Background: Approximately 10% adults with Sickle Cell disease (SCD) has Pulmonary hypertension (PHTN). There are several etiologies like hypoxia driven and microvascular obstruction contributing to PHTN. However, the in-hospital outcomes of PHTN on SCD has not been well studied.
Methods: We queried 2018 National Inpatient Sample (NIS) database to identify SCD and different types of SCD hospitalizations using appropriate ICD-10 codes. SCD with PHTN group was compared to SCD without PHTN. Chi-square test and linear regression were used for categorical and continuous variables, respectively. Multivariate logistic regression was used to adjust for potential hospital and patient confounders (age, sex, race, diabetes, systolic heart failure, chronic kidney disease, charlson co-morbidity index, hospital location, teaching status, bed size and patient income status). Discharge weights provided in the database was used to calculate the national estimates. STATA 16.1 was used to perform all statistical analysis.
Results: 156,120 weighted SCD hospitalizations were identified. Of which, 9,094 (5.8%) of them had PHTN. SCD patients with PHTN were older (43 vs 35 yrs), more often female (59% vs 40%) and less often had CKD (33% vs 67%). We observed statistically significant increase in mortality [Odds Ratio (OR): 1.8 (1.2-2.7); P = <0.01], length of stay [7.1 days vs 4.6; P = <0.01], total hospitalization charges [$67,673 vs $41,043; P = <0.01], right heart failure [OR: 43 (20-91); P = <0.01], cardiogenic shock [OR: 9.7 (4.7-19.9); P < 0.01] in SCD patients with PHTN when compared to those without PHTN after adjusting for potential patient and hospital level confounders.
Conclusions: Presence of pulmonary hypertension is an independent predictor of mortality, length of stay, hospitalization charges, right heart failure and cardiogenic shock in patients with sickle cell disease. This study helps to assume prognosis and raise awareness on the intensity of care toward these patients. More research is required in SCD with PHTN pathophysiology and to decrease the morbidity and mortality in this specific group.