[Survival status and prognostic factors analysis in patients with hereditary hemorrhagic telangiectasia-associated pulmonary arterial hypertension].

<b>Objective:</b> To analyze the survival status and prognostic factors of patients with hereditary hemorrhagic telangiectasia-associated pulmonary arterial hypertension (HHT-PAH). <b>Methods:</b> This prospective study included patients diagnosed with HHT-PAH at the Fuwai Hospital of the Chinese Academy of Medical Sciences and Guangdong General Hospital from August 2009 to December 2019. Patients were followed up every 6 months±2 weeks, with all-cause mortality as the study endpoint. Multivariate Cox proportional hazards regression mode was used to further screen and validate the prognostic factors. Survival curves were plotted using the Kaplan-Meier method, and the log-rank test was used to compare the survival time differences between patients with and without hepatic arteriovenous malformations. <b>Results:</b> A total of 18 HHT-PAH patients were included, with 14 females (77.8%) and a median age [<i>M</i> (<i>Q</i>₁, <i>Q</i>₃)] of 39 (32, 45) years. The median follow-up time was 129 (51, 156) months. During the follow-up period, 12 patients (66.7%) died, with a median survival time of 70 (32, 123) months. The 1-year, 3-year, 5-year, and 10-year survival rates were 100.0%, 70.8%(95%<i>CI</i>: 52.3%-96.0%), 53.1%(95%<i>CI</i>: 34.0%-83.0%), and 26.6%(95%<i>CI</i>: 10.6%-66.4%), respectively. HHT-PAH patients with hepatic arteriovenous malformations had a shorter median survival time than those without hepatic arteriovenous malformations [31.0 months (95%<i>CI</i>: 16.6-45.4 months) vs 84.0 months (95%<i>CI</i>: 54.4-113.6 months), <i>P</i>=0.024]. Cox proportional hazards regression analysis showed that hepatic arteriovenous malformation (<i>HR</i>=4.493, 95%<i>CI</i>: 1.064-18.978) was a risk factor for all-cause mortality in HHT-PAH patients. <b>Conclusions:</b> The 10-year survival rate of HHT-PAH patients is poor. The presence of hepatic arteriovenous malformations is a risk factor for mortality in HHT-PAH patients.