All issues > Volume 47(10); 2004

Case Report

Korean J Pediatr. 2004;47(10):1114-1118. Published online October 15, 2004.

Congenital Pulmonary Vein Stenosis Manifested by Severe Cyanosis in Infancy

Jae Ju JJ Cho¹, Woo Sung WS Park¹, I-Seok IS Kang², Tae-Gook TG Jun³, Mi-Jin MJ 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

Correspondence Mi-Jin MJ 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.

Keywords :Congenital pulmonary vein stenosis, Echocardiography, Pulmonary hypertension, Cyanosis