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.

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