반복적인 객혈을 보인 영아에서 진단된선천성 폐정맥 협착 1례
A Case of Congenital Pulmonary Vein Stenosis Diagnosedin an Infant with Recurrent Hemoptysis
1
조회
0
다운로드
1
인용
0
참고문헌
초록
선천성 폐정맥협착증은 조기 진행성 폐고혈압과 심부전을 영아에서 일으킬 수 있으며 사망에 이르게 할 수 있는 질환으로, 반복적인 호흡기 감염과 객혈을 보인 영아에서 심도자술을 통해 확진한 1례를 보고하는 바이다.
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.
참고문헌 (11)
- Reye RD, "Congenital stenosis of the pulmonary veins in their extrapulmonary course", pp.801~2
- Latson LA, "Congenital and acquired pulmonary vein stenosis", 115, pp.103~108 (2007)
- Driscoll DJ, "Congenital stenosis of individual pulmonary veins clinical spectrum and unsuccessful treatment by transvenous balloon dilation", pp.1767~72
- Sade RM, "Stenosis of individual pulmonary veins Review of the literature and report of a surgical case", pp.953~62, Castaneda AR
- Holt DB, "Primary pulmonary vein stenosis", 99, pp.568~572 (2007)
- Sadr IM, "Mechanism of pulmonary vein stenosis in infants with normally connected veins", 86, pp.577~579 (2000)
- Fong LV, "Morphologic features of stenosis of the pulmonary veins", pp.1136~8
- Breinholt JP, "Pulmonary vein stenosis with normal connection: associated cardiac abnormalities and variable outcome", 68, pp.164~168 (1999)
- Minich LL, "Complete follow-up echocardiograms are needed to detect stenosis of normally connecting pulmonary veins", 18, pp.589~592 (2001)
- Nuesslein TG, "Pulmonary haemosiderosis in infants and children", 7, pp.45~48 (2006)
- van Son JA, "Repair of congenital and acquired pulmonary vein stenosis", 60, pp.144~150 (1995)