Uhl's Anomaly:
Clinical:
Uhl's anomaly is a rare disorder characterized by focal or complete absence of the parietal wall of the right ventricular myocardium. The parietal wall consists of the opposing endocardial and epicardial ventricular surfaces with no fatty tissue interposed between these layers (this permits distinction from arrhythmogenic right ventricular dysplasia). The septal wall of the right ventricle is intact. The disorder is identified in the neonatal or infant period. The most common clinical presentation is congestive heart failure. There is no associated increased incidence of other cardiac anomalies [1].REFERENCES:
(1) Br Heart J 1993; Gerlis LM, et al. Dysplastic conditions of the
right ventricular myocardium: Uhl's anomaly v arrhythmogenic right ventricular
dysplasia. 69: 142-150
