Despite major advances in antiretroviral treatment, pulmonary arterial hypertension (PAH) remains a common, severe complication in patients diagnosed with HIV. Treatment of HIV-associated PAH targets the nitric oxide, endothelin, and prostacyclin pathways, with limited evidence for novel therapies, as these patients have been excluded from most pivotal randomized controlled trials. We report a 58-year-old male with HIV-PAH on dual oral PAH-targeted therapy who developed acute right ventricular (RV) failure triggered by sepsis. Despite escalation of guideline-directed therapy with intravenous prostacyclin and catecholamines, the patient continued to exhibit decompensated right heart failure. Sotatercept was initiated, after which the patient showed marked improvement within 24 h, including improved echocardiographic RV function, a decline in NT-proBNP, recovery of renal function, and successful weaning from catecholamines and prostacyclin. The patient was subsequently transferred to a general ward in stable condition. This case suggests a potential role for sotatercept as rescue therapy in acute decompensated HIV-PAH, with a rapid temporal association with hemodynamic improvement. Further randomized controlled studies are warranted in this high-risk population.
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