左冠動脈—肺動脈瘻を伴なった全身性進行性硬化症の一症例

A case of progressive systemic sclerosis with left coronary artery-pulmonary artery fistula

　先天性冠動脈瘻は，従来，稀な疾患と考えられてきたが^1〜3），選択的冠動脈造影法の普及により，本邦における本症の報告例は現在までに300例をこす多数を認めるようになり，冠動脈瘻はもはや稀な疾患とはいえなくなっている現状である。

　一方，全身性進行性硬化症（PSS）は，膠原病のひとつであり，主として，皮膚，肺，腎，消化器系の臓器を侵す全身性疾患であるが，心臓をも侵すことが知られ^4），PSSによる心病変（"scleroderma heart"）に関する報告例も増加してきている^{5〜13,20〜22）}。しかし，PSSによる心病変と先天性冠動脈瘻を合わせ有する症例の報告は，調べえた限りにおいて認められなかった。

A 24 years old female with progressive systemic sclerosis was referred to our hospital for the evalua-tion of intraventricular conduction defect on the electrocardiogram and the decrease in the ejection fraction of the left ventricle as well as the depressed left ventricular wall motion in the inferior, posteriorand lateral walls, found by echocardiography, suggestive of cardiomyopathy. Cardiac catheteriza-tion study disclosed the increased pulmonary arterial wedge pressure and left ventricular diastolic pres-sure. Left ventriculography disclosed the increase in the left ventricular end-diastolic volume index (113ml/cm²) and the decrease in ejection fraction (33%). In addition, segments 1 through 4, 6 and 7 of the left ventricular wall were found to be hypokinetic, and segment 5 akinetic. Myocardial biopsy specimens obtained from the subendocardium of the left ventricle showed mild patchy fibrosis of the myocardium, suggestive of cardiomyopathy due to progressive systemic sclerosis. On the other hand, we performed selective coronary arteriography, and found a coronary artery fistula between the left anterior descending artery and main pulmonary artery. However, there were no narrowings in the right and left coronary arteries. The patient with congenital coronary artery fistula, particularly the left coronary artery-pulmonary artery fistula, asso-ciated with cardiomyopathy probably caused by progressive systemic sclerosis like this patient seems to be relatively rare.