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与马凡综合征相关的大型肺动脉瘤。

Large pulmonary artery aneurysm associated with Marfan syndrome.

作者信息

Ting Peter, Jugdutt Bodh I, Le Tan Ju

机构信息

National Heart Centre, Singapore;

出版信息

Int J Angiol. 2010 Spring;19(1):e48-50. doi: 10.1055/s-0031-1278365.

DOI:10.1055/s-0031-1278365
PMID:22477577
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2949994/
Abstract

Pulmonary artery aneurysms are rare, and published data on the subject are limited. The first case of Marfan syndrome associated with a large pulmonary artery aneurysm complicated by pulmonary artery thrombi and pulmonary hypertension is described. Serial echocardiograms showed progressive dilation of the aneurysm, which reached a massive size of 90 mm. Cardiac catheterization, pulmonary angiography and computed tomography angiography were used in the preoperative evaluation. The patient died suddenly while awaiting a decision for surgical intervention, thereby underscoring the need for aggressive management.

摘要

肺动脉瘤较为罕见,关于该主题的已发表数据有限。本文描述了首例与大型肺动脉瘤相关的马凡综合征,该动脉瘤并发肺动脉血栓和肺动脉高压。系列超声心动图显示动脉瘤逐渐扩张,最大直径达90毫米。术前评估采用了心导管检查、肺血管造影和计算机断层血管造影。患者在等待手术干预决策时突然死亡,这凸显了积极治疗的必要性。

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本文引用的文献

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Mitral valve prolapse in Marfan syndrome: an old topic revisited.马凡综合征中的二尖瓣脱垂:一个重新审视的老话题。
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Pulmonary artery root dilatation in Marfan syndrome: quantitative assessment of an unknown criterion.马凡综合征中的肺动脉根部扩张:对一项未知标准的定量评估。
Heart. 2002 May;87(5):470-1. doi: 10.1136/heart.87.5.470.
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Idiopathic main pulmonary artery aneurysm.特发性主肺动脉瘤
Ann Thorac Surg. 2001 May;71(5):1688-90. doi: 10.1016/s0003-4975(00)02568-6.
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Untreated idiopathic aneurysm of the pulmonary artery; long-term follow-up.
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Lung transplantation for primary pulmonary hypertension and giant pulmonary artery aneurysm.原发性肺动脉高压和巨大肺动脉瘤的肺移植
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Revised diagnostic criteria for the Marfan syndrome.马凡综合征的修订诊断标准。
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