Tessier Steven, Lipton Brooke A, Ido Firas, Longo Santo, Nanda Sudip
Lewis Katz School of Medicine, Temple University, Philadelphia, USA.
Department of Pulmonary and Critical Care, St. Luke's University Health Network, Bethlehem, PA, USA.
Int J Crit Illn Inj Sci. 2021 Jul-Sep;11(3):167-176. doi: 10.4103/IJCIIS.IJCIIS_127_20. Epub 2021 Sep 25.
Arteriovenous malformations (AVMs) are abnormal communications between arteries and veins that lack intervening capillary beds. They have been described in almost every organ in the body, emerging sporadically or as part of well-described syndromes. Hereditary hemorrhagic telangiectasia (HHT) is a rare, progressive, and lifelong disease characterized by AVMs and recurrent hemorrhaging. In the last 2 decades, significant advances have been made in understanding the pathogenesis of this condition. The accumulation of knowledge has led to a natural evolution of therapy, from open surgery to endovascular procedures, and now to a role for medications in certain AVMs. Here, we review a case of HHT and describe the most up-to-date clinical practice, including diagnosis of HHT, subtypes of HHT, and medical therapy.
动静脉畸形(AVM)是动脉与静脉之间的异常连通,其间缺乏毛细血管床。它们在人体几乎每个器官中都有描述,可散发出现或作为某些明确综合征的一部分出现。遗传性出血性毛细血管扩张症(HHT)是一种罕见的、进行性的终身疾病,其特征为动静脉畸形和反复出血。在过去20年里,在了解这种疾病的发病机制方面取得了重大进展。知识的积累导致了治疗方法的自然演变,从开放手术到血管内介入手术,现在药物在某些动静脉畸形的治疗中也发挥了作用。在此,我们回顾一例HHT病例,并描述最新的临床实践,包括HHT的诊断、HHT的亚型以及药物治疗。