Abstract

to examine the national estimates for admission of patients with hereditary hemorrhagic telangiectasia (HHT) who were diagnosed with pulmonary arteriovenous malformations (PAVMs) and received arteriography of pulmonary arteries (PA arteriography) during their inpatient stays. Data from the National Inpatient Sample (NIS) 2012–2015 was used for this study. A survey analysis approach was used to calculate nationally representative estimates of inpatient stays of HHT patients with PAVM diagnosis. Patient and hospital characteristics, as well as short-term outcomes were compared based on PAVM diagnosis and need for PA arteriography. HHT patients were responsible for an estimated 4210 to 4450 number of admissions per year (2012–2015), 4.3% to 7.6% of which were related to a PAVM diagnosis and 1.0% to 2.0% of them required PA arteriography (45-85 per year). HHT patients with PAVM who received PA arteriography were younger (43.1 ± 3.6 vs. 60.0 ± 0.4), were more likely to be female (69% vs. 58%) and had lower frequency of chronic blood loss anemia (6% vs. 14%), congestive heart failure (3% vs. 17%), chronic pulmonary disease (14% vs. 26%) and coagulopathy (3% vs. 8%) when compared to an average pool of HHT admissions. PA arteriography was more frequently performed in large (80.0%), private-owned (85.7%), and urban teaching (91.4%) hospitals. PA arteriography admissions were associated with no significant mortality (frequency <0.1%), an average 7.4 ± 1.5 days of in-hospital stay, 77.1% routine disposition, and total hospital charge of 141,742 ± 27,994 USD. PA arteriography for PAVM in HHT patients is an uncommon procedure (1.0%-2.0% of all admissions of HHT patients), that is more commonly performed in patients with better comorbidity profiles and is associated with limited mortality.Tabled 1National Outcomes for HHT Admission According to PAVM and PA ArteriographHHTHHT + PAVMHHT + PAVM Receiving ArteriographyDied (%)2.0 (0.2)2.0 (1.1)<0.1 (<0.1)Length of Stay (days)4.6 (0.1)5.5 (0.5)7.4 (1.5)*Routine discharge (%)68.0 (0.9)71.4 (3.6)77.1 (0.7)*Total charges (USD)44,190 (1505)55,282 (4654)141,742 (27,994)**P for trend <0.05. Open table in a new tab

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