76-year-old woman was diagnosed with hypertrophic cardiomyopathy and atrial fibrillation 14 years prior to the present admission. Her mother and elder sister were also known to have hypertrophic cardiomyopathy. She was treated chiefly with diuretics and warfarin in the outpatient

clinic. Transthoracic echocardiogram showed low left ventricular (LV) ejection fraction, LV dilatation, and progressive LV wall thinning in recent years. Based on these findings, the disease stage was considered the dilated phase of hypertrophic cardiomyopathy. She developed dyspnea associated with congestive heart failure and was treated by intravenous diuretics by the family physician. However, urinary output was limited and systolic blood pressure was 70 mmHg. The symptoms of heart failure deteriorated and she was transferred to the emergency room in our hospital by an ambulance. On admission, blood pressure was 100/70 mmHg and heart rate was 60 beats/min under infusion of dopamine 3␥. Arterial oxygen saturation was 90% during oxygen breathing at 3 L/min through a nasal cannula. The electrocardiogram (ECG) showed sinus rhythm with complete right bundle branch block (Fig. 1) and transthoracic echocardiogram showed LV dilatation and severe diffuse hypokinesis (LV end-diastolic diameter: 69 mm; LV ejection fraction: 20%). During clinical examination, she developed rapid fall in heart rate and hypotension, with imminent cardiogenic

Therapeutic hypothermia in a patient with in-hospital cardiac arrest

Figure 2

Chest X-ray on hospital admission.

shock and she was quickly taken to the cardiac catheterization room. Within minutes, she developed cardiopulmonary arrest and the ECG monitor showed pulseless electrical activity (PEA). According to the protocol of advanced cardiovascular life support, CPR was started immediately together with tracheal intubation, but spontaneous circulation did not resume immediately. ROSC was achieved exactly 56 min after cardiopulmonary arrest, during which a temporary pacing lead, an aortic balloon for intra-aortic balloon pumping, and a central venous catheter were inserted. The patient was subsequently transferred to the intensive care unit (ICU) for close monitoring and treatment. In the ICU, the Glasgow Coma Scale was E1 V1 M1 . Both pupils were dilated (∅ 5 mm) and the light reflex was absent.

e47

Body temperature was 36.2 ◦ C as measured by a probe inserted into the bladder. A plain chest X-ray showed cardiomegaly and pulmonary congestion (Fig. 2). The laboratory tests were performed. Serum creatinine (1.81 mg/dL) and creatine phosphokinase levels (1113 U/L) were increased and the level of B-type natriuretic peptide (BNP) was 4174 pg/mL; however, serum troponin T level was normal (0.03 ng/mL). Therapeutic hypothermia was considered as a curative option for this patient (Fig. 3). Mild to moderate hypothermia was maintained with a water blanket and was combined with continuous hemodiafiltration to treat the associated acute renal failure. The patient was sedated with propofol and fentanyl, and treated with vecuronium bromide to prevent shivering. The target body temperature of 33.0 ◦ C was achieved 10 h after cardiopulmonary arrest. The core temperature was maintained between 33.0 and 35.0 ◦ C for 72 h and no arrhythmia was detected during this period. Re-warming was initiated at a rate of 1 ◦ C/day. During the re-warming phase, atrial fibrillation with tachycardia often occurred, necessitating intravenous infusion of procainamide as well as cardioversion. On day 6, the core temperature returned to 37 ◦ C, thus ending the period of therapeutic hypothermia. At that stage, the intra-aortic balloon pump was removed and the doses of inotropic agents were increased on day 7. On day 8, infusion of the sedative drug was discontinued, the patient was extubated, and she finally regained consciousness (Glasgow come scale: E3 V5 M6 ) on day 9. On day 12, infusion of inotropic agents was gradually decreased and treatment with oral angiotensin-converting enzyme inhibitor, ␤-blocker, and amiodarone was initiated. At that stage, detailed neurological examination showed no functional deficits. She had no heart failure-related symptoms and the

Figure 3 Clinical course. CHDF, continuous hemodiafiltration; DOA, dopamine; DOB, dobutamine; dPAP, diastolic pulmonary arterial pressure; hANP, human atrial natriuretic peptide; IABP, intra-aortic balloon pumping; LVDd, left ventricular diastolic diameter; LVEF, left ventricular ejection fraction; NA, noradrenaline; d/sPAP, diastolic/systolic pulmonary arterial pressure.

e48 BNP level decreased to 450 pg/mL on hospital discharge. The LV end-diastolic dimension at discharge (69 mm) was comparable to that on admission, however LV ejection fraction increased from 20% on admission to 44% at discharge. A close follow-up at the outpatient clinic over the 2 years after discharge showed no recurrence of heart failure.

