Heart transplantation in the arrhythmogenic right ventricular dysplasia

Abstract

Introduction: The arrhythmogenic right ventricular dysplasia (ARVD) is amyocardial disease, with family origin, were the myocardium is replaced by fi brofatty tissue, witha predominance in the right ventricle. The clinical picture is characterized by the emergenceof right ventricular arrhythmias that can lead to sudden death. Objective: This paper aims topresent the clinical course of four patients with ARVD, witch were submitted to the orthotopicheart transplant technique bicaval. Patients and Methods: All the four patients had a rapidand severe course, leading to heart failure, with left ventricular involvemtent, and uncontrolledarrhythmias, being indicated the heart transplantation. Results: In all cases the transplant wasperformed by technical bicaval with the tricuspid valve annuloplasty prophylactic. Only onepatient developed hyperacute rejection and infection, leading to death on the 7th day after surgery.The other three cases showed clinical remission of symptoms. The pathological examinationof the breasts removed confi rmed the presence of the disease. Conclusions: The ARVD isa serious disease that can develop with malignant arrhythmias, ventricular dysfunction with apredominance of the right ventricle and sudden death. The orthotopic heart transplant must beconsidered in serious cases and shows clinical remission of symptoms.