Journal of the Korean Pediatric Society 1979;22(6):476-480.
Published online June 15, 1979.
A Case of Uhls Anomaly.
Jun Hee Sul1, Ki Chun Kim1, Ki Sub Chung1, Sung Kyu Lee1, Chang Jin Kim2, Jae Yun Rho2
1Department of pediatrics, Yonsei University, Collegect Medicine, Korea.
2Department of Pathology, Yonsei University, Collegect Medicine, Korea.
우심실근의 형성부전증(Uhl씨 가형) 1예
설준희1, 김기춘1, 정기섭1, 이승규1, 김창진2, 노재윤2
1연세대학교 의과대학 소아과학교실
2연세대학교 의과대학 병리학교실
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.




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