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

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Abstract
With the rare exception of connective-tissue replacement of the right ventricular myocardium, underdevelopment of the right ventricle, as a clinical entity, has been related to malformation of the tricuspid or pulmonic valves. 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 features. Cardiac catheterization and venous angiocardiography showed bi-directional interatrial shunt, normal pulmonary arterial pressure, no pressure gradient across the pulmonic and tricuspid valves, and a diminutive right ventricle.