title: 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.
authors.affiliation: Department of General Medicine, IPGMER and SSKM Hospital, Kolkata, West Bengal, India.
authors.forename: Rakesh
authors.initials: R
authors.lastname: Agarwal
authors.affiliation: Department of General Medicine, IPGMER and SSKM Hospital, Kolkata, West Bengal, India.
authors.forename: Rajarshi
authors.initials: R
authors.lastname: Datta
authors.affiliation: Department of General Medicine, IPGMER and SSKM Hospital, Kolkata, West Bengal, India.
authors.forename: Manjari
authors.initials: M
authors.lastname: Saha
authors.affiliation: Department of General Medicine, IPGMER and SSKM Hospital, Kolkata, West Bengal, India.
authors.forename: Nirmalendu
authors.initials: N
authors.lastname: Sarkar
keywords: Absent myocardium and portal hypertension; Uhl's anomaly; adult Uhl's anomaly