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原发性肺动脉高压合并肺动脉瘤的病例报告

Development of pulmonary aneurysm in primary pulmonary hypertension: a case report.

作者信息

Nienaber C A, Spielmann R P, Montz R, Bleifeld W, Mathey D G

出版信息

Angiology. 1986 Apr;37(4):319-24. doi: 10.1177/000331978603700409.

Abstract

Selected cases of severe primary pulmonary arterial hypertension and associated pulmonary vascular disease have been related to the oral ingestion of aminorex fumarate, an anorexigen obviously responsible for an epidemic of primary pulmonary hypertension in Western Europe between 1967 and 1970. This report describes a fifteen year follow-up of a female patient with aminorex fumarate related pulmonary hypertension and the uncommon finding of the formation of an excessive fusiform pulmonary trunk aneurysm in the late stage of the disease process. The progressive clinical course was followed by serial chest x-ray films and repeat right heart catheterization. The diagnosis of a main stem pulmonary artery aneurysm was noninvasively established by two-dimensional echocardiography and confirmed by contrast-enhanced computed tomography and radionuclide blood pool imaging. The patient is alive, thus no histologic correlate of this entity is available at present.

摘要

部分严重原发性肺动脉高压及相关肺血管疾病病例与口服富马酸阿米雷司有关,这种食欲抑制剂显然是导致1967年至1970年间西欧原发性肺动脉高压流行的原因。本报告描述了一名患有富马酸阿米雷司相关性肺动脉高压女性患者的15年随访情况,以及在疾病后期出现罕见的梭形肺动脉主干瘤形成的情况。通过系列胸部X光片和重复右心导管检查跟踪疾病的进展过程。通过二维超声心动图无创诊断出主肺动脉瘤,并经对比增强计算机断层扫描和放射性核素血池显像证实。患者仍然存活,因此目前尚无该病变的组织学相关证据。

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