Discussion Therapeutic hypothermia is recommended for patients with ROSC after out-of-hospital cardiac arrest due to VF and has been reported to improve survival and neurological function [2,3]. One study documented improved metabolic endpoints (lactate and O2 extraction) when comatose patients were cooled after ROSC from out-of-hospital cardiac arrest in which the initial rhythm was PEA/asystole [4]. A small study also reported benefits after therapeutic hypothermia in comatose survivors of non-VF arrest [5]. On the other hand, few studies have reported the benefits for patients with in-hospital cardiac arrest. The largest published inhospital cardiac arrest database by the National Registry of Cardiopulmonary Resuscitation Investigators indicates that pulseless arrest rhythm in adults is typically PEA/asystole associated with preexisting conditions, such as progressive respiratory failure, circulatory shock, or both, compared with VF or pulseless ventricular tachycardia [6]. Furthermore, the reported survival rate to hospital discharge following PEA/asystole is only 11% [6]. The ultimate goal of resuscitation in patients with inhospital cardiac arrest is survival to hospital discharge with good neurological outcome. Interventions likely to improve survival include early recognition, faster and better resuscitation, and stabilization of such patients. The present case developed PEA associated with congestive heart failure and deterioration of LV function. About 1 h passed until the achievement of ROSC although CPR was initiated immediately after cardiac arrest. The reason for the effectiveness of therapeutic hypothermia in this case could not be elucidated. It is questionable whether therapeutic hypothermia is suitable for patients having severe cardiac dysfunction because drastic changes of body temperature may cause arrhythmia, leading to hemodynamic deterioration. Therefore, a gentle protocol on temperature regulation was adopted. Actually, atrial fibrillation with tachycardia often occurred during the re-warming phase and we had trouble ceasing a surge of arrhythmia in this case. This case

Y. Arima et al. may be appropriate for percutaneous cardiopulmonary support (PCPS), but she was a person of advanced age and her hemodynamics could be supported by intra-aortic balloon pumping alone after successful resuscitation. The PCPS has not been managed in the clinical course although we thought about the use of PCPS in this case. However, we actively induced therapeutic hypothermia in addition to mechanical support and aggressive treatment in the hope of improvement of survival and preservation of neurological function. The indication of therapeutic hypothermia is limited according to the 2005 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care [2]. Further evaluation in a large number of patients is required to confirm the beneficial effects of hypothermia in patients who develop in-hospital cardiac arrest with non-VF rhythms and have preexisting severe conditions. Therapeutic hypothermia may be a promising post-resuscitation therapy for comatose survivors of inhospital cardiac arrest, leading to improvement of survival with minimal or no neurological deficits.

References [1] Shimokawahara H, Sonoda M, Tanaka H, Kashima K, Nagayoshi S, Kawasaki D, Ikeda D, Nagano S, Tanaka Y, Nakamura K. A case of transient mid-ventricular ballooning syndrome with a rapid and uncommon recovery. J Cardiol 2009, doi:10.1016/j.jjcc.2008.12.004. [2] 2005 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care. Part 7.5: Post resuscitation support. Circulation 2005; 112:IV-84-8. [3] Bernard SA, Gray TW, Buist MD, Jones BM, Silvester W, Gutteridge G, Smith K. Treatment of comatose survivors of outof-hospital cardiac arrest with induced hypothermia. N Engl J Med 2002;346:557—63. [4] Hachimi-Idrissi S, Corne L, Ebinger G, Michotte Y, Huyghens L. Mild hypothermia induced by a helmet device: a clinical feasibility study. Resuscitation 2001;51:275—81. [5] Bernard SA, Jones BM, Horne MK. Clinical trial of induced hypothermia in comatose survivors of out-of-hospital cardiac arrest. Ann Emerg Med 1997;30:146—53. [6] Nadkarni VM, Larkin GL, Peberdy MA, Carey SM, Kaye W, Mancini ME, Nichol G, Lane-Truitt T, Potts J, Ornato JP, Berg RA for the National Registry of Cardiopulmonary Resuscitation Investigators. First documented rhythm and clinical outcome from in-hospital cardiac arrest among children and adults. JAMA 2006;295:50—7.

