Uhl's anomaly: A rare case of portal hypertension

Abstract

Uhl's anomaly is a rare congenital heart disease characterized by partial or complete absence of the right ventricular myocardium and high early mortality rates. We describe a case of Uhl's anomaly in a 27-year-old young male patient presenting with portal hypertension and esophageal varices. In this article, we review the literature associated with this condition and highlight a rare presentation of a rare disease. This report adds to our current knowledge of this exceedingly rare disorder.

Keywords

• Absent myocardium and portal hypertension
• adult Uhl's anomaly
• Uhl's anomaly