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Pulmonary Hypertension in Hereditary Hemorrhagic Telangiectasia

Eileen Harder

Wassim Fares


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Conference: 2018 PHA International PH Conference & Scientific Sessions

Release Date: 06.28.2018

Presentation Type: Abstracts

File Download: Conference 2018_2027

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2018 International PH Conference and Scientific SeAbstract presented at the 2018 International PH Conference and Scientific Sessions in Orlando, Fla., June 28-July 1, 2018.


The purpose of this analysis is to study the in-hospital characteristics and outcomes of hereditary hemorrhagic telangiectasia admissions with pulmonary hypertension in a large inpatient database.


Hereditary hemorrhagic telangiectasia (HHT) is a rare genetic disease resulting in abnormal blood vessel development throughout multiple organs. As such, it has a wide variety of manifestations including pulmonary hypertension (PH). PH associated with HHT may occur as either a pre-capillary pulmonary arterial hypertension (PAH) due to HHT-related genetic mutations or as a post-capillary form due to high cardiac output. Despite the potential severity of HHT-associated PH, there is little information available on outcomes in this group. The purpose of this analysis was therefore to evaluate the in-hospital characteristics and outcomes of the HHT-PH population.


The 2000-2014 National Inpatient Sample (NIS) database was queried for all adult (18 years) discharges with HHT (International Classification of Disease, Ninth Revision, Clinical Modifications [ICD-9-CM] code 448.0 in any diagnosis column). The HHT population was then stratified by the presence of any pulmonary hypertension (identified by ICD-9-CM codes 416.0, 416.8, and 416.9). The primary outcome of interest was in-hospital mortality. Additional secondary outcomes included length of stay, total admission cost, and hospital and patient characteristics were also evaluated. Statistics were performed with survey procedures in SAS.


From 2000 to 2014, there were 55,187 hospitalizations with HHT. Of these, 4,601 (8.3%) carried a concurrent diagnosis of PH (HHT-PH). The rate of HHT-PH admissions increased steadily from 5.1% in 2000 to 13.9% in 2014 (p<0.0001). HHT-PH hospitalizations more commonly occurred in females (vs. HHT admissions without PH [non-PH HHT], 71.6% vs. 59.2%, p<0.0001), the northeast (27.7% vs. 19.4%, p=0.0004), and urban teaching hospitals (61.0% vs. 48.7%, p=0.0001). Furthermore, HHT-PH admission was more frequently associated with private insurance (vs. non-PH HHT hospitalization, 76.7% vs. 68.1%, p=0.0003). HHT-PH admissions were associated with longer length of stay (vs. non-PH HHT, 5.4 vs. 4.4 days, p<0.0001) and higher total hospital cost ($39,874 vs. $28,735, p<0.0001). In HHT-PH, more common Elixhauser comorbidities included congestive heart failure (vs. non-PH HHT, 45.6% vs. 14.5%, p<0.0001), valvular heart disease (21.5% vs. 7.4%, p<0.0001), renal failure (15.1% vs. 7.2%, p<0.0001) and fluid-electrolyte disturbances (26.8% vs. 15.9%, p<0.0001). In-hospital mortality was higher in HHT-PH hospitalizations (vs. non-PH HHT, 3.5% vs. 1.8%, p=0.0005). On multivariate analysis (MVA) adjusted for patient demographics, hospital characteristics, and pre-existing comorbidities, HHT-PH status remained a significant predictor of in-hospital death (odds ratio [OR] 1.592, 95% confidence interval [CI] 1.024-2.475, p=0.039).


PH is a relatively common complication of HHT, and admissions in HHT-PH have increased in frequency from 2000 to 2014. Hospitalization in HHT-PH is associated with a significantly higher incidence of inpatient mortality.