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遗传性出血性毛细血管扩张症患者的肺动静脉畸形和栓塞并发症

Pulmonary arteriovenous malformations and embolic complications in patients with hereditary hemorrhagic telangiectasia.

作者信息

Angriman Federico, Ferreyro Bruno L, Wainstein Esteban J, Serra Marcelo M

机构信息

Departamento de Medicina Interna, Hospital Italiano de Buenos Aires, Universidad de Buenos Aires, Buenos Aires, Argentina.

Departamento de Medicina Interna, Hospital Italiano de Buenos Aires, Universidad de Buenos Aires, Buenos Aires, Argentina.

出版信息

Arch Bronconeumol. 2014 Jul;50(7):301-4. doi: 10.1016/j.arbres.2013.08.006. Epub 2014 Mar 12.

DOI:10.1016/j.arbres.2013.08.006
PMID:24629762
Abstract

Patients with hereditary hemorrhagic telangiectasia (HHT) and pulmonary arteriovenous malformation (PAVM) face higher risk of embolic complications. It is not clear whether poor outcomes are related to PAVM severity or pulmonary symptoms. Furthermore, there is currently no available data on HHT patients in Argentina. We conducted a cross sectional study in a teaching hospital in Buenos Aires, Argentina. We describe baseline characteristics of HHT and compare the prevalence of embolic complications in patients with significant PAVM compared to patients without significant PAVM. One hundred and eight consecutive patients were included. Significant PAVM was defined as: contrast echocardiography grade 2 or greater; bilateral PAVM or feeding artery bigger than 3mm; or previous PAVM treatment. Primary composite outcome was defined as: cerebrovascular accident, cerebral abscess or peripheral embolism. 20% of participants had embolic complications, the most frequent one was stroke. Embolic complications were associated with significant PAVM and respiratory symptoms.

摘要

患有遗传性出血性毛细血管扩张症(HHT)和肺动静脉畸形(PAVM)的患者面临更高的栓塞并发症风险。目前尚不清楚不良预后是否与PAVM严重程度或肺部症状有关。此外,目前阿根廷尚无关于HHT患者的可用数据。我们在阿根廷布宜诺斯艾利斯的一家教学医院进行了一项横断面研究。我们描述了HHT的基线特征,并比较了有显著PAVM的患者与无显著PAVM的患者中栓塞并发症的发生率。共纳入108例连续患者。显著PAVM的定义为:对比超声心动图2级或更高;双侧PAVM或供血动脉大于3mm;或既往有PAVM治疗史。主要复合结局定义为:脑血管意外、脑脓肿或外周栓塞。20%的参与者有栓塞并发症,最常见的是中风。栓塞并发症与显著PAVM和呼吸道症状相关。

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