Conde-Camacho Rafael, Tuta-Quintero Eduardo, Bastidas-Goyes Alirio, Giraldo-Cadavid Luis F, Barón Oscar, Mora Angelica
Department of Pulmonary Hypertension, Fundación Neumológica Colombiana, Bogotá, Colombia.
Department of Biosciences, Universidad de La Sabana, Chía, Colombia.
Sci Prog. 2025 Apr-Jun;108(2):368504251336066. doi: 10.1177/00368504251336066. Epub 2025 Apr 15.
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 ( < 0.001) and REVEAL risk score 2.0 ( < 0.001) was associated with lower survival. Idiopathic etiology was associated with a lower survival rate ( = 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.
目的
第1组肺动脉高压（PAH）会导致血管阻力增加、右心衰竭和生存率降低。尽管慢性缺氧可能对疾病进展和风险分层产生影响，但高海拔地区PAH的生存数据有限。目的是评估高海拔地区PAH患者按欧洲心脏病学会/欧洲呼吸学会（ESC/ERS）基线风险评分、REVEAL风险评分2.0以及病因分层的生存率。
方法
进行了一项回顾性队列研究，以使用ESC/ERS基线风险评分和REVEAL风险评分2.0评估功能和血流动力学变量以及风险分层。从进入肺动脉高压项目到死亡或随访结束确定1年和5年生存率。
结果
共纳入188例患者。1年总生存率为95.8%，5年为86.8%。确切地说，50%（188例中的95例）患有先天性心脏病，34%（188例中的63例）为特发性，13%（188例中的24例）患有结缔组织病。使用REVEAL风险评分2.0进行风险分层，将58%（188例中的109例）患者分类为低风险，26%为中度风险，16%（188例中的30例）为高风险。使用ESC/ERS基线风险评分进行分层，结果显示78%（188例中的146例）为低风险，21%（188例中的40例）为中度风险，1%（188例中的2例）为高风险。根据ESC/ERS基线风险评分（<0.001）和REVEAL风险评分2.0（<0.001），高风险与较低的生存率相关。特发性病因与较低的生存率相关（=0.011）。
结论
高海拔专家中心PAH患者的1年生存率为95.8%，5年生存率为86.8%。使用REVEAL风险评分2.0和ESC/ERS基线风险评分进行风险分层与分类为高风险患者的较高死亡率相关。
