Uhl′s anomaly: Absence of the right ventricular myocardium

Abstract

We report a case of Uhl′s anomaly in a 3-week-old infant that underwent central shunt placement, patent duct us arteriosus and main pulmonary artery ligation. The infant presented with room air saturation of 43%, dilated right ventricle with decreased function and dilated right atrium. Diagnosis was established with a myocardial biopsy.

Keywords

• Cyanosis in an infant
• right-sided heart failure
• right ventricular dysfunction
• Uhl′sanomaly