Journal of Cardiology Cases (2010) 1, e49—e51

available at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/jccase

Case Report

Idiopathic dissection from left subclavian artery to brachial artery: Spontaneous repair with conservative management Akira Funada (MD) ∗, Hidekazu Ino (MD, FJCC), Noboru Fujino (MD), Kenshi Hayashi (MD), Katsuharu Uchiyama (MD), Eiichi Masuta (MD), Yuichiro Sakamoto (MD), Toshinari Tsubokawa (MD), Akihiko Muramoto (MD), Masakazu Yamagishi (MD, FJCC) Division of Cardiovascular Medicine, Kanazawa University Graduate School of Medicine, 13-1, Takara-machi, 920-8640, Kanazawa, Japan Received 20 April 2009; received in revised form 2 July 2009; accepted 14 July 2009

KEYWORDS Arteries; Angiography; Ultrasonic diagnosis

Summary We report an unusual case of a 58-year-old female with idiopathic dissection of the left subclavian artery to the brachial artery which provoked vessel narrowing in the acute phase and was spontaneously repaired without surgical procedures in the chronic phase. We describe the serial imaging findings of the angiography and ultrasonography which demonstrate restoration of the dissection. In carefully selected patients, conservative management could be an alternative treatment to surgery or stenting with an excellent outcome. © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.

Introduction

Case report

Idiopathic subclavian artery (SA) dissection is very rare. SA dissection has been reported with anomalies of the aortic arch and in an anatomically normal arch following trauma or iatrogenic injury during catheterization. Patients have been reported to present with chest and back pain and sometimes neurological symptoms [1—3].

A 58-year-old woman with hypertension was admitted to our hospital due to easy fatigability of her left arm for 5 months. The symptoms were unstable and intermittent. She had no history of pain in the left shoulder, catheterization, or injury such as from a traffic accident. On physical examination, the pulse in her left arm was weak and faint. Blood pressure in the right arm was 140/80 mmHg, and we were unable to measure blood pressure in the left arm. Her blood fibrin degradation product was 1.4 ␮g/ml and fibrin degradation product D-dimer was 0.4 ␮g/ml, so the possibility of thromboembolism was low. We therefore considered that her symptoms were due to vascular insufficiency.

∗ Corresponding author. Tel.: +81 76 265 2254; fax: +81 76 234 4251. E-mail address: [email protected] (A. Funada).

1878-5409/$ — see front matter © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.jccase.2009.07.002

e50 Three-dimensional computed tomography angiography (3DCT) showed severe stenosis of the left SA, axillary artery, and brachial artery (BA), and a left SA aneurysm at a proximal site (Fig. 1a). Ten days later, left SA angiography

A. Funada et al. demonstrated mild stenosis of the left SA, severe stenosis of the left BA, and a left SA aneurysm at a proximal site (Fig. 1b). Ultrasonography (US) of the left SA to the BA showed an intimal flap and a true-lumen compressed by a thrombosed pseudo-lumen (Fig. 1c and d). She was diagnosed as having a dissection from the left SA to the BA. We selected conservative management with antiplatelet therapy to prevent acute occlusion with close follow-up, because her symptoms were spontaneously improving and her left radial artery became well palpable. Angiographic findings also improved over 10 days. Two weeks later, her symptoms disappeared and the second left SA angiography showed repair of the dissection (Fig. 2a). Simultaneous intravascular US also showed the retraction of thrombosed pseudo-lumen (Fig. 2b). US of the left SA to BA showed retraction of the thrombosed pseudo-lumen and restoration of blood flow (Fig. 2c). The second 3D-CT angiography also demonstrated restored SA dissection, but the SA aneurysm remained (Fig. 2d).

Discussion

Figure 1 First three-dimensional computed tomography angiography showed severe stenosis of the left subclavian artery (SA), axillary artery, and brachial artery (BA) and a left SA aneurysm at a proximal site (a). First left SA angiography demonstrated mild stenosis of the left SA, severe stenosis of the left BA, and a left SA aneurysm at a proximal site (b). Gray scale images of first ultrasonography of the left BA showed an intimal flap (c). Two-dimensional color Doppler images of first ultrasonography of the left BA showed true-lumen compressed by a thrombosed pseudo-lumen (d).

