Correa Ricardo Amorim, Rezende Camila Farnese, Mancuzo Eliane Viana, Mickael Claudia, Loureiro Camila M C, K F Oliveira Rudolf, Hilton Joan F, Graham Brian B
Medical School, Hospital das Clínicas, Pulmonary Vascular Diseases Section Federal University of Minas Gerais Belo Horizonte Minas Gerais Brazil.
Hospital das Clínicas Federal University of Minas Gerais, Pulmonary Vascular Diseases Section Belo Horizonte Minas Gerais Brazil.
Pulm Circ. 2025 Apr 27;15(2):e70086. doi: 10.1002/pul2.70086. eCollection 2025 Apr.
Data about pulmonary arterial hypertension (PAH) patients living in low- and middle-income countries remain scarce. This study assessed prognostic factors associated with time to clinical worsening (CW) or death of a cohort of PAH patients in Minas Gerais, Brazil. This retrospective cohort study describes baseline clinical variables by PAH etiology and estimates time from diagnosis to CW [all-cause death, any-cause hospitalization, or disease progression (decrease of ≥ 15% in 6MWD and need for additional PAH therapy or worsening of functional class (FC)] and time to death. 79 out of 102 participants developed CW and 38 died while under follow-up. The most prevalent etiologies were PAH associated with schistosomiasis (PAH-Sch), idiopathic (IPAH), with congenital heart disease (PAH-CHD), and with connective tissue disease (PAH-CTD). The overall median event-free time to CW was 3.3 (95% CI, 2.3-4.6) years, which was similar across etiologies (log-rank test: = 0.12). WHO FC III-IV, DLCO < 70%, heart rate recovery in 1 min after the 6-min walk test (HRR1) < 18 beats/minute, and baseline mPAP ≥ 50 mmHg were predictive of CW-free time. The median time to all-cause mortality was 10.2 (95% CI, 6.8 - > 10) years and varied among etiologies (log-rank test: < 0.001). Time to CW was statistically independent of PAH etiology but depended on baseline WHO FC, DLCO, HRR, and mPAP. After CW events, PAH-Sch and PAH-CTD survived less on average than IPAH and PAH-CHD participants.
关于生活在低收入和中等收入国家的肺动脉高压（PAH）患者的数据仍然匮乏。本研究评估了巴西米纳斯吉拉斯州一组PAH患者临床恶化（CW）或死亡时间的相关预后因素。这项回顾性队列研究按PAH病因描述了基线临床变量，并估计了从诊断到CW[全因死亡、任何原因住院或疾病进展（6分钟步行距离（6MWD）下降≥15%且需要额外的PAH治疗或功能分级（FC）恶化）]和死亡时间。102名参与者中有79人出现CW，38人在随访期间死亡。最常见的病因是与血吸虫病相关的PAH（PAH-Sch）、特发性（IPAH）、先天性心脏病相关的PAH（PAH-CHD）和结缔组织病相关的PAH（PAH-CTD）。CW的总体无事件中位时间为3.3（95%CI，2.3 - 4.6）年，各病因之间相似（对数秩检验：=0.12）。WHO FC III-IV、DLCO<70%、6分钟步行试验后1分钟心率恢复（HRR1）<18次/分钟以及基线平均肺动脉压（mPAP）≥50 mmHg可预测无CW时间。全因死亡率的中位时间为10.2（95%CI，6.8 ->10）年，且各病因之间有所不同（对数秩检验：<0.001）。CW时间在统计学上与PAH病因无关，但取决于基线WHO FC、DLCO、HRR和mPAP。CW事件发生后，PAH-Sch和PAH-CTD参与者的平均生存期低于IPAH和PAH-CHD参与者。
