Survival rates at one and five years for patients with group 1 pulmonary arterial hypertension at high altitude: A retrospective cohort study
- PMID: 40233134
- PMCID: PMC12035330
- DOI: 10.1177/00368504251336066
Survival rates at one and five years for patients with group 1 pulmonary arterial hypertension at high altitude: A retrospective cohort study
Abstract
ObjectivesGroup 1 pulmonary arterial hypertension (PAH) causes increased vascular resistance, right heart failure, and reduced survival. High-altitude PAH survival data are limited despite chronic hypoxia's potential impact on disease progression and risk stratification. The objective was to evaluate survival stratified by the European Society of Cardiology/European Respiratory Society (ESC/ERS) baseline risk score, REVEAL risk score 2.0, and etiologies in patients with PAH at high altitudes.MethodsA retrospective cohort study was conducted to evaluate functional and hemodynamic variables and risk stratification using the ESC/ERS baseline risk score and REVEAL risk score 2.0. One-year and five-year survival rates were determined from admission to the pulmonary hypertension program until death or the end of follow-up.ResultsA total of 188 patients were included. The overall survival rate at one year was 95.8%, and at five years it was 86.8%. Precisely, 50% (95 out of 188) had congenital heart disease, 34% (63 out of 188) had idiopathic, and 13% (24 out of 188) had connective tissue disease. Risk stratification using REVEAL risk score 2.0 classified 58% (109 out of 188) of patients as low risk, 26% as intermediate risk, and 16% (30 out of 188) as high risk. Using the ESC/ERS baseline risk score, Stratification indicated 78% (146 out of 188) low risk, 21% (40 out of 188) intermediate risk, and 1% (2 out of 188) high risk. High risk according to ESC/ERS baseline risk score (p < 0.001) and REVEAL risk score 2.0 (p < 0.001) was associated with lower survival. Idiopathic etiology was associated with a lower survival rate (p = 0.011).ConclusionThe survival of patients with PAH at a high-altitude expert center was 95.8% at one year and 86.8% at five years. Risk stratification using the REVEAL risk score 2.0 and the ESC/ERS baseline risk score was associated with higher mortality in patients classified as high risk.
Keywords: Pulmonary hypertension; altitude; observational study; survival; treatment.
Conflict of interest statement
Conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Figures
References
-
- Benza RL, Miller DP, Barst RJ, et al. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL registry. Chest 2012; 142: 448–456. - PubMed
-
- Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987; 107: 216–223. - PubMed
-
- Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022; 43: 3618–3731. - PubMed
-
- Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European respiratory society (ERS): endorsed by: association for European Paediatric and Congenital Cardiology (AEPC), international society for heart and lung transplantation (ISHLT). Eur Heart J 2016; 37: 67–119. - PubMed
MeSH terms
LinkOut - more resources
Full Text Sources
Medical
