Transplante cardíaco na displasia arritmogênica do ventrículo direito* Heart transplantation in the arrhythmogenic right ventricular dysplasia

Introduction: The arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial disease, with family origin, were the myocardium is replaced by fi brofatty tissue, with a predominance in the right ventricle. The clinical picture is characterized by the emergence of right ventricular arrhythmias that can lead to sudden death. Objective: This paper aims to present the clinical course of four patients with ARVD, witch were submitted to the orthotopic heart transplant technique bicaval. Patients and Methods: All the four patients had a rapid and severe course, leading to heart failure, with left ventricular involvemtent, and uncontrolled arrhythmias, being indicated the heart transplantation. Results: In all cases the transplant was performed by technical bicaval with the tricuspid valve annuloplasty prophylactic. Only one patient 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 examination of the breasts removed confi rmed the presence of the disease. Conclusions: The ARVD is a serious disease that can develop with malignant arrhythmias, ventricular dysfunction with a predominance of the right ventricle and sudden death. The orthotopic heart transplant must be considered in serious cases and shows clinical remission of symptoms.