Systemic Hypertension and Survival in Pulmonary Hypertension

Importance Pulmonary hypertension (PH), a progressive and life-threatening condition with rising prevalence among older adults, frequently coexists with systemic hypertension, the prognostic impact of which is poorly understood. Objective To evaluate the association between systemic hypertension and survival in patients with PH. Design, Setting, and Participants This multicenter cohort study analyzed data from the Pulmonary Vascular Research Institute GoDeep meta-registry, integrating global retrospective and prospective PH cohorts (n = 3238 in Europe, n = 3014 in North America, n = 96 in Africa, n = 802 in Asia, and n = 851 in Oceania). Survival was assessed using multivariable Cox proportional hazards models and adjusted for key confounders, with sensitivity analyses to validate robustness. Independent external validation was performed in a cohort in the UK. A total of 12 771 patients with incident PH (median [IQR] age, 65 [53-73] years; 7372 [58%] female) were diagnosed via right heart catheterization and comprehensive clinical data, encompassing PH groups 1-5 and all age categories. The study spans diagnosis decades from 1980 to 2020. Data were analyzed from February 2025 until September 2026. Exposures Center-reported diagnosis of systemic hypertension, baseline systolic systemic arterial pressure (sSAP), and heart rate as well as longitudinal sSAP and heart rate trajectories. Main Outcomes and Measures All-cause mortality, adjusted for confounding variables, including age, PH group, antihypertensive and PH-specific medications, and cardiac index. Results Higher baseline sSAP and preexisting systemic hypertension were independently associated with improved survival. Patients with hypertension exhibited a 26% reduced mortality hazard (hazard ratio [HR], 0.74; 95% CI, 0.68-0.79) compared to their counterparts without hypertension. Survival benefits correlated positively with increasing sSAP (80-180 mm Hg), remained consistent across PH groups and age groups, and were independent of antihypertensive and PH-specific medications. Elevated heart rate was associated with higher mortality. sSAP increments correlated with improved cardiac index and tricuspid annular plane systolic excursion. Decreased sSAP and higher heart rate during follow-up correlated with higher mortality. External validation in a separate UK cohort confirmed these findings. Conclusion and Relevance In this global PH cohort, higher sSAP was associated with enhanced survival, while a higher baseline heart rate and declining systemic blood pressure during follow-up were significantly associated with increased mortality risk.