Hospitalizations with hereditary hemorrhagic telangiectasia and pulmonary hypertension in the United States from 2000 to 2014.

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease that causes widespread abnormal vasculature development, resulting in multiple complications including pulmonary hypertension (PH). Despite the potential severity of PH, there is a lack of data on hospitalization characteristics and outcomes in the HHT-PH population. The purpose of this analysis was to describe trends and outcomes of HHT-PH hospitalizations within the National (Nationwide) Inpatient Sample (NIS).

METHODS: Adult hospitalizations (age ≥18 years) with a principal or secondary diagnosis of HHT were identified from the 2000-2014 NIS. Records were stratified by a concurrent PH diagnosis. Trends, characteristics, and outcomes of hospitalizations with HHT and PH were analyzed.

RESULTS: There were 55,189 adult hospitalizations with HHT from 2000 to 2014, of which 4602 (8.3%) had a concurrent diagnosis of PH. HHT-PH hospitalizations rose steadily from 165 (5.1%) in 2000 to 540 (13.8%) in 2014 (p < 0.001). They were more common in females (vs. HHT without PH, 71.6% vs. 59.2%, p < 0.001) and were associated with a higher comorbidity burden (total 4.0 ± 0.1 vs. 2.4 ± 0.03, p < 0.001). Inpatient mortality was higher in HHT-PH hospitalizations than in HHT without PH (3.5% vs. 1.8%, p < 0.001). On multivariable logistic regression, the diagnosis of PH remained significantly associated with a higher risk of inpatient death (odds ratio 1.71, 95% confidence interval 1.11-2.63, p = 0.015) in HHT hospitalizations.

CONCLUSIONS: HHT-PH hospitalizations rose from 2000 to 2014. Notably, HHT patients with a concurrent PH diagnosis were at significantly higher risk of in-hospital mortality.