Congenital Pulmonary Vein Stenosis Manifested by Severe Cyanosis in Infancy.
- Author:
Jae Ju CHO
1
;
Woo Sung PARK
;
I Seok KANG
;
Tae Gook JUN
;
Mi Jin JUNG
Author Information
1. Department of Pediatrics, College of Medicine, Dankook University, Cheonan, Korea. mi_jinjung@dankook.ac.kr
- Publication Type:Case Report
- Keywords:
Congenital pulmonary vein stenosis;
Echocardiography;
Pulmonary hypertension;
Cyanosis
- MeSH:
Constriction, Pathologic*;
Cyanosis*;
Echocardiography;
Follow-Up Studies;
Heart Failure;
Heart Septal Defects, Atrial;
Hypertension, Pulmonary;
Pulmonary Veins*
- From:Korean Journal of Pediatrics
2004;47(10):1114-1118
- CountryRepublic of Korea
- Language:Korean
-
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.
