Outcomes in children with idiopathic pulmonary arterial hypertension
Outcomes in children with idiopathic pulmonary arterial hypertension
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DOI：
10.1161/01.cir.0000138104.83366.e9
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发表时间：
2004-08-10
期刊：
影响因子：
37.8
通讯作者：
Barst, RJ
中科院分区：
文献类型：
--
作者：
Yung, D;Widlitz, AC;Barst, RJ
背景-儿童特发性肺动脉高压的治疗包括钙通道阻滞剂（CCB）治疗急性反应者和血管扩张剂试验，慢性依前列醇治疗无反应者。我们试图确定参数与生存率和治疗success.Methods和Results-A先前确定的队列77名儿童诊断1982年和1995年之间的特发性肺动脉高压随访至2002年。对于接受CCB治疗的急性反应者（n=31），1年、5年和10年生存率分别为97%、97%和81%;治疗成功率分别为84%、68%和47%。所有接受依前列醇治疗的儿童（n=35）在1、5和10年的生存率分别为94%、81%和61%;治疗成功率分别为83%、57%和37%。由于1995年之前依前列醇的可用性不一致，我们定义了一个“最近医学时代”子集，从总共77例推荐使用依前列醇但无法使用的患者队列中排除儿童。在最近的医学时代（n=44），1年、5年和10年的生存率分别为97%、97%和78%;治疗成功率分别为93%、86%和60%。当急性应答者变为无应答者时，CCB的治疗成功率显著降低。诊断时的年龄预测治疗成功率在最近的医疗eager. Conclusions生存的儿童特发性肺动脉高压已显着改善与CCB和依前列醇。急性应答儿童接受CCB治疗;如果变为无应答者，则接受依前列醇治疗。所有儿童5年后生存率和治疗成功率的下降支持了在治疗失败前进行移植评估的作用。
Background-Treatment for idiopathic pulmonary arterial hypertension in children includes calcium channel blockade (CCB) for acute responders with vasodilator testing and chronic epoprostenol for nonresponders. We sought to determine parameters associated with survival and treatment success.Methods and Results-A previously identified cohort of 77 children diagnosed between 1982 and 1995 with idiopathic pulmonary arterial hypertension was followed up through 2002. For acute responders treated with CCB (n=31), survival at 1, 5, and 10 years was 97%, 97%, and 81%, respectively; treatment success was 84%, 68%, and 47%, respectively. Survival for all children treated with epoprostenol (n=35) at 1, 5, and 10 years was 94%, 81%, and 61%, respectively; treatment success was 83%, 57%, and 37%, respectively. Because of the inconsistent availability of epoprostenol before 1995, we defined a "recent medical era" subset by excluding children from the total 77 patient cohort for whom epoprostenol was recommended but was unavailable. Survival in the recent medical era (n=44) at 1, 5, and 10 years was 97%, 97%, and 78%; treatment success was 93%, 86%, and 60%, respectively. Treatment success on CCB decreased significantly when acute responders became nonresponders. Age at diagnosis predicted treatment success in the recent medical era.Conclusions-Survival for children with idiopathic pulmonary arterial hypertension has significantly improved with CCB and epoprostenol. Children who are acute responders are treated with CCB; they are treated with epoprostenol if they become nonresponders. The decrease in survival and in treatment success after 5 years in all children supports the role for transplant evaluation before treatment failure.