特発性肺動脈拡張症の1剖検例

Abstract

A 63-year-old female was admitted to our hospital in April, 1988, for evaluation of cardiac murmur and a very prominent pulmonary artery segment on chest roentgenogram. She complained of intermittent palpitation. She was known to have hepatic dysfunction for seven years. On physical examination, grade 3/6 ejection murmur and diastolic regurgitant murmur were audible in the left third intercostal space adjacent to the sternum. Echocardiogram, CT scan of the chest, and pulmonary artery angiogram revealed marked dilatation of the main trunk of the pulmonary artery. On cardiac catheterization, mild pulmonary hypertension was also present. She was diagnosed as having idiopathic dilatation of the pulmonary artery (IDPA) and discharged in May, 1988. She was seen at anther hospital and died suddenly in August, 1988. Autopsy revealed marked dilatation of the main trunk of the pulmonary artery. Pulmonary emphysema, mild cardiac hypertrophy, and type B hepatic cirrhosis were also observed. Light microscopically, a decrease of elastic fibers was noted in the wall of the main pulmonary artery and alcian-blue stain positive deposits were seen in the lesion. Similar findings were present in the wall of the aorta and the main trunk of the left coronary artery. Although the etiology of IDPA is not fully understood, the microscopic findings in this case suggest an etiological similarity between Marfan syndrome and IDPA.