Heart Transplantation for Uhl Anomaly in an Adult

Omar Toubat; Jason J Han; Jarrod D Predina; Lee R Goldberg; Michael E Ibrahim

doi:10.1016/j.jaccas.2024.102322

Heart Transplantation for Uhl Anomaly in an Adult

JACC Case Rep. 2024 Apr 4;29(10):102322. doi: 10.1016/j.jaccas.2024.102322. eCollection 2024 May 15.

Authors

Omar Toubat¹, Jason J Han¹, Jarrod D Predina², Lee R Goldberg³, Michael E Ibrahim¹

Affiliations

¹ Division of Cardiovascular Surgery, Department of Surgery, Hospital of the University of Pennsylvania, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
² Division of Thoracic Surgery, Department of Surgery, Hospital of the University of Pennsylvania, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
³ Division of Cardiovascular Medicine, Department of Medicine, Hospital of the University of Pennsylvania, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

Abstract

Uhl anomaly is characterized by the morphologic absence of right ventricular myocardium and is an exceedingly rare cause of nonischemic cardiomyopathy. We report the first case of a successful heart transplantation in a 41-year-old patient who presented in cardiogenic shock from Uhl anomaly causing decompensated right ventricular failure.

Keywords: Uhl anomaly; cardiac transplantation; heart failure.

Publication types

Case Reports