SA dissection is a rare entity and is usually associated with anomalies of the aortic arch, trauma, and vascular catheterization [1]. Surgery and endovascular stent-graft treatment have been reported as conventional treatments of SA dissection [2,4,5]. On the other hand, there have been some reports of successful treatment of vascular dissection with conservative management using heparin and antiplatelets [6]. Recently, a hybrid therapy with a conservative and an interventional approach was also reported for a long dissection from the SA to the BA [7]. In our case, a surgical approach was difficult because of anatomic constraints, and exposure of a large area was required. Bypass surgery by synthetic vascular prosthesis from the left SA to the BA is fraught with the possibility of occlusion, insufficient blood flow of the branch, and neurological disorder. Stenting from the SA to the BA included the possibility of compression, deformation, migration, restenosis, occlusion or additional dissection, and lifelong antiplatelet medication is necessary. There is only a small number of case reports of stenting or surgery for SA dissection because of the rarity of the disease. Long-term prognosis and frequency of complications with these two therapies are unknown at present. Therefore no clear-cut recommendations could be made. At the first angiography, no sign of critical distal ischemia existed, her symptoms were improving and angiographical findings demonstrated that severe stenosis due to dissection was improved from the proximal site. We thought her dissection was in the natural recovery process, so conservative management with close follow-up was selected on the condition that whenever her symptoms deteriorated, emergency surgery could be performed. We used antiplatelet therapy without heparin because there was no critical ischemia [8]. Two weeks later, angiography and echocardiography demonstrated an excellent outcome. In carefully selected patients, conservative management with close follow-up could be an alternative treatment to surgery or stenting with an excellent outcome. Conservative management can be indicated if there is the minimum necessary blood flow and the symptoms are not exacerbated. Spontaneous recovery may be

Spontaneous repair of subclavian artery dissection

e51 expected under these conditions. Although blood pressure and symptoms are of course the most important indices during follow-up, we also considered US useful for the diagnosis and follow-up of dissection, because the dissection can be clearly visualized noninvasively. SA aneurysm is also a rare entity and is usually associated with atherosclerosis, trauma, thoracic outlet syndrome, and infection [9]. The 3D-CT findings of our case suggested that the entry point of dissection was just distal to the takeoff of the left costocervical trunk and extended to the BA, and the entry point of the dissection subsequently became an aneurysm. Therefore, we speculated that idiopathic SA dissection may be one of the possible causes of SA aneurysm.

Conclusions We presented an uncommon case of idiopathic dissection from the SA to the BA which showed spontaneous repair without surgery or stenting. In cases of unstable or intermittent symptoms of the upper limb such as easy fatigability or faint pulse, we must distinguish spontaneous artery dissection without pain or an episode of injury. In patients with dissection without critical ischemia, conservative management with close follow-up could be recommended to avoid unnecessary surgery or stenting. And in such cases, US is useful for the diagnosis and follow-up of the dissection.

References

Figure 2 The second left subclavian artery (SA) angiography showed repair of the dissection (a). Intravascular ultrasonography of the left brachial artery (BA) showed the retraction of thrombosed pseudo-lumen (b). Two-dimensional color Doppler images of the second ultrasonography of the left BA showed retraction of the thrombosed pseudo-lumen and restoration of blood flow (c). Second three-dimensional computed tomography angiography demonstrated restored SA dissection but the SA aneurysm remained (d).

[1] Garewal M, Selhorst JB. Subclavian artery dissection and triple infarction of the nervous system. Arch Neurol 2005;62:1917—9. [2] Ananthakrishnan G, Bhat R, Zealley I. Spontaneous subclavian artery dissection causing ischemia of the arm: diagnosis and endovascular management. Cardiovasc Intervent Radiol 2009;32:326—8. [3] Iwamuro Y, Nakahara I, Tanaka M, Higashi T, Watanabe Y, Harada K, Fujimoto M, Oku T. Occlusion of the vertebral artery secondary to dissection of the subclavian artery case report. Neurol Med Chir (Tokyo) 2005;45:97—9. [4] Ilkay E, Rahman A, Ozdemir H, Ozbay Y, Yavuzskir M, Burma O. Endovascular stent management of acute traumatic subclavian artery occlusion by intimal flap. EJVES Extra 2003;6:32—5. [5] Guhathakurta S, Agarwal R, Borker S, Sherma A. Chronic dissection of the left subclavian artery with pseudocoarctation. Tex Heart Inst J 2003;30:221—4. [6] Frohwein S, Ververis JJ, Marshall JJ. Subclavian artery dissection during diagnostic cardiac catheterization: the role of conservative management. Cathet Cardiovasc Diagn 1995;34:313—7. [7] Collins NJ, Beecroft JR, Horlick EM. Complex right subclavian artery dissection during diagnostic cardiac catheterization. J Invasive Cardiol 2008;20:E61—3. [8] McNeill DH, Dreisbach J, Marsden RJ. Spontaneous dissection of the internal carotid artery: its conservative management with heparin sodium. Arch Neurol 1980;37:54—5. [9] Davidovi´ c LB, Markovi´ c DM, Pejki´ c SD, Kovacevi´ c NS, Coli´ c MM, Dori´ c PM. Subclavian artery aneurysms. Asian J Surg 2003;26:7—11.

