Columbia University Medical Center, New York, New York, USA.
Am J Cardiol. 2011 May 1;107(9):1381-5. doi: 10.1016/j.amjcard.2010.12.051. Epub 2011 Mar 2.
Eisenmenger syndrome (ES) is a known complication of congenital heart disease associated with unrepaired systemic to pulmonary shunts. Evidence for use of targeted pulmonary arterial hypertension therapy in ES is limited. The early experience using ambrisentan was evaluated in a cohort of consecutive patients with ES who initiated ambrisentan at Columbia University's Pulmonary Hypertension Center from January 1, 2007, to August 1, 2008. Effects of ambrisentan on rest and exercise systemic arterial oxygen saturation (S(a)O(2)), exercise capacity, functional status, hemoglobin levels, and hemodynamics were evaluated and compared using paired Student's t tests. Seventeen patients were evaluated at short-term (mean 163 ± 57 days) and longer term (mean 2.5 ± 0.5 years) follow-up. At short-term follow-up, there was an improvement in exercise capacity (6-minute walking distance 389 ± 74 vs 417 ± 77 m, p=0.03, n=11) and maintenance of rest S(a)O(2) (89 ± 7% vs 89% ± 6%, p=0.75, n=15), exercise S(a)O(2) (75 ± 15% vs 77% ± 15%, p=0.33, n=11), functional class (improvement in 2 patients, no change in 13), and hemoglobin (16.5 ± 2.8 vs 15.8 ± 1.8 g/dl, p=0.11, n=14). At longer term follow-up compared to baseline and short-term follow-up, there was stability of exercise capacity, S(a)O(2), functional class, and hemoglobin. In conclusion, in this single-center cohort of patients with ES, ambrisentan was safe and was associated with increasing exercise capacity at short-term follow-up, with patients maintaining S(a)O(2), functional class, and hemoglobin, and with no significant evidence of clinical deterioration at longer term follow-up. Additional studies are required to further assess the efficacy of ambrisentan in patients with ES.
艾森曼格综合征（Eisenmenger syndrome，ES）是一种已知的先天性心脏病并发症，与未修复的体肺分流有关。针对 ES 采用靶向肺动脉高压治疗的证据有限。我们评估了 2007 年 1 月 1 日至 2008 年 8 月 1 日期间在哥伦比亚大学肺动脉高压中心接受安立生坦治疗的连续 ES 患者的早期经验。使用配对学生 t 检验评估安立生坦对静息和运动时全身动脉血氧饱和度（S(a)O(2)）、运动能力、功能状态、血红蛋白水平和血液动力学的影响，并进行比较。对 17 例患者进行了短期（平均 163 ± 57 天）和长期（平均 2.5 ± 0.5 年）随访。短期随访时，运动能力（6 分钟步行距离 389 ± 74 与 417 ± 77 m，p=0.03，n=11）和静息时 S(a)O(2)（89 ± 7% 与 89% ± 6%，p=0.75，n=15）、运动时 S(a)O(2)（75 ± 15% 与 77% ± 15%，p=0.33，n=11）、功能分级（2 例改善，13 例无变化）和血红蛋白（16.5 ± 2.8 与 15.8 ± 1.8 g/dl，p=0.11，n=14）均有改善。与基线和短期随访相比，长期随访时运动能力、S(a)O(2)、功能分级和血红蛋白均稳定。总之，在这项 ES 患者的单中心队列研究中，安立生坦安全，短期随访时运动能力增加，S(a)O(2)、功能分级和血红蛋白保持稳定，长期随访时无临床恶化的证据。需要进一步的研究来进一步评估安立生坦在 ES 患者中的疗效。
