Heart Transplantation in Arrhythmogenic Right Ventricular Dysplasia: Case Reports

• Published in: Transplantation proceedings Vol. 41; no. 3; pp. 962 - 964
• Main Authors: Fiorelli, A.I, Coelho, G.H.B, Oliveira, J.L, Nascimento, C.N.G, Vilas Boas, L.B, Napolitano, C.F.C, Bacal, F, Bochi, E.A, Stolf, N.A.G
• Format: Journal Article Conference Proceeding
• Language: English
• Published: Amsterdam Elsevier Inc 01.04.2009; Elsevier
• Subjects: Adolescent; Arrhythmias, Cardiac - etiology; Arrhythmias, Cardiac - surgery; Biological and medical sciences; Cardiac dysrhythmias; Cardiology. Vascular system; Cardiomyopathies - etiology; Cardiomyopathies - genetics; Cardiomyopathies - surgery; Chromosomes, Human; Electrocardiography; Female; Fundamental and applied biological sciences. Psychology; Fundamental immunology; Genes, Dominant; Heart; Heart Transplantation - methods; Heart Transplantation - mortality; Humans; Male; Medical sciences; Surgery; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Tissue, organ and graft immunology; Ventricular Dysfunction, Right - surgery; Young Adult; Heart; Human; Arrhythmia; Cardiovascular disease; Transplantation; Homotransplantation; Case study; Medicine; Treatment; Arrhythmogenic right ventricular dysplasia; Heart disease; Surgery; Graft; Circulatory system
• ISSN: 0041-1345; 1873-2623
• DOI: 10.1016/j.transproceed.2009.02.010

Abstract Objective Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial disease of familiar, origin where the myocardium is replaced by fibrofatty tissue predominantly in the right ventricle. Herein we have presented the clinical courses of 4 patients with ARVD who underwent orthotopic heart transplantation. Patients and Methods Among 358 adult patients undergoing heart transplantation, 4 (1.1%) displayed ARVD. The main indication for transplantation was the progression to heart failure associated with arrhythmias. All 4 patients displayed rapid, severe courses leading to heart failure with left ventricular involvement and uncontrolled arrhythmias. Results In all cases the transplantation was performed using a bicaval technique with prophylactic tricuspid valve annuloplasty. One patient developed hyperacute rejection and infection, leading to death on the 7th day after surgery. The other 3 cases showed a good evolution with clinical remission of the symptoms. Pathological study of the explanted hearts confirmed the presence of the disease. Conclusions ARVD is a serious cardiomyopathy that can develop malignant arrhythmias, severe ventricular dysfunction with right ventricular predominance, and sudden cardiac death. Orthotopic heart transplantation must always be considered in advanced cases of ARVD with malignant arrhythmias or refractory congestive heart failure with or without uncontrolled arrhythmias, because it is the only way to remit the symptoms and the disease.