Epoprostenol treatment in children with severe pulmonary hypertension
Epoprostenol treatment in children with severe pulmonary hypertension
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DOI：
10.1136/hrt.2006.096412
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发表时间：
2007-06-01
期刊：
影响因子：
5.7
通讯作者：
Haworth, Sheila G.
中科院分区：
文献类型：
--
作者：
Lammers, Astrid E.;Hislop, Alison A.;Haworth, Sheila G.
导言：严重的、持续性的肺动脉高压会导致运动能力的进行性降低、右心衰竭和死亡。患者和方法：这项研究包括自1997年11月以来在大奥蒙德街儿童医院(英国伦敦)持续静脉注射依前列烯醇治疗的39名儿童。患者开始治疗时年龄为4个月至17岁(中位数5.4岁)。男女之比为1：1.3。特发性肺动脉高压25例，合并先天性心脏病、结缔组织病、慢性肺病或HIV的肺动脉高压14例。平均肺动脉压(SD)59(17)mm Hg，平均肺血管阻力23.3(11.6)单位×m(2)。患者定期(间隔2-3个月)进行体格检查、心电图、经胸超声心动图和6分钟步行试验。结果：平均随访27(21)个月。死亡7例，移植8例。1、2、3年累计生存率分别为94%、90%和84%。6分钟步行距离平均提高了77米(p<；0.003)。世卫组织职能级别在第一年有所改善(p<；0.001)，并在长达3年的时间里保持改善。体重显著改善，从基线z评分-1.55(1.74)提高到-1.16(1.8)(p<；0.03)。28名儿童接受了额外的口服特效治疗。希克曼线的变化为0.33/病人年。结论：依普前列醇能改善重度肺动脉高压患儿的存活率、WHO功能分级、运动耐量和生存能力。埃普前列烯醇是一种有效和可行的治疗方法，即使在幼儿中也是如此。
Introduction: Severe, sustained pulmonary arterial hypertension leads to a progressive reduction in exercise capacity, right heart failure and death. Use of intravenous epoprostenol has improved survival in adults, but data are limited in children.Patients and methods: This study included all 39 children treated with continuous intravenous epoprostenol since November 1997 at Great Ormond Street Hospital for Children ( London, UK). Patients were aged 4 months to 17 years ( median 5.4 years) at the onset of therapy. The male: female ratio was 1: 1.3. 25 patients had idiopathic pulmonary arterial hypertension and 14 had pulmonary arterial hypertension associated with congenital heart disease, connective tissue disease, chronic lung disease or HIV. All were in WHO functional class III and IV. Mean pulmonary arterial pressure (SD) was 59 ( 17) mmHg and mean pulmonary vascular resistance was 23.3 ( 11.6) unitsxm(2). Patients were assessed regularly ( 2-3 monthly intervals) by physical examination, electrocardiography, transthoracic echocardiography and a 6-min walk test, when practicable.Results: The mean duration of follow-up was 27 ( 21) months. 7 patients died and 8 underwent transplantation. Cumulative survival at 1, 2 and 3 years was 94, 90 and 84%. The 6-min walking distance improved by a mean of 77 m ( p < 0.003). WHO functional class improved during the first year ( p < 0.001) and improvement was maintained for up to 3 years. Weight improved significantly from a baseline z score of -1.55 ( 1.74) to -1.16 (1.8) ( p < 0.03). 28 children had additional oral specific therapy. Hickman line changes were 0.33/patient year.Conclusions: Epoprostenol therapy improved survival, WHO functional class, exercise tolerance and ability to thrive in children with severe pulmonary arterial hypertension. Epoprostenol represents an effective and feasible therapy even in young children.