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Ann Thorac Surg 2005;80:2358-2360
© 2005 The Society of Thoracic Surgeons

Case report

Three Cases of Orthotopic Heart Transplantation for Arrhythmogenic Right Ventricular Cardiomyopathy

Masataka Yoda, MD * , Kazutomo Minami, MD, PhD, Dirk Fritzsche, MD, PhD, Gero Tendrich, MD, PhD, Sebastian Schulte-Eistrup, MD, Reiner Koerfer, MD, PhD

Department of Thoracic and Cardiovascular Surgery, Heart Center North Rhine-Westphalia, University of Bochum, Bad Oeynhausen, Germany

Accepted for publication July 29, 2004.

* Address correspondence to Dr Yoda, Department of Thoracic and Cardiovascular Surgery, Heart Center North Rhine-Westphalia, University of Bochum, Georgstrasse 11, 32545 Bad Oeynhausen, Germany (Email: masatakayoda{at}aol.com).


   Abstract
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 Abstract
Introduction
 Case Reports
 Comment
 References
 
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a cardiomyopathy that primarily affects the heart muscle in the right ventricle. The ventricular muscle is replaced by fatty or fibrous tissue in a diffuse or spotty process. We performed orthotopic heart transplantations in 3 patients and all patients are alive. When ARVC has progressed to heart failure in the right or left ventricles, orthotopic heart transplantation is an effective therapeutic option.


   Introduction
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 Abstract
 Introduction
Case Reports
 Comment
 References
 
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a myocardial disease, often familial, that is characterized pathologically by fibrofatty replacement of right ventricular myocardium and clinically by right ventricular arrhythmias that may lead to sudden death in young people [1–4]. The left ventricle may be involved in the process, but severe left ventricular dysfunction rarely occurs. We report 3 patients who underwent orthotopic heart transplantation at our hospital for ARVC.


   Case Reports
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 Abstract
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 Case Reports
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 References
 
Case 1
A 28-year-old female was admitted to our hospital due to congestive right heart failure that presented massive abdominal ascites and hepatomegaly, New York Heart Association (NYHA) class IV. In 1999, she was implanted an implantable cardioverter-defibrillator (ICD) due to ventricular tachycardia (VT) requiring cardiopulmonary resuscitation and cardioversion. The patient underwent right ventricular endomyocardial biopsy, which revealed severe myocyte hypotrophy and fibrofatty replacement of myocardium.

At admission, a two-dimensional echocardiography showed slightly left ventricular impairment (ejection fraction [EF] = 51%), mild mitral valve regurgitation (grade II/IV), and moderate tricuspid valve regurgitation (grade IV/IV). The left ventricle was not enlarged; the endosystolic dimension (ESD) was 41 mm and endodiastolic dimension (EDD) was 52 mm. The right ventricle was rather enlarged (RVEDD = 40 mm) and its fractional shortening (FS) was 10%. Central venous pressure (CVP) was 31 mm Hg, right ventricular mean pressure was 21 mm Hg, pulmonary capillary wedge pressure (PCWP) was 29 mm Hg, and cardiac index (CI) was 1.82 L x min–1 x m–2 in a Swan-Ganz catheter examination. The coronary angiography was normal. An electrocardiogram demonstrated normal sinus rhythm with an atrioventricular block 1 degree, Q-wave in lead III, and QRS duration of 100 ms. No {varepsilon}waves were identified. The patient underwent orthotopic heart transplant 3 years after diagnosis of ARVC. The explanted heart revealed typical features of ARVC. The right ventricle was massively dilated and its wall was very thin. Some areas of the anterior right ventricular wall were replaced with fatty and fibrous tissue and devoid of muscle fibers. The right ventricle's layer was minimal, 1 mm in thickness. A small area of the left ventricular myocyte was intermingled with fibrofatty tissue. The subendocardial areas of both ventricles were replaced with fibrous tissue (Fig 1).



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  		Fig 1. Pathologic finding of explanted heart. The upper panel illustrates massive fatty infiltration (hematoxylin & eosin stain, original magnification x50). The lower panel shows transmural sever fatty infiltration, subendocardial fibrosis (blue area; masson-goldner stain, original magnification x50).

 
Case 2
A 62-year-old man was hospitalized due to right ventricular failure, NYHA class IV. He had a family history of ARVC, his brother had ARCV that had been certified by right ventricular biopsy, another brother and a niece died suddenly, and his sister had hypertrophic obstructive cardiomyopathy.

In 1992, he had nonsustained VT, which required Sotalol. A two-dimensional echocardiogram revealed an enlarged right ventricle with hypokinesia all around (FS = 17% and RVEDD = 41 mm). The left ventricular function was normal (EF = 60%, LVESD = 34 mm, and LVEDD = 45 mm). The coronary angiography was normal. An electrocardiogram showed normal sinus rhythm, Q-wave in lead II, III and aVF. There were {varepsilon}waves present in lead V1 and V2 (Fig 2).



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  		Fig 2. Electrocardiogram of patient 2. Epsilon wave ({varepsilon}W, arrow) is apparent in leads V1 and V2.

