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.

No abstract

As long ago as the middle of 1949 it had been demonstrated by the senior author that any blunt dilating mechanism applied within the stenotic aortic orifice would, upon expansion, force the separation of one or more of the fused commissures. Thus the diminutive valve orifice could be enlarged without the creation of additional insufficiency and with the restoration of such part of the original valve function as the pathologic distortion of the leaflets would permit. In other words the principles and effectiveness of such an aortic commissurotomy would be comparable to those of the similarly named operation for mitral stenosis. It has remained for us finally to develop an instrument and a technic capable of implementing these concepts, and this has at last been accomplished.

No abstract

No abstract