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광범위한 폐정맥 폐쇄와 동반된 원발성 폐동맥 고혈압 1 예
김영훈,김혜경,강경호,진동규,오동주,박창규,심완주,박정의,서홍석,노영무,임도선 대한내과학회 1993 대한내과학회지 Vol.45 No.3
Primary pulmonary hypertension has been subdivided into several subsets, including plexogenic, theromoembolic, pulmonary veno-occlusive disease, and others. Pulmonary veno-occlusive disease (PVOD) is one of the least cause of clinical pulmonary hypertension, with approximately 100 patients reported in would medical literature, and no case reported in Korea. Here we report on a 35-year-old woman had primary pulmonary hypertension presenting with massive hemoptysis and secondary atelectasis. Her simple chest x-ray, C-T scan, pulmonary angiogram and cardiac catheterization findings were compatible with typical primary pulmonary hypertension, except elevated pulmonary capillary wedge pressure up to 26 mmHg. that was pathognomonic feature of pulmonary veno-occlusive disease subset of primary pulmonary hypertension. The laboratory findings were compatible with primary pulmonary hypertension and could rule out the secondary pulmonary hypertension and other cardiac diseases. Left lingular segment atelectasis due to massive hemoptysis clots was resolved by removal of large blood clots by bronchoscopy. Her pulmonary artery pressure was not response to the vasodilator trials including nifedipine and captopril. We report a case of PVOD with a review of the literature.