Congenital aplasia or marked hypoplasia of the myocardium of the right ventricle (Uhl's anomaly): Clinical, angiocardiographic, and hemodynamic findings

Abstract

Summary The previously unreported angiocardiographic and hemodynamic findings in a proved case of congenital aplasia or marked hypoplasia of the myocardium of the right ventricle are presented. The clinical picture consisted of progressive heart failure in infancy, marked cardiomegaly, feeble triple heart tones, no significant murmur, and absence of clinical evidences of right ventricular hypertrophy, pulmonary hypertension, or tricuspid regurgitation. The chest roentgenograms demonstrated marked cardiac enlargement with normal or diminished pulmonary vascular markings. The electrocardiogram showed right atrial hypertrophy and left ventricular hypertrophy. Cardiac catheterization revealed normal systolic pressures and markedly elevated presystolic waves in the right ventricular and pulmonary arterial pressure curves which were identical with the right atrial “a” waves. We have not encountered nor have we seen any reports in the literature showing identical curves. The angiocardiograms demonstrated enlarged right heart chambers, diminished pulmonary blood flow, unusual thinness of the right ventricular wall, and conspicuous absence of it trabeculae. We believe that the correct diagnosis may be derived from the clinical, cardiac catheterization, and angiocardiographic findings. It is suggested that a superior vena cavaright pulmonary artery anastomosis be performed for palliative treatment of this anomaly.