A case of Pulmonary Veno-occlusive Disease

폐정맥 패쇄에 의한 폐고혈압증 1예

Pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension in which the primary abnormality is obliterative obstruction of pulmonary veins, especially venules. Clinicaly, we should suspect this disease in the case of congestive cardiac failure with pulmonary hypertension, chronic interstitial pulmonary edema, and normal or elevated wedge pressure on cardiac catheterization. We experience a case of pulmonary hypertension due to pulmonary veno-occlusive disease. A 55-years -old woman developed progressive dry cough and dyspnea for 3 months. Physical examination showed normal heart sounds, diffuse crackles in the whole lung fields. The liver was not palpable and pitting edema was absent. The diagnosis was made by chest HRCT, 2-D echocardiography, normal pulmonary capillary wedge pressure on cardiac catheterization, and confirmed by thoracoscopic lung biopsy. This patient was treated with vasodilator(calcium antagonist) and with mild symptomatic improvement. We reported a case of pulmonary veno-occlusive disease with review of literatures.

폐정맥 폐쇄성 폐고혈입증은 드문 질환이기는 하나 폐부종이 동반된 폐고혈압증에서 반드시 의심해야 하며 폐생검을 통해 확진할 수 있고, 폐조직 검사상 폐동맥의 침범이 있다해도 이질환의 가능성을 배제할 수 없다. 저자들은 폐부종을 동반한 폐고혈압증에서 심도자 검사상 폐동맥쐐기압이 정상이고 다른 심장 질환이 없는 것올 확인한 후 폐조직 생검으로 진단된 폐정맥 폐쇄에 의한 폐고혈압증을 경험하였기에 문헌 고찰과 함께 보고하는 바이다.