Isolated Right Ventricular Hypoplasia with Atrial Septal Defect or Patent Foramen Ovale

Abstract

With the rare exception of connective-tissue replacement of the right ventricular myocardium ("parchment heart"), underdevelopment of the right ventricle, as a clinical entity, has been related to tricuspid atresia or stenosis, pulmonary atresia and certain cases of transposition of the great vessels. This report is concerned with four cases, aged 2 months to 39 years, in which a diminutive right ventricle was not dependent on these factors. Three cases occurred in the same family and, in association with atrial septal defect, produced distinctive clinical and hemodynamic features. Right-sided heart failure predominated, dyspnea was mild to moderate, and cyanosis was present in association with clinical, electrocardiographic, and roentgenographic evidence of left ventricular enlargement. Cardiac catheterization and venous angiocardiography showed a bidirectional interatrial shunt, normal pulmonary arterial pressure, no pressure gradient across the pulmonic and tricuspid valves, and a diminutive right ventricle. Anastomosis of the superior vena cava to the right pulmonary artery and closure of the atrial septal defect may ameliorate the hemodynamic disturbance.