Journal of Cardiology Cases (2010) 1, e52—e55

available at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/jccase

Case Report

Acute coronary syndrome due to coronary artery compression by a metastatic cardiac tumor Takefumi Ozaki (MD, PhD) a,∗, Satoru Chiba (MD, PhD) b, Kazuya Annen (MD, PhD) c, Yuji Kawamukai (MD, PhD) c, Nobuyuki Kohno (RT) d, Masashi Horimoto (MD, PhD, FJCC) b a

Division of Cardiology, Hakodate Chuoh Hospital, Honcho 33-2, Hakodate City, Hokkaido 040-8585, Japan Division of Cardiology, Chitose City Hospital, Chitose City, Hokkaido, Japan c Division of Surgery, Chitose City Hospital, Chitose City, Hokkaido, Japan d Division of Radiology, Chitose City Hospital, Chitose City, Hokkaido, Japan b

Received 20 April 2009; received in revised form 15 July 2009; accepted 16 July 2009

KEYWORDS Acute coronary syndrome; Metastatic cardiac tumor; Coronary compression; CTA/SPECT fusion image

Summary A 60-year-old female without coronary risk factors was admitted to the hospital with ST-elevation acute coronary syndrome (ACS). She had previously suffered breast cancer and received radical mastectomy followed by chemotherapy and radiation. Emergent coronary angiography showed an occlusion of the proximal left anterior descending coronary artery (LAD) and coronary angioplasty was performed. Coronary computed tomography (CT) angiography (CTA) disclosed a tumor invading the left ventricular anterior wall and surrounding the coronary artery. Myocardial single-photon-emission CT (SPECT) using 123I-BMIPP showed a defect in the same portion. A fusion image of the CTA and the SPECT delineated a tumor surrounding the coronary artery. She finally died two months later from a terminal condition. Autopsy demonstrated a tumor involving the left ventricular anterior wall and surrounding the LAD. Pathology of the affected LAD showed only fibrous plaque without vulnerable plaque, thrombus, or tumor invasion to the coronary wall. Thus, compression of the coronary artery by the metastatic tumor was the most likely mechanism of ACS. © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.

Introduction

∗ Corresponding author. Tel.: +81 138 52 1231 fax: +81 138 2454 7520. E-mail address: [email protected] (T. Ozaki).

Acute coronary syndrome (ACS) is mostly associated with thrombotic obstruction of the coronary artery secondary to a plaque rupture [1]. We report a rare case of ACS, in which coronary obstruction was not due to plaque rupture but to compression of the coronary artery by a metastatic cardiac tumor. A fusion image of coronary computed

1878-5409/$ — see front matter © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.jccase.2009.07.005

Acute coronary syndrome associated with a metastatic cardiac tumor tomography (CT) angiography (CTA) and myocardial singlephoton-emission CT (SPECT) was very useful to delineate the relationship between the tumor and the affected coronary artery.

Case report A 60-year-old female was admitted to our hospital because of strong chest pain from the morning. She had received radical mastectomy for breast cancer 16 years earlier and subsequent chemotherapy. Seven years later she underwent operation and chemotherapy for the tumor metastasis to the lumbar vertebra. Because of relapses of the metastatic lesion, she repeatedly received operation, chemotherapy, and radiation therapy, and was subjected to terminal home care. On admission, her blood pressure was 110/60 mmHg and heart rate was 130 bpm with regular sinus tachycardia. An electrocardiogram showed pronounced ST-segment elevation in the precordial leads, indicating ST-elevation ACS (Fig. 1A). Echocardiography showed hypokinesis of the left ventricular anterior wall, however it did not identify any abnormal mass. Emergent coronary angiography (CAG) showed an occlusion of the proximal left anterior descending coronary artery (LAD) and an intracoronary injection of nitrate did not relieve the occlusion. Thus, coronary angioplasty was performed. At coronary angioplasty, we selected plain balloon angioplasty instead of stent deployment, because antiplatelet therapy after the stent deployment seemed unfavorable for both her terminal condition and persistent bloody urine as radiation cystitis. A guide-wire was easily crossed through

