Uhl's Anomaly With Left Ventricular Noncompaction: Role of Multimodality Imaging in a Rare Association

Adeba Mohammad; Purvi Parwani; Carlo Manalo; Brent M Gordon; Ahmed Kheiwa

doi:10.1016/j.jaccas.2021.06.042

Uhl's Anomaly With Left Ventricular Noncompaction: Role of Multimodality Imaging in a Rare Association

JACC Case Rep. 2021 Sep 15;3(12):1463-1467. doi: 10.1016/j.jaccas.2021.06.042.

Authors

Adeba Mohammad¹, Purvi Parwani², Carlo Manalo³, Brent M Gordon⁴, Ahmed Kheiwa²

Affiliations

¹ Department of Internal Medicine, Loma Linda University Health, Loma Linda, California, USA.
² Department of Cardiology, Loma Linda University Health, Loma Linda, California, USA.
³ Department of Radiology, Loma Linda University Health, Loma Linda, California, USA.
⁴ Department of Pediatric Cardiology, Loma Linda University Health, Loma Linda, California, USA.

Abstract

Uhl's anomaly is a rare congenital heart disease characterized by partial or complete absence of the right ventricle myocardium. We report the first case, in a 21-year-old man, of Uhl's anomaly-associated left ventricular noncompaction. This association represents a unique clinical entity and has important implications for management strategies. (Level of Difficulty: Intermediate.).

Keywords: ARVD/C, arrhythmogenic right ventricular dysplasia or cardiomyopathy; ASD, atrial septal defect; EDP, end-diastolic pressure; EF, ejection fraction; LV, left ventricular; LVNC, left ventricular noncompaction; PVD, pulmonary vascular disease; RA, right atrial; RV, right ventricular; TCPC, total cavopulmonary connection; TV, tricuspid valve; Uhl’s anomaly; atrial septal defect; cardiac magnetic resonance; congenital heart defect; left ventricle; left ventricular noncompaction; right ventricle.

Publication types

Case Reports