Clavé Mariana M, Maeda Nair Y, Castro Claudia R P, Bydlowski Sergio P, Lopes Antonio A
1 Heart Institute, University of São Paulo School of Medicine, São Paulo, Brazil.
2 Pró-Sangue Foundation, São Paulo, Brazil.
Pulm Circ. 2017 Jul-Sep;7(3):635-642. doi: 10.1177/2045893217721928. Epub 2017 Aug 25.
In patients with Eisenmenger syndrome, life expectancy is usually longer than in patients with other forms of pulmonary arterial hypertension (PAH). We conducted a cohort study in which patients were followed over a long period of time in an attempt to identify potential predictors of clinical outcomes. Sixty-seven treatment-naïve patients were enrolled (age range = 12-60 years; median age = 33 years). Baseline demographic, diagnostic, and functional parameters, plasma levels of endothelial dysfunction markers, and treatment-related data were tested for possible correlations with event-free survival. Patients were started on oral PAH drugs at the beginning of follow-up (n = 23), during follow-up (n = 33), or remained untreated (n = 11). The duration of follow-up was 0.54-9.89 years (median = 7.13 years), with an overall survival rate of 82% and an event-free survival rate of 70%. The estimated mean for event-free survival time was 7.71 years (95% confidence interval [CI] = 6.86-8.55 years). Of the 16 variables that were analyzed, the duration of exposure to PAH drugs was identified as an independent protective factor (hazard ratio [HR] = 0.25 for quartiles, 95% CI = 0.14-0.47, P < 0.001). The initial functional class (HR = 3.07; 95% CI = 1.01-9.34; P = 0.048), the severity of right ventricular dysfunction (HR = 2.51 [mild, moderate or severe dysfunction]; 95% CI = 1.22-5.19; P = 0.013) and plasma von Willebrand factor concentration (HR = 1.74 for quartiles; 95% CI = 1.07-2.83; P = 0.026) were identified as risk factors. The length of exposure to oral PAH therapies influences survival favorably in Eisenmenger patients. This may be of interest for communities where access to medications is restricted.
患有艾森曼格综合征的患者的预期寿命通常比患有其他形式肺动脉高压（PAH）的患者更长。我们进行了一项队列研究，对患者进行长期随访，以试图确定临床结局的潜在预测因素。纳入了67例未接受过治疗的患者（年龄范围 = 12 - 60岁；中位年龄 = 33岁）。对基线人口统计学、诊断和功能参数、内皮功能障碍标志物的血浆水平以及治疗相关数据进行测试，以确定其与无事件生存期的可能相关性。患者在随访开始时（n = 23）、随访期间（n = 33）开始口服PAH药物治疗，或未接受治疗（n = 11）。随访时间为0.54 - 9.89年（中位值 = 7.13年），总生存率为82%，无事件生存率为70%。无事件生存时间的估计均值为7.71年（95%置信区间[CI] = 6.86 - 8.55年）。在分析的16个变量中，PAH药物暴露时间被确定为独立的保护因素（四分位数的风险比[HR] = 0.25，95% CI = 0.14 - 0.47，P < 0.001）。初始功能分级（HR = 3.07；95% CI = 1.01 - 9.34；P = 0.048）、右心室功能障碍的严重程度（HR = 2.51[轻度、中度或重度功能障碍]；95% CI = 1.22 - 5.19；P = 0.013）和血浆血管性血友病因子浓度（四分位数的HR = 1.74；95% CI = 1.07 - 2.83；P = 0.026）被确定为危险因素。口服PAH治疗的暴露时间长度对艾森曼格综合征患者的生存有积极影响。这对于药物获取受限的社区可能具有重要意义。