e53

the coronary lesion, however, an adequately size balloon slipped at the lesion on its inflation over 2 atm pressures. The lesion was finally dilated by a deep engaging technique with a guide-catheter. Final CAG showed relatively smooth coronary artery wall and no intracoronary thrombi. Chest pain and the ST-segment elevation disappeared after the angioplasty (Fig. 1B). During hospitalization, she did not have any arrhythmia or heart failure and a serum level of creatine kinase was within normal range. Coronary CTA (Brilliance 64, Philips, Eindhoven, the Netherlands) performed one week later identified a mild stenosis in the proximal LAD (Fig. 2A) and a tumor invading the anterior myocardium and involving the coronary artery (Fig. 2B). The mild stenosis suggested recoil of once dilated coronary artery. Myocardial SPECT with 123 I-BMIPP showed a defect in the mid antero-septal and anterior wall (Fig. 3A), whereas a fusion image (MultiDataFusion, Ziosoft, Inc., Tokyo, Japan) of the coronary CTA and the myocardial SPECT delineated the tumor overlying the LAD as well as the low radioisotope uptake in the anterior wall. The tumor was not involved in fatty acid metabolism (Fig. 3B). She died two months later and autopsy defined a tumor overlying the left ventricular anterior wall and the LAD (Fig. 4A). The transverse section of the left ventricle showed metastatic cardiac tumors invading the anterior wall and the ventricular septum (Fig. 4B). No continuity between these tumors was observed. The tumors were histologically composed of sarcoma and spotty hemorrhage was shown in the tumor surrounding the coronary artery. Cross-sections of the LAD disclosed a focal eccentric and fibrous plaque without vulnerable plaque, plaque rupture, or thrombus (Fig. 4C). There was no tumor invasion into the coronary artery wall.

Figure 1 Electrocardiograms. An electrocardiogram (A) on admission shows pronounced ST elevation in the precordial leads as compared with the electrocardiogram after reperfusion (B).

e54

Figure 2 Coronary computed tomography angiography. (A) A maximum intensity projection image shows a mild stenosis (shown by an arrow) at the proximal left anterior descending coronary artery. (B) A long-axis view reveals a cardiac tumor (shown by an arrow) surrounding the coronary artery and invading the left ventricular anterior wall.

Metastatic tumors to the lumbar vertebra and the lung also showed sarcoma in histology.

Discussion About 5% of acute myocardial infarctions are not based on coronary atherosclerosis and plaque rupture [2], and various causes are involved such as infectious disease, amyloidosis, embolism, neoplasm, radiation-associated fibrosis, and cocaine abuse. Metastatic cardiac tumors are frequently asymptomatic but, depending on the tumor location and extent, heart failure, pericardial effusion, arrhythmia, and sudden cardiac death can occur [3].

T. Ozaki et al.

Figure 3 (A) Myocardial single-photon-emission computed tomography (SPECT) with 123 I-BMIPP shows a defect in the area consistent with the tumor location. (B) A fusion image of coronary computed tomography angiography and myocardial SPECT with BMIPP (AP-cranial view) reveals that the tumor unassociated with fatty acid metabolism overlies the proximal left anterior descending coronary artery.

As a mechanism of myocardial infarction in this case, we first suspected coronary tumor embolism, because she had no coronary risk factors and was in a terminal stage of breast cancer and its metastatic tumor to lumbar vertebrae. However, CAG documented neither tumor embolism nor thrombus formation. Further, CAG after angioplasty showed a relatively smooth coronary artery wall and no slow flow, indicating that plaque rupture or thrombus was not associated with the coronary obstruction. Thus, we speculated that ACS was caused by coronary compression of the metastatic cardiac tumor. The autopsy finding macroscopically coincided with the CTA/SPECT fusion image. The coronary lesion showed focal fibrous plaque without thrombus, plaque rupture, or tumor invasion into the coronary artery wall. The plaque had histologically two differential layers (Fig. 4C). In the inner layer, most of the intimal thickening appeared to be composed of immature extracellular matrix, that is thought to

Acute coronary syndrome associated with a metastatic cardiac tumor

e55

reflect neointimal proliferation after balloon angioplasty two months previously. Since the tumors in the heart and the lung histologically showed sarcoma, we considered that the metastatic tumor in the lumbal vertebrae was transformed to sarcoma by repeated radiation to the vertebrae and thus the tumors in the heart and the lung originated from the vertebral tumor. Conclusively, ACS in this case was due to a coronary artery compression by a metastatic cardiac tumor. A CTA/SPECT fusion image is very useful to clarify the relationship between the tumor and the affected coronary artery.

