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A False Diagnosis of Hypoplastic Left Heart Syndrome in a Fetus

Journal of the Korean Pediatric Society 2002;45(2):267-272.
Published online February 15, 2002.
A False Diagnosis of Hypoplastic Left Heart Syndrome in a Fetus
Youn Jeong Shin1, Sung Hee Jang1, Jung Yun Choi2, Eun Sook Han3
1Department of Pediatrics, Kangnam General Hospital Public Co., Seoul, Korea
2Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea
3Department of Pediatric Echocardiography, Seoul National Uninversity, Seoul, Korea
태아기에 좌심형성부전증후군으로 잘못 진단되었던 1례
신윤정1, 장성희1, 최정연2, 한은숙3
1지방공사 강남병원 소아과
2서울대학교 의과대학 소아과학교실
3서울대학교 어린이병원 심장초음파실
Correspondence: 
Jung Yun Choi, Email: choi3628@plaza.snu.ac.kr
Abstract
Typical hypoplastic left heart syndrome(HLHS) is a distinct pathologic entity with aortic atresia, mitral atresia, very hypoplastic or absent left ventricle and thread like ascending aorta. Occasionally, the lesser degree of hypoplasia is found and is called hypoplastic left heart complex(HLHC) by some authors. This HLHC is often associated with critical aortic stenosis. Fetal echocardiography has enabled us to observe human fetal heart in-utero and to diagnose congenital heart disease prenatally over the last 20 years. The diagnosis of HLHS in fetal echocardiography is based on 2-dimensional echocardio -graphic evidence of a diminutive ascending aorta, aortic atresia, mitral atresia or severe stenosis and a hypoplastic left ventricle. Abnormal flow direction through atrial septum or through isthmus greatly aids the diagnosis. This report shows a fetal case who showed hypoplastic left side chambers and retrograde isthmic flow and was diagnosed with hypoplastic left heart syndrome. After birth, although the baby had tachy-dyspnea for the first 3 weeks, she finally recovered without any intervention and showed catch up growth of left side chambers. This case illustrates the extreme difficulty of assessing left ventricle in a fetus.
Key Words: Fetal Echocardiography, Hypoplastic Left Heart Syndrome


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