Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups
Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups
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DOI：
10.1093/eurheartj/eht072
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发表时间：
2014-03-01
影响因子：
39.3
通讯作者：
Galie, Nazzareno
中科院分区：
文献类型：
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作者：
Manes, Alessandra;Palazzini, Massimiliano;Galie, Nazzareno
目的比较先天性心脏病合并肺动脉高压(PAH-CHD)：艾森门格综合征(ES)、体肺分流术(SPS)、小缺损型(SDS)和先天性心脏病矫正术(CDS)患者的临床、功能、血流动力学特征和近期存活率。一组同期的特发性PAH患者被用于比较。治疗是按照PAH指南进行的，包括联合治疗，使用批准的PAH特异性药物。采用Kaplan-Meier分析评估首次经侵入性血流动力学证实的PAH患者的存活率，并通过LOG-RANK检验进行亚组间比较。192例患者(平均年龄41±17岁，女性61例)中，ES 90例(41+/-16岁)，SP 48例(47+/-18岁)，SD 10例(25±21岁)，CD 44例(36+/-17岁)。ES患者基础肺血管阻力最高，运动能力最低。78%的患者接受了批准的多环芳烃特效药治疗，44人接受了联合治疗。20年后ES、SP和CD的Kaplan-Meier生存估计(95可信区间)分别为87(7793)、86(6096)和36(1272，P=0.0001 vs ES；P=0.004 vs SP)，15年SD为66(1691，P=0.015 vs ES；P=0.016 vs SP)。278例特发性PAH患者的生存率明显低于PAH-CHD亚组。结论不同亚组间的临床、功能、血流动力学及生存期存在差异。尤其是CD和SD患者的存活率最低。在规划PAH-CHD患者的内科或介入治疗策略时，应考虑这些发现。
Aims This study compared the clinical, functional, and haemodynamic characteristics and current era survival of subgroups of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD): Eisenmenger syndrome (ES); PAH-CHD associated with systemic-to-pulmonary shunts (SPs); PAH with small defects (SDs); and PAH after defect correction (CDs).Methods and results Data from consecutive PAH-CHD patients referred to our centre from 1 January 1998 to 31 May 2011 were collected. A contemporary group of idiopathic PAH patients was utilized for comparison. Treatment was per PAH guidelines, including combination therapy, with approved PAH-specific drugs. Survival was assessed with Kaplan-Meier analysis from the first invasive haemodynamic confirmation of PAH and compared across subgroups by log-rank test. Of 192 patients (mean age 41 +/- 17 years; 61 female), 90 had ES (aged 41 +/- 16 years); 48 SP (aged 47 +/- 18 years); 10 SD (aged 25 +/- 21 years); and 44 CD (aged 36 +/- 17 years). Patients with ES had the highest baseline pulmonary vascular resistance and the lowest exercise capacity. Seventy-eight per cent were treated with approved PAH-specific drugs, and 44 were treated with combination therapy. Kaplan-Meier survival estimates (95 confidence interval) at 20 years for ES, SP, and CD were 87 (7793), 86 (6096), and 36 (1272, P = 0.0001 vs. ES; P = 0.004 vs. SP), respectively, and at 15 years for SD was 66 (1691, P = 0.015 vs. ES; P = 0.016 vs. SP). The survival of the 278 patients with idiopathic PAH appeared to be worse when compared with the PAH-CHD subgroups.Conclusion Relevant clinical, functional, haemodynamic, and survival differences were observed among subgroups. In particular, patients with CD and SD had the worst survival. These findings should be considered when planning medical or interventional treatment strategies in PAH-CHD patients.