Uhl's anomaly of the right ventricle in a 1-year-old child requiring a transplant: A brief report

Abstract

Uhl's anomaly of the right ventricle is a rare anomaly. At present, only about 84 cases have been reported in over a century. We report a case of a 1-year-old male child who presented to our outpatient pediatric cardiology clinic with a history of recurrent cough and difficulty in breathing and failure to gain weight in the preceding 2 months. Chest X-ray showed cardiomegaly, whereas electrocardiogram showed a normal sinus rhythm with poor right ventricular (RV) forces. Echocardiography showed severely dilated right ventricle with thinned out right ventricle wall and poor RV function with severe tricuspid regurgitation. He has been placed on aspirin, lasilactone, and clexane. However, there was no satisfactory surgical option available, and heart transplant was recommended. Uhl's anomaly of the right ventricle is a very rare anomaly which defies all surgical option except transplant. Early recognition institution of drugs that improves cardiac function may improve the quality of life of the child.