References [1] Ross R. Atherosclerosis—–an inflammatory disease. N Engl J Med 1999;340:115—26. [2] Waller BF, Fry ET, Hermiller JB, Peters T, Slack JD. Nonatherosclerotic causes of coronary artery narrowing—–part III. Clin Cardiol 1996;19:656—61. [3] Tamura A, Matsubara O, Yoshimura N, Kasuga T, Akagawa S, Aoki N. Cardiac metastasis of lung cancer. A study of metastatic pathways and clinical manifestations. Cancer 1992;70:437—42.

Figure 4 Autopsy findings. (A) Autopsy macroscopically showed a tumor overlying the left ventricular anterior wall and surrounding the left anterior descending coronary artery. (B) Cross-section of the left ventricle shows tumor metastases in the anterior wall and ventricular septum. (C) The left anterior descending coronary artery histologically shows an eccentric and focal fibrous plaque in the absence of vulnerable plaque or thrombus (Elastica-Masson staining ×10). The tumor surrounding the coronary artery does not invade the arterial wall.

Journal of Cardiology Cases (2010) 1, e56—e62

available at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/jccase

Case Report

Long-term follow-up of ‘‘reversible’’ dilated cardiomyopathy with improvement of cardiac sympathetic nerve activity after cardiac resynchronization therapy (CRT) Do ‘‘CRT superresponders’’ have ‘‘dyssynchrony-induced cardiomyopathy’’? Kazuya Ishibashi (MD, PhD) a,∗, Tomoko Osamura (MD) a, Hirokazu Shiraishi (MD) b, Takeshi Shirayama (MD, PhD) b, Yasuhiro Yamahara (MD, PhD) a, Hiroaki Matsubara (MD, PhD) b a b

Department of Cardiology, Saiseikai Kyoto Hospital, Kyoto, Japan Department of Cardiovascular Medicine, Kyoto Prefectural University School of Medicine, Kyoto, Japan

Received 11 May 2009; received in revised form 22 June 2009; accepted 27 July 2009

KEYWORDS Dilated cardiomyopathy; Cardiac resynchronization therapy; Beta-blocker; 123 I-metaiodobenzylguanidine; Superresponder; Carvedilol

Summary We report a case of idiopathic dilated cardiomyopathy with severe heart failure and complete left bundle branch block (CLBBB) which exhibited an excellent response to cardiac resynchronization therapy (CRT). A 71-year-old male had been treated for 9 years with medication for chronic heart failure. He was referred to hospital with a complaint of dyspnea. An electrocardiogram showed CLBBB, with a QRS-width of 200 ms. Markedly dilated left ventricular (LV) chamber with a low ejection fraction (EF) of 18% and severe mitral regurgitation were registered by echocardiogram. Myocardial neuronal 123 I-metaiodobenzylguanidine uptake was reduced, with a heart-to-mediastinum (H/M) ratio of 1.88. Immediately after the introduction of CRT, clinical improvement was observed. At 1-year follow-up, LV chamber size and cardiac function were almost normalized, with an EF of 53%. Cardiac sympathetic nerve activity (CSNA) was simultaneously normalized, with an H/M ratio of 2.32 and a washout rate of 14.7%. However, after the cessation of carvedilol administration, CSNA and LV systolic function were slightly aggravated, with an H/M ratio of 2.20, a washout rate of 15.9%, and an EF of 44%. In

∗ Corresponding author at: Department of Cardiology, Saiseikai Kyoto Hospital, 8 Minamihirao, Imazato, Nagaokakyo, Kyoto 617-0814, Japan. Tel.: +81 75 955 0111; fax: +81 75 954 8255. E-mail address: [email protected] (K. Ishibashi).

1878-5409/$ — see front matter © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.jccase.2009.07.004

Superresponder to CRT

e57 the present case, the excellent improvement in cardiac function and CSNA was caused by the combined effects of beta-blocker therapy and CRT. © 2009 Japanese College of Cardiology. Published by Elsevier Ireland Ltd. All rights reserved.

