Management and outcomes in pulmonary arterial hypertension patients with sepsis

Background: Sepsis is a common cause of death in patients with pulmonary arterial hypertension (PAH). Treatment requires careful fluid management and hemodynamic support. This study compares patients with or without PAH presenting with sepsis with a focus on initial fluid resuscitation.

Methods: This retrospective analysis compared adults with and without PAH admitted for sepsis at two academic hospitals between 2013 and 2022. Prior PAH diagnosis was verified by review of right heart catheterization data and sepsis present on admission was verified by chart review. Demographics, vital signs, laboratory values, imaging results, treatment approaches, and all-cause mortality data were obtained. Controls were propensity score weighted by age, sex, and Charlson Comorbidity index. Logistic regression models controlling for age and Charlson comorbidity indices were used to examine factors associated with survival.

Results: Thirty patients admitted for sepsis with pre-existing PAH were compared to 96 matched controls. Controls received significantly more fluids at 24 h compared to PAH patients (median 0 mL v. 1216 mL, p < 0.001), while PAH patients were more likely to receive vasoactive medications (23.3% vs. 8.3%, p = 0.037). At 30 days, 7 PAH patients (23.3%) and 13 control patients (13.5%) had died (p = 0.376). PAH patients that received more fluids had decreased mortality (OR 0.31, 95% CI 0.11-0.92, p = 0.03) and patients who received fluids had shorter mean time to antibiotics (2.3 h v. 6.5 h, p = 0.04), although decreased time to antibiotics was not associated with mortality. Patients who received no fluids more often had previously identified right ventricular systolic dysfunction (62.5% v. 28.6%, p = 0.136).

Conclusion: Patients with PAH and sepsis have high mortality and receive different treatments than controls, with more reliance on vasopressors and less on fluid resuscitation. PAH patients who received less fluids had higher mortality and those who received no fluids had a longer time to receiving antibiotics, indicating a potential delay in recognizing sepsis. Timely recognition of sepsis and dynamic decision-making around fluid resuscitation remains critical in this high-risk population.