Uhl's anomaly

Q: What is Uhl's anomaly in the heart?

Answer: Uhl's anomaly is also called parchment right ventricle. It was first described seven decades ago by Dr. Uhl. This condition is characterized by extremely thin and devoid of muscle fiber right ventricle (RV), or part of RV. This makes RV in complete apposition of endocardium and epicardium.

Caution: In some places, it has been described as arrhythmogenic right ventricular cardiomyopathy (ARVC) or arrhythmogenic right ventricular dysplasia (ARVD).  Uhl's anomaly is different from ARVC/ARVD with its own pathologic features, clinical findings, and presentations. Uhl anomaly is more common in childhood and marked by heart failure in contrast to ARVC, which is marked by arrhythmia, syncope, and/or sudden death.

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References:

1. UHL HS. A previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle. Bull Johns Hopkins Hosp 1952; 91:197.

2. Gerlis LM, Schmidt-Ott SC, Ho SY, Anderson RH. Dysplastic conditions of the right ventricular myocardium: Uhl's anomaly vs arrhythmogenic right ventricular dysplasia. Br Heart J 1993; 69:142.