Introduction Cardiac resynchronization therapy (CRT) for idiopathic dilated cardiomyopathy (DCM) with severely impaired cardiac function [New York Heart Association (NYHA) functional class III/IV] and complete left bundle branch block (CLBBB) is well established. However, approximately 30% of the patients who fulfill the CRT selection criteria are regarded as ‘‘nonresponders’’, who do not clinically benefit after CRT [1]. On the other hand, it has recently been reported that some cases exhibit excellent responses to CRT [2—4]. In these cases, cardiac function was almost normalized by CRT (EF > 50%) and they were called ‘‘superresponders’’ (or ‘‘hyperresponders’’). We assessed the cardiac sympathetic nerve activity in the case of a ‘‘superresponder’’ to CRT, who had been followed up over a long-term of 9 years. Moreover, we considered whether it was valid to regard ‘‘superresponders’’ as having ‘‘dyssynchrony-induced cardiomyopathy.’’

Case report A 71-year-old male was first informed that he had intermittent left bundle branch block and left ventricular (LV) dysfunction with dyssynchrony during a complete physical checkup at the age of 61, when he was asymptomatic (Fig. 1). He had no habits of excessive alcohol drinking. Moreover, he had no history of tachyarrhythmia. At the age of 62, he was admitted to our hospital because of exertional dyspnea. Coronary angiography showed normal coronary arteries and left ventriculography revealed diffusely severe hypokinesis of LV, with an ejection fraction (EF) of 30%. He was then diagnosed with DCM. Conventional medical treatment including beta-blocker (carvedilol), diuretic (furosemide), and angiotensin-converting enzyme inhibitor (ACEI) (enarapril) was initiated. Since he was 63 years’ old, electrocardiograms (ECG) have consistently shown CLBBB. The subjective symptoms were unchanged for 7 years, with NYHA functional class II, although LV remodeling gradually developed (Table 1). The serum brain natriuretic peptide (BNP) concentration was getting higher during the clinical course (Table 1). At the age of 70, despite medical treatment with diuretics (azosemide and spironolactone), angiotensin II receptor blocker (ARB) (candesartan), and beta-blocker (carvedilol 10 mg/day), he was suffering from progressive dyspnea on exertion. Home oxygen therapy was introduced by his personal doctor. As his symptoms took a turn for the worse 2 weeks prior to referral, he was admitted to our hospital. A chest radiograph showed pulmonary congestion and marked cardiomegaly, with a cardio-thoracic ratio (CTR) of 63% (Fig. 2). An ECG showed CLBBB with a QRS-width of 200 ms (Fig. 2). Laboratory data revealed moderate renal dysfunction (urea nitrogen/creatinine of 34.9/1.70 mg/dl) and an extremely high level of BNP (1091.8 pg/ml).

Echocardiography revealed marked LV dilatation [dimension of LV end-diastole (Dd)/dimension of LV end-systole (Ds) of 78/72 mm], wall thinning in the LV anteroseptal area and impaired LV contraction, with EF of 18% (Fig. 3). Dyspnea was relieved with intensive diuresis and oxygen supply. 123 I-metaiodobenzylguanidine (MIBG) scintigraphy was conducted to evaluate the degree of cardiac sympathetic nervous system derangement, reflecting the severity of myocardial damage in DCM. The heart-to-mediastinum (H/M) ratio (H/M) was moderately decreased in the early phase, with a value of 1.88 (Table 2). Because of mechanical problems, no delayed planar MIBG image was acquired. The patient was transferred to another hospital for the implantation of a CRT device with a defibrillator (CRT-D) (Concerto C154DWK, Medtronic, Minneapolis, MN, USA). Pacing leads were positioned in the right atrial appendage, the right ventricular apex, and the lateral branch of the coronary vein. The lower rate and the atrio-ventricular delay were optimized at 50 bpm and 120 ms, respectively. Immediately after the initiation of CRT, clinical improvement was observed. Medical therapy, including diuretics, ARB, and beta-blocker, was maintained after the CRT. One year later, CRT enabled the patient to walk up a steep slope as long as 4 km without feeling shortness of breath. At the 1-year follow-up, ECG showed a narrow QRS-width of 110 ms under biventricular pacing (Fig. 4). A chest radiograph indicated a normal CTR of 47% (Fig. 4). Echocardiography revealed a normal LV chamber size (Dd/Ds of 52/38 mm), mild concentric hypertrophy (‘‘elimination of wall thinning’’), and improved contractile function, with an EF of 53% (Fig. 3). LV dyssynchrony was visually wellcorrected. No significant mitral regurgitation was detected by color Doppler imaging. The serum BNP concentration was 13.7 pg/ml (normal range: