Korean J Pediatr.  2004 Oct;47(10):1114-1118.

Congenital Pulmonary Vein Stenosis Manifested by Severe Cyanosis in Infancy

• Cho JJ
• Park WS
• Kang IS
• Jun TG
• Jung MJ

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.

Keyword

Congenital pulmonary vein stenosis; Echocardiography; Pulmonary hypertension; Cyanosis

MeSH Terms

Constriction, Pathologic*
Cyanosis*
Echocardiography
Follow-Up Studies
Heart Failure
Heart Septal Defects, Atrial
Hypertension, Pulmonary
Pulmonary Veins*