Comorbid pulmonary hypertension in hospitalized sickle cell disease patients was associated with increased mortality compared to sickle cell disease alone (3.5% vs 1.0%; aOR 1.77, 95% CI 1.34-2.34).
Observational (n=199,140)
Yes
Effect estimate: aOR 1.77 (95% CI 1.34-2.34)
Absolute Event Rate: 3.5% vs 1%
Abstract Rationale Although the pathogenesis of pulmonary hypertension (PH) in patients with sickle cell disease (SCD) is not completely understood, it is a frequent complication with negative prognostic implications. PH is an independent risk factor for mortality among patients with SCD. We sought to use the National Inpatient Sample (NIS) to compare the outcome of patients hospitalized with concurrent PH and SCD as compared to patients with SCD alone. Methods We conducted a retrospective review of the NIS database from January 2018 to December 2022. Patients were identified using ICD-10 codes for SCD and PH in any diagnosis field. Survey-weighted methods analyzed prevalence, trends, and outcomes. Logistic regression estimated crude and adjusted odds ratios (aOR) for mortality, adjusting for age, sex, income, Charlson index, hospital region, bed size, payer, and teaching status. Linear regression assessed length of stay (LOS) and charges; trend tests used logistic models. Results Of 199, 140 hospitalized patients with SCD, PH was comorbid in 5. 5% of cases. The frequency of comorbid PH rose from 5. 2% in 2018 to 5. 7% in 2022 (trend p 0. 001). SCD patients with comorbid PH were older (48. 9 vs. 31. 7 years, p 0. 001), more female (61% vs. 58%, p = 0. 017), and likelier to have a Charlson Comorbidity index score of ≥ 3 (50. 8% vs. 15. 9%, p 0. 001). Comorbid PH was associated with increased mortality (3. 5% vs. 1. 0%; crude OR 3. 59, 95% CI 2. 79-4. 60; aOR 1. 77, 95% CI 1. 34-2. 34), LOS (6. 9 vs. 5. 0 days, p 0. 001) and charges (81, 883 vs. 54, 353, p 0. 001). Frequency of multimorbid CKD (33. 8% vs. 9. 5%), ESRD (17. 8% vs. 4. 9%), and COPD (19. 6% vs. 7. 9%) were elevated in SCD patients with PH (all p 0. 001). PH predictors included age (aOR 1. 03/year) and obesity class 3 (aOR 1. 60). Discussion The rising prevalence of PH may reflect a manifestation of late-stage disease complication as the average lifespan of SCD patients increases, though this is confounded by the broadening definition of PH over the study timeframe. The presence of PH among patients hospitalized with SCD was associated with over a threefold increase in mortality and significantly increase in length of stay. After adjusting for covariates, PH remained an independent risk factor for mortality with an aOR of 1. 77. SCD patients with PH were older with more comorbidities. These findings are consistent with the known mortality risks associated with SCD complicated by PH. These finding highlight the growing need for vigilant PH screening and management in SCD to mitigate adverse outcomes. This abstract is funded by: None
Cohen et al. (Fri,) conducted a observational in Sickle cell disease (n=199,140). Comorbid pulmonary hypertension vs. Sickle cell disease alone was evaluated on Mortality (aOR 1.77, 95% CI 1.34-2.34). Comorbid pulmonary hypertension in hospitalized sickle cell disease patients was associated with increased mortality compared to sickle cell disease alone (3.5% vs 1.0%; aOR 1.77, 95% CI 1.34-2.34).