Congenital Pulmonary Vein Stenosis Manifested by Severe Cyanosis in Infancy / 소아과
Korean Journal of Pediatrics
;
: 1114-1118, 2004.
Article
in Korean
| WPRIM
| ID: wpr-108565
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pulmonary Veins
/
Echocardiography
/
Follow-Up Studies
/
Constriction, Pathologic
/
Cyanosis
/
Heart Failure
/
Heart Septal Defects, Atrial
/
Hypertension, Pulmonary
Type of study:
Observational study
/
Prognostic study
Language:
Korean
Journal:
Korean Journal of Pediatrics
Year:
2004
Type:
Article
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