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[大动脉炎中的肺动脉高压]

[Pulmonary hypertension in Takayasu arteritis].

作者信息

Skvarilová M, Lindner J, Hanzová B, Dusková J, Jansa P, Palecek T, Aschermann M

机构信息

Centrum pro plicní arteriální hypertenzi II, interní kliniky kardiologie a angiologie 1, lékarské fakulty UK a VFN v Praze.

出版信息

Vnitr Lek. 2007 Jan;53(1):71-7.

PMID:17472018
Abstract

Takayasu arteritis (TA) is a rare chronic vasculitis primarily involving the aorta and its main branches. The disease affects women much more frequently than men, the ratio being 8:1. The onset occurs in the teenage years, always before age of 40. Aortic regurgitation is rare. The pulmonary artery stenosis may also rarely occur. We report the case of a 49-year old female patient with Takayasu arteritis who presented with severe left pulmonary trunk stenosis resulting in pulmonary hypertension, severe aortic regurgitation due to the dilatation of the ascending aorta, mitral insufficiency, critical left renal artery stenosis, and probably with left main coronary artery stenosis. No severe stenosis or occlusion in the mid portion of mid subclavian artery lesion were present. Because the patient presented with serious congestive heart failure, she underwent mitral valvuloplasty and aortic valve replacement. However, the patient died in early postoperative period due to pulmonary hypertension which failed to respond to pharmacotherapy.

摘要

高安动脉炎(TA)是一种罕见的慢性血管炎,主要累及主动脉及其主要分支。该病女性患者比男性更为常见,比例为8:1。发病于青少年时期,通常在40岁之前。主动脉瓣关闭不全罕见。肺动脉狭窄也很少发生。我们报告一例49岁患有高安动脉炎的女性患者,其表现为严重的左肺动脉主干狭窄导致肺动脉高压,升主动脉扩张引起严重主动脉瓣关闭不全,二尖瓣关闭不全,严重的左肾动脉狭窄,可能还有左冠状动脉主干狭窄。锁骨下动脉病变中部未出现严重狭窄或闭塞。由于患者出现严重的充血性心力衰竭,她接受了二尖瓣成形术和主动脉瓣置换术。然而,患者在术后早期因肺动脉高压对药物治疗无反应而死亡。

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