Journal of the Korean Pediatric Society 1987;30(3):281-290.
Published online March 31, 1987.
Clinical Study on the Hypoplastic Right Heart Syndrome.
Sejung Sohn, Soon Ung Kang, Chung Il Noh, Jung Yun Choi, Yong Soo Yun, Chang Yee Hong, Dug Ha Kim, Jong Lin Rhi
Department of Pediatrics, College of Medicine, Seoul National University
우심형성부전증에 대한 임상적 관찰
손세정, 강순웅, 노정일, 최정연, 윤용수, 홍창의, 김덕하, 이종린
서울대학교 의과대학 소아과학교실
A clinical observation was made on 12 cases of the hypoplastic right heart syndrome who were admitted to the department of Pediatrics, Seoul National University Hospital from January 1978 to May 1986. The -results were as follows; 1) The ratio of female to male was 1:1 and, according to the anatomic classification by Khoury, 6 cases were in type 1, 4 cases in type II and 2 cases in type III. 2) Congenital heart disease was detected at birth in 6 cases, 5 cases within 1 month and 1 case at 5 year of age. 3) Presenting symptoms and signs were cyanosis (100%), dyspnea (92%),heart murmur (92%), anoxic spell (42%) and heart failure (17%). 4) Simple chest X-ray showed decreased pulmonary vascularity in 10 cases (83%) and cardiomegaly in 2 cases (17%). Electrocardiogram showed right axis deviation in 6 cases (50%), normal axis deviation in 4 cases {33%) and left axis deviation in 2 cases (17%). Right atrial enlargement was observed in 7 cases (58%). 5) Hemoglobin level was distributed from 12.8 gm/dl to 22.5 gm/dl and the mean value was 18.5 gm/dl. 6) The mean values of “a” wave in right atrium and left atrium was 12.8 mmHg, 9.4 mmHg respectively. And the pressure difference between right and left atrium was over 2 mmHg in 10 cases (83%). The mean values of end-diastolic pressure in right and left ventricle were 12 mmHg, 7.6 mmHg respectively. 7) Pulmonary valve was atretic in 7 cases, stenotic in 5 cases, and tricuspid valve was atretic in 3 cases, insufficient or stenotic in 9 cases. Associated anomalies were as follows: ASD in 10 cases, PDA in 6 cases, VSD in 2 cases and PFO in 1 case. 8) Surgical intervention was done in 6 cases (50%). Three cases were operated with shunt procedure, 2 cases with pulmonary valvotomy and 1 case with Fontan operation. There were 4 deaths among 11 cases.
Key Words: Hypoplastic right heart syndrome.

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