A Case of Uhls Anomaly

ABSTRACT

Congenital hypoplasia of the right ventricular myocardium, also known as parchment heart or Uhl's anomaly, is a rare congenital heart defect. It was first described in Oslers principles and Practice of Medicine in 1905 and reviewed by Segall We found 29 reported cases, but there was no reported case in Korea. Recently, we experienced a case of Uhls anomaly associated with patent ductus arteriosus, atrial septal defect, pulmonary atresia and tricuspid abnormality in a 3 month-old infant, which was confirmed by autopsy. We present a case with a brief review of litteratures.