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Published on: August 1, 2018
Uhl's Anomaly: A Rare Case of Portal Hypertension
Rakesh Agarwal1, Rajarshi Datta1, Manjari Saha1
1Department of General Medicine, IPGMER and SSKM Hospital, Kolkata, West Bengal, India.
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.
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