Bonderman D, Nowotny R, Skoro-Sajer N, Adlbrecht C, Lang I M
Department of Cardiology, Medical University of Vienna, Vienna, Austria.
Eur J Clin Invest. 2006 Sep;36 Suppl 3:71-2. doi: 10.1111/j.1365-2362.2006.01683.x.
Hereditary haemorrhagic telangiectasia (HHT) is a disorder of arteriovenous malformations and telangiectases. In rare cases affected individuals may develop typical pulmonary arterial hypertension (PAH). Vasodilator therapy has not been recommended because of a potential increase in arteriovenous shunt volume. This report is on two patients with severe HHT-associated PAH who were treated with bosentan, an oral endothelin ET(A)/ET(B) receptor antagonist. After 1 year, symptomatic and functional improvements were confirmed by haemodynamic evaluation demonstrating a significant decrease of mean pulmonary artery pressures and an increase in cardiac index, without evidence for an increase in effective shunt volume.
遗传性出血性毛细血管扩张症（HHT）是一种动静脉畸形和毛细血管扩张的疾病。在罕见情况下，受影响个体可能会发展为典型的肺动脉高压（PAH）。由于可能增加动静脉分流体积，不推荐使用血管扩张剂治疗。本报告介绍了两名患有严重HHT相关性PAH的患者，他们接受了波生坦治疗，波生坦是一种口服内皮素ET(A)/ET(B)受体拮抗剂。1年后，血流动力学评估证实了症状和功能的改善，显示平均肺动脉压显著降低，心脏指数增加，且无有效分流体积增加的证据。
