A Case of Congenital Pulmonary Vein Stenosis Diagnosed in an Infant with Recurrent Hemoptysis / 소아알레르기및호흡기학회지

ABSTRACT

Congenital pulmonary vein stenosis is a rare cardiac malformation with a poor prognosis and is not easy to diagnose, because symptoms emerge in infants accompanying progressively worsening pulmonary hypertension. A nine-month-old infant visited our hospital due to recurrent respiratory infections and hemoptysis. He was diagnosed with congenital pulmonary vein stenosis by cardiac catheterization. Cardiac catheterization findings revealed that two pulmonary veins were stenotic and one pulmonary vein was occluded. Pulmonary artery catheterization showed the evidence of severe pulmonary hypertension with a mean pulmonary artery pressure of 55 mmHg and a pulmonary artery wedge pressure of 30 mmHg. Due to the aggravation of pulmonary hypertension symptoms, we performed sutureless pericardial marsupialization to reduce the obstruction and the restenosis, but he died a week. In cases of infants with recurrent hemoptysis, we should rule out congenital pulmonary vein stenosis.