Key result
CMR confirms Uhl anomaly by revealing an extremely dilated right ventricle devoid of myocardium.
Why the study?
Uhl's anomaly is a rare condition characterized by right ventricular myocardial absence and requires recognition for diagnosis.
Case Report (n=1)
Supports CMR for diagnosing Uhl's anomaly in adolescents; extends sparse case data but should not yet change practice.
A 17-year-old boy presented with facial puffiness and swelling in the lower limbs for 6 months and one episode of syncope 15 days earlier. Transthoracic echocardiography showed a dilated right atrium and right ventricle with right ventricular systolic dysfunction. The free wall of the right ventricle was thinned out and devoid of myocardium and trabeculations. Cardiac magnetic resonance imaging showed an extremely dilated thin-walled right ventricle and absence of trabeculations, with no fat signal in the right ventricular wall, in contrast to that seen in arrhythmogenic ventricular dysplasia, which confirmed the diagnosis of Uhl's anomaly.
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Kumar et al. (2016) conducted a case report in Uhl's anomaly (n=1). Cardiac magnetic resonance imaging confirmed the diagnosis of Uhl's anomaly in a 17-year-old boy by demonstrating an extremely dilated thin-walled right ventricle devoid of myocardium and fat.
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