Markedly Dilated Right Heart 17 Years After Initial Treatment Repaired by Total Right Ventricular Exclusion and Total Cavopulmonary Connection

• DOI: 10.1161/circulationaha.107.760561
• Publication Date: 2008-08-25T21:03:06Z
• Authors: Taro Kariya; Yasushi Imai; Arata Murakami; Sachito Minegishi; Tatsuo Katori; Hitoshi Kato; Kohsuke Ajiki; Yasunobu Hirata; Ryozo Nagai
• Keywords: Cardiothoracic surgery

The total right ventricular (RV) exclusion procedure for isolated congestive RV failure, in which most of the dilated right atrium (RA) and RV wall is resected, was first reported by Sano et al in 2002.1 This procedure improves the cardiac index2 and suppresses supraventricular/ventricular tachyarrhythmia. Using this procedure with an extracardiac conduit, we successfully treated severe heart failure characterized by a markedly dilated right heart lacking tricuspid leaflets and by recurrent tachyarrhythmia, 17 years after the patient had undergone initial definitive intracardiac repair including RV outflow tract plasty. A 22-year-old woman presented with general fatigue, dyspnea, palpitations, and loss of appetite of 6 months’ duration caused by low cardiac output. She was admitted to the University of Tokyo Hospital to undergo surgery to improve her low cardiac output. She was cyanotic at birth after an uneventful pregnancy and caesarian delivery. Cardiac catheterization performed when she was 47 days old revealed pulmonary atresia with atrial septal defect and patent ductus arteriosus. A Blalock-Taussig anastomosis was established. Subsequently, she was diagnosed with absent tricuspid valve, pulmonary atresia, and atrial septal defect. These were definitively treated surgically in 1990 when the patient was 5 years old via RV outflow tract plasty and atrial septal defect closure. Intraoperative findings included weak RV contraction and an anterior RV wall almost totally lacking myocardium. Five years after the operation, she suffered from ventricular tachycardia originating from the RV wall, which was treated by electrical cardioversion and subsequent administration of oral antiarrhythmic medication. Six months before admission, dyspnea …