Uhl’s Anomaly: Case Report and Differential Diagnosis in an Adult

Introduction

Uhl’s anomaly (UA) is a disorder exclusive to the right ventricle (RV) characterized by the absence of total or partial myocardium, in such a way that the ventricular wall is comprised of the overlapping of the endocardium and the epicardium, with no fatty tissue, or inflammatory or infiltrative process. The muscles of the atria, the interventricular septum and the left ventricle (LV) are not involved in the process.^,

The clinical manifestations are due to right ventricular failure (RVF). Forms with partial involvement may be mildly symptomatic and remain undiagnosed for long periods, and may be confused with other more frequent pathologies that affect the right heart in adult life.^,

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