Korean Journal of Pediatrics 2004;47(10):1114-1118.
Published online October 15, 2004.
Congenital Pulmonary Vein Stenosis Manifested by Severe Cyanosis in Infancy
Jae Ju Cho1, Woo Sung Park1, I-Seok Kang2, Tae-Gook Jun3, Mi-Jin Jung1
1Department of Pediatrics, College of Medicine, Dankook University, Cheonan, Korea
2Department of Pediatrics, Samsung Seoul Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea
3Department of Thoracic and Cardiovascular Surgery, Samsung Seoul Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea
극심한 청색증으로 표현된 선천성 폐정맥 협착증 1례
조재주1, 박우성1, 강이석2, 전태국3, 정미진1
1단국대학교 의과대학 소아과학교실
2성균관대학교 의과대학 삼성서울병원 소아과
3성균관대학교 의과대학 삼성서울병원 흉부외과
Correspondence: 
Mi-Jin Jung, Email: mi_jinjung@dankook.ac.kr
Abstract
Congenital pulmonary vein stenosis(CPVS) with anatomically normal connection, a rare anomaly, usually leads to progressive pulmonary hypertension, cardiac failure in infancy, and death if untreated. Most are combined with other anomalies, particularly left to right shunt lesions. Very often, the detection of CPVS is overlooked on the initial cardiac echocardiogram, because it may be mild in its severity initially, but progresses over time. CPVS shows the turbulence at color Doppler echocardiogram and a pulsed-wave Doppler signal of >1.6 m/sec with loss of phase. We experienced a case showing a small sized secundum atrial septal defect and mild turbulence at the right pulmonary vein on the initial color Doppler echocardiogram, and at follow-up, severe cyanosis, pulmonary hypertension, right heart failure, and reopened ductus with bidirectional shunt. Complete examination of echocariogram must be warranted at the initial stage and follow-up, in order not to miss CPVS.
Key Words: Congenital pulmonary vein stenosis, Echocardiography, Pulmonary hypertension, Cyanosis


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