Children with pulmonary arterial hypertension and prostanoid therapy: long-term hemodynamics.
Children with pulmonary arterial hypertension and prostanoid therapy: long-term hemodynamics.
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DOI：
10.1016/j.healun.2013.01.1055
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发表时间：
2013-05
期刊：
影响因子：
--
通讯作者：
Feinstein JA
中科院分区：
文献类型：
--
作者：
Siehr SL;Ivy DD;Miller-Reed K;Ogawa M;Rosenthal DN;Feinstein JA
重度肺动脉高压（PAH）的儿科患者接受静脉依前列醇或静脉或皮下曲前列尼尔治疗。关于依前列醇、曲前列尼尔的纵向血流动力学和结局以及从依前列醇转换为曲前列尼尔的情况知之甚少。这是一项回顾性研究，纳入了1992年至2010年在2家中心接受依前列醇或曲前列尼尔治疗的77例儿科患者（47例特发性PAH，24例先天性心脏病-PAH）。结局定义为存活vs死亡/移植。基线时的平均年龄为7.7 ± 5.2岁，随访时间为4.3 ± 3.4年。37例患者接受依前列醇治疗，20例患者接受曲前列尼尔治疗，20例患者从依前列醇转换为曲前列尼尔治疗。基线、1、2、3和4年时，依前列醇的平均肺-体血管阻力比（Rp/Rs）分别为1.0 ± 0.4、0.8 ± 0.4、0.8 ± 0.4、1.0 ± 0.4和1.2 ± 0.4。对于曲前列环素，在基线、1年、2年和3 - 4年时，Rp/Rs分别为0.9 ± 0.3、0.7 ± 0.3、0.5 ± 0.2（p < 0.01 vs基线）和1.1 ± 0.2。平均肺动脉压和肺血管阻力指数变化相似。依前列醇转换为曲前列尼尔后1年的Rp/Rs从0.6增加至0.8（n = 7）。除非另有说明，否则变化不具有统计学显著性。8例患者在基线2年内死亡或接受移植;与队列的其他患者相比，该组的平均基线Rp/Rs、右心房压和肺血管阻力指数显著更差。39名患者仍在使用前列腺素类药物，17名患者停用，16名患者死亡，5名患者接受了心肺移植。Kaplan-Meier 5年无移植生存率为70%（95%置信区间，56%-80%）。在1 - 2年时，两种治疗的Rp/Rs均有所改善，但未持续。5年无移植生存率优于类似的成人研究。
Pediatric patients with severe pulmonary arterial hypertension (PAH) are treated with intravenous epoprostenol or intravenous or subcutaneous treprostinil. Little is known about longitudinal hemodynamics and outcomes of epoprostenol, treprostinil, and transitions from epoprostenol to treprostinil. This was retrospective study of 77 pediatric patients (47 idiopathic PAH, 24 congenital heart disease-PAH) receiving epoprostenol or treprostinil from 1992 to 2010 at 2 centers. Outcomes were defined as living vs dead/transplant. Mean age at baseline was 7.7 ± 5.2 years, with follow-up of 4.3 ± 3.4 years. Thirty-seven patients were treated with epoprostenol, 20 with treprostinil, and 20 were transitioned from epoprostenol to treprostinil. Mean pulmonary-to-systemic vascular resistance ratio (Rp/Rs) for epoprostenol was 1.0 ± 0.4, 0.8 ± 0.4, 0.8 ± 0.4, 1.0 ± 0.4, and 1.2 ± 0.4, respectively, at baseline, 1, 2, 3, and 4 years. For treprostinil, Rp/Rs was 0.9 ± 0.3, 0.7 ± 0.3, 0.5 ± 0.2, (p < 0.01 vs baseline), and 1.1 ± 0.2, respectively, at baseline, 1, 2, and 3 to 4 years, respectively. There were similar changes in mean pulmonary artery pressure and pulmonary vascular resistance index. The Rp/Rs 1 year after epoprostenol to treprostinil transition increased from 0.6 to 0.8 (n = 7). Changes not statistically significant unless noted. Eight patients died or received a transplant within 2 years of baseline; compared with the rest of the cohort, mean baseline Rp/Rs, right atrial pressure, and pulmonary vascular resistance index were significantly worse in this group. Thirty-nine patients remain on prostanoids, 17 are off, 16 died, and 5 received heart-lung transplant. Kaplan-Meier 5-year transplant-free survival was 70% (95% confidence interval, 56%-80%). There was improvement in Rp/Rs on both therapies at 1 to 2 years that was not sustained. The 5-year transplant-free survival was better than in similar adult studies.
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影响因子：
37.8
作者：
Rosenzweig, EB;Kerstein, D;Barst, RJ
通讯作者：
Barst, RJ
影响因子：
2.8
作者：
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5.7
作者：
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影响因子：
37.8
作者：
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通讯作者：
Fishman, AP