 
Central venous pressure was 21 mm Hg, right ventricular mean pressure was 21 mm Hg, PCWP was 22 mm Hg, and CI was 2.61 L x min–1 x m–2 in a Swan-Ganz catheter examination. The patient underwent orthotopic heart transplant 10 years after diagnosis of ARVC. Areas of the anterolateral right ventricular wall were replaced with fatty and fibrous tissue. A small area of the left ventricular myocyte was intermingled with fibrofatty tissue.

Case 3
A 36-year-old man was diagnosed with ARVC with recurrent VT by right ventricular biopsy 10 years ago. In 2002, ablation therapy and ICD implantation were performed. After 1 year, he was admitted to our hospital due to right ventricular decompensation, NYHA class IV. We scheduled the heart transplantation. A two-dimensional echocardiography depicted normal left ventricular contraction (FS = 26%, EF = 65%). The right ventricle was enlarged (RVEDD = 61 mm) and there was impairmental contraction (FS = 9%). An electrocardiogram revealed normal sinus rhythm, Q-wave in lead II, III, and aVF. There were {varepsilon}waves present in lead V2 and V3. The explanted heart showed that the muscle fibers were replaced by fibrofatty tissue. The left ventricular wall was not replaced by fibrous tissue.


   Comment
Top
 Abstract
 Introduction
 Case Reports
 Comment
References
 
(All patients are alive for 6 months, 1 year, and 3 years.) Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a cardiomyopathy that primarily affects the heart muscle in the right ventricle. The ventricular muscle is replaced by fatty or fibrous tissue in a diffuse or spotty process. This process starts on the outside surface of the right ventricle and replaces the heart muscle cells that die prematurely. ARVC has been found to be genetic in 30% to 50% of individuals and transmitted from one affected parent to a child as a dominant disorder. Seven chromosomes have been identified that have areas or gene loci that have been linked to ARVC [4]. Most people who have ARVC are able to live normal lives with occasional episodes of abnormal heart rhythms.

The two biggest causes of death in patients with ARVC are severe ventricular arrhythmias and progressive heart failure, with a similar expected rate of approximately 1% per year [5].

Arrhythmias can be treated with drugs that have specific properties to prevent abnormal heart rhythms, such as Sotalol or Amiodarone, or medications that block the stimulating effects of adrenaline on the heart such as ß-blockers. Some patients who have episodes of ventricular tachycardia or ventricular fibrillation require an ICD.

Long-term follow-up data from clinical studies indicate that the right ventricle may become more diffuse, and it is dependent on time [6]. Later in the natural history of ARVC, the left ventricle may be progressively affected with subsequent biventricular failure.

Corrado and colleagues [7] reported that macroscopic or histologic involvement of the left ventricle was found in 76% of hearts with ARVC. Moreover, it was age dependent, more common in patients with longstanding clinical history.

Pinamonti and associates [8] reported that there were left ventricular asynergic areas or a mild depression of EF present in 19 of 45 patients. Furthermore, after a mean follow-up of 8.4 years, 11.6% of patients died from congestive heart failure, and all these patients were more than 50 years old.

In our institution, between March 1989 and December 2003, orthotopic heart transplantation was performed in 1382 patients. Only 3 patients underwent the orthotopic heart transplantation for ARVC. Severe diffuse biventricular involvement simulating dilated cardiomyopathy and requiring heart transplantation appears to be rare.

This case report has been published about orthotopic heart transplantation for ARVC. In our cases, the patients in cases 1 and 2 had slight or no left ventricular failure, but massive right ventricular failure resisted medications, which was why we performed the trasplantation. When ARVC has progressed to heart failure in the right or left ventricles, orthotopic heart transplantation is an effective therapeutic option.


   References
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 Abstract
 Introduction
 Case Reports
 Comment
 References
 

  1. McRae 3rd AT, Chung MK, Asher CR. Arrhythmogenic right ventricular cardiomyopathya cause of sudden death in young people. Cleve Clin J Med 2001;68:459-467.[Abstract/Free Full Text]
  2. Thiene G, Nava D, Corrado D, Rossi L, Pennelli N. Right ventricular cardiomyopathy and sudden death in young people N Engl J Med 1988;318:129-133.[Medline]
  3. Maron BJ, Shirani J, Poliac LC, Mathenge R, Roberts WC, Mueller FO. Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles JAMA 1996;276:199-204.[Medline]
  4. Basso C, Wichter T, Danieli GA, et al. Arrhythmogenic right ventricular cardiomyopathyclinical registry and database, evaluation of therapies, pathology registry, DNA banking. Eur Heart J 2004;25:531-534.[Free Full Text]
  5. Fontaine G. Arrhythmogenic right ventricular dysplasia Curr Opin Cardiol 1995;10:16-20.[Medline]
  6. Blomstrom-Lundqvist C, Sabel KG, Olsson SB. A long term follow up 15 patients with arrhythmogenic right ventricular dysplasia Br Heart J 1987;58:477-488.[Abstract/Free Full Text]
  7. Corrado D, Basso C, Thiene G. Arrhythmogenic right ventricular cardiomyopathydiagnosis, prognosis, and treatment. Heart 2000;83:588-595.[Free Full Text]
  8. Pinamonti B, Di Lenarda A, Sinagra G, Silvestri F, Bussana R, Camerini F. Long-term evolution of right ventricular dysplasia-cardiomyopathy. The Heart Muscle Disease Study Group Am Heart J 1995;129:412-415.[Medline]



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