肺動脈四尖弁による肺動脈弁狭窄兼閉鎖不全症に巨大肺動脈瘤を合併した1例

Giant Pulmonary Artery Aneurysm Associated with Pulmonary Valve Regurgitation and Stenosis Due to Quadricuspid Pulmonary Valve：Report of a Case

はじめに　肺動脈瘤はまれな疾患であり，肺高血圧を伴う先天性心疾患や肺動脈弁狭窄に合併することが知られている．肺動脈四尖弁による肺動脈弁狭窄兼閉鎖不全症に伴う肺動脈瘤の手術例を経験したので，報告する．

A 64-year-old woman was diagnosed with dilatation of the pulmonary artery and pulmonary valve stenosis approximately 10 years ago. At the age of 63, she developed hemoptysis and was referred to our hospital. The pulmonary trunk was enlarged to 63 mm with moderate pulmonary valve insufficiency. The transpulmonary valve pressure gradient was 25 mmHg；thus, surgery was performed. A median sternotomy revealed a markedly dilated pulmonary trunk growing into the pericardial cavity. After opening the patient’s pulmonary trunk to check the pulmonary valve, a thickened and shortened quadricuspid valve was observed. We replaced the pulmonary valve with a bioprosthetic valve and used a vascular prosthesis to reconstruct the pulmonary artery. The postoperative course was uneventful, and she was discharged 22 days after the surgery. Histopathological examination of the pulmonary artery aneurysm wall revealed cystic medial necrosis.