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遗传性出血性毛细血管扩张症(朗杜-奥斯勒-韦伯综合征)患者肺动静脉畸形的栓塞治疗

Embolotherapy for pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome).

作者信息

Andersen P E, Kjeldsen A D, Oxhøj H, Vase P, White R I

机构信息

Department of Radiology, Odense University Hospital, Denmark.

出版信息

Acta Radiol. 1998 Nov;39(6):723-6. doi: 10.3109/02841859809175505.

DOI:10.3109/02841859809175505
PMID:9817050
Abstract

PURPOSE

To evaluate the clinical results of embolization of pulmonary arteriovenous malformations (PAVMs) in patients with hereditary hemorrhagic telangiectasia (HHT), the Rendu-Osler-Weber syndrome.

MATERIAL AND METHODS

Twelve patients in the county of Fyn, Denmark, were treated with transcatheter embolization of 20 PAVMs using 12 detachable silicone balloons and 26 steel coils.

RESULTS

All PAVMs were completely occluded and we observed a significant rise in PaO2 after treatment and a significant decrease in right-to-left shunt estimated by contrast echocardiography. All patients experienced an improved functional level. One patient experienced severe pleurisy and another a rise in temperature following treatment, but otherwise no symptomatic complications were observed.

CONCLUSION

Embolotherapy is a definitive treatment for PAVMs: it is very effective with a high success rate and few complications. Patients with HHT are at risk of PAVM and should be screened and treated for PAVMs when these reach a size that is associated with complications. In the detection of PAVMs, contrast echocardiography is a very sensitive method, and follow-up of these patients can be done with contrast echocardiography.

摘要

目的

评估遗传性出血性毛细血管扩张症(HHT,即遗传性出血性毛细血管扩张综合征)患者肺动静脉畸形(PAVM)栓塞治疗的临床效果。

材料与方法

丹麦菲英岛的12例患者接受了经导管栓塞治疗,使用12个可脱卸硅胶球囊和26个钢圈对20处肺动静脉畸形进行栓塞。

结果

所有肺动静脉畸形均完全闭塞,治疗后观察到动脉血氧分压显著升高,对比超声心动图估计的右向左分流显著减少。所有患者的功能水平均有改善。1例患者治疗后出现严重胸膜炎,另1例体温升高,但未观察到其他有症状的并发症。

结论

栓塞治疗是肺动静脉畸形的确定性治疗方法:非常有效,成功率高且并发症少。遗传性出血性毛细血管扩张症患者有患肺动静脉畸形的风险,当肺动静脉畸形达到与并发症相关的大小时,应进行筛查并治疗。在肺动静脉畸形的检测中,对比超声心动图是一种非常敏感的方法,这些患者的随访可以通过对比超声心动图进行。

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