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.

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Journal
JAMA Cardiology
Published
2026-10-30
DOI
https://doi.org/10.1001/jamacardio.2026.4834
Primary Topic
Pulmonary Hypertension Research and Treatments
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article
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article

Systemic Hypertension and Survival in Pulmonary Hypertension

Luke Howard, Jean Elwing, Alexandra Arvanitaki, Joanna Pepke‐Żaba et al.
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JAMA Cardiology
Pulmonary Hypertension Research and Treatments
article

Systemic Hypertension and Survival in Pulmonary Hypertension

Luke Howard, Jean Elwing, Alexandra Arvanitaki, Joanna Pepke‐Żaba, Evan L. Brittain, David Gerard Kiely, Arun Jose, Philipp Douschan, O.O. Torbas, Werner Seeger, Paul M. Hassoun, Anastasia Anthi, Raphael W. Majeed, Khaled Musa Mohammad Saleh, Zhenguo Zhai, Laura Scelsi, Stephen Y. Chan, Effrosyni Dima, Mona Lichtblau, Mark R. Toshner, Yu.M. Sіrenko, Hector R. Cajigas, Imad Al Ghouleh, Hiromi Matsubara, Roham T. Zamanian, Max Berger, Thenappan Thenappan, James Anderson, Aparna Balasubramanian, Sandeep Sahay, Marlize Frauendorf, Stefano Ghio, Lars Harbaum, Hans F. E. Klose, Hani Mohamed Sabbour, Eckart M. D. D. De Bie, Andrew J. Sweatt, Jeffrey S. Annis, Khodr Tello, Allan Lawrie, Melanie A. Lavender, Meike Fünderich, Paul G Williams, Athiththan Yogeswaran, Hossein Ardeschir Ghofrani, Ekkehard Grünig, Martin R. Wilkins, Keiichiro Kuronuma, Gábor Kovács, Ziad Konswa, George Giannakoulas, Robert A. Frantz, Silvia Ulrich, Friedrich Grimminger, Jochen Wilhelm, John Cannon, Zhu Zhang, Christina A. Eichstädt, Horst Olchewski, PVRI GoDeep Study Group
article en

Abstract

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.

JAMA Cardiology
Houston Methodist (US), University of Minnesota (US), Mayo Clinic (US), Johns Hopkins University (US), University of Pittsburgh (US), Chinese Academy of Medical Sciences & Peking Union Medical College (CN), National and Kapodistrian University of Athens (GR), Medical University of Graz (AT), Aristotle University of Thessaloniki (GR), University of Cambridge (GB), Brown University (US), Milpark Hospital (ZA), Royal Papworth Hospital (GB), Johns Hopkins Medicine (US), Shupyk National Healthcare University of Ukraine (UA), Fiona Stanley Hospital (AU), China-Japan Friendship Hospital (CN), Royal Hallamshire Hospital (GB), Pulmonary Vascular Research Institute (GB), University Hospital Heidelberg (DE), National Institute for Health and Care Research (GB), Humboldt-Universität zu Berlin (DE), University Hospital of Zurich (CH), Sunshine Coast University Hospital (AU), University Medical Center Hamburg-Eppendorf (DE), German Center for Lung Research (DE), Universities of Giessen and Marburg Lung Center (DE), Stanford Medicine (US), Westfälische Hochschule (DE), National Scientific Center "M.D. Strazhesko Institute of Cardiology" (UA), Policlinico San Matteo Fondazione (IT), Okayama Medical Center (JP), Cardio-Pulmonary Institute (DE), Evangelismos Hospital (GR), Cleveland Clinic Abu Dhabi (AE), NIHR Sheffield Biomedical Research Centre (GB), Imperial College London (GB), University of Cincinnati (US), Charité - Universitätsmedizin Berlin (DE), RWTH Aachen University (DE), Vanderbilt University Medical Center (US), University of Sheffield (GB), Stanford University (US)
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Pulmonary Hypertension Research and Treatments
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