Survival rates at one and five years for patients with group 1 pulmonary arterial hypertension at high altitude: A retrospective cohort study

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 (<i>p</i> < 0.001) and REVEAL risk score 2.0 (<i>p</i> < 0.001) was associated with lower survival. Idiopathic etiology was associated with a lower survival rate (<i>p</i> = 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.