Six-minute walking distance and decrease in oxygen saturation during the six-minute walk test in pediatric pulmonary arterial hypertension

Six-minute walking distance and decrease in oxygen saturation during the six-minute walk test in pediatric pulmonary arterial hypertension
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DOI:
10.1016/j.ijcard.2015.08.155
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发表时间:
2016-01-01
影响因子:
3.5
通讯作者:
Berger, Rolf M. F.
Berger, Rolf M. F.
中科院分区:
医学2区
文献类型:
--
作者:
Douwes, Johannes M.;Hegeman, Anneke K.;Berger, Rolf M. F.

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目的:探讨 6 分钟步行测试 (6-MWT) 中获得的 6 分钟步行距离 (6-MWD)、经皮饱和度 (tcSO2) 和心率 (HR) 对小儿肺动脉高压 (PAH) 的预后价值。 方法:这是一项观察性研究,对象为 47 名年龄 >= 7 岁的小儿 PAH 患者,并在荷兰国家小儿 PAH 转诊中心进行诊断和随访。所有患者均进行了全面的 6 分钟步行测试 (6-MWT),测量步行测试之前(“基线”)、期间(“运动”)和 5 分钟(“恢复”)后的 6-MWD 和 tcSO2 和 HR。 结果:6-MWD 以米或性别和年龄校正 z 分数表示,与无移植生存相关,与性别、年龄和是否存在移植无关。分流缺陷。较短的 6-MWD 与较高的 WHO-FC 和较高的 NT-pro-BNP 相关。运动时的绝对 tcSO2 和 6-MWT 期间 tcSO2 的减少与无移植生存相关,与 6-MWD 无关。将 tcSO2 降低与 6-MWD 相结合提供了最强的预后模型。 6-MWD > 352 m(中位 6-MWD)的患者比 6-MWD 较小的患者有更好的结果。 6-MWT 期间 tcSO2 大幅下降(有分流缺陷的患者>19%,无分流缺陷的患者>5%)表明 6-MWD 高于和低于中位 6-MWD 的患者的无移植生存率较差。结论:6-MWD 是儿科 PAH 预后的独立预测因子,反映了疾病严重程度和临床相关的运动耐受性,因此有资格作为治疗目标。 6-MWT 期间 tcSO2 下降的幅度(根据分流的存在进行调整)表明 PAH 儿童预后的另一个危险因素。 (C) 2015 Elsevier Ireland Ltd. 保留所有权利。
Objective: To investigate the prognostic value of the 6-minute walking distance (6-MWD), transcutaneous saturation (tcSO2) and heart rate (HR) obtained during the 6-minute walk test (6-MWT) in pediatric pulmonary arterial hypertension (PAH).Methods: This was an observational study with forty-seven pediatric PAH patients, aged >= 7 years, and diagnosed and followed at the national referral center for pediatric PAH in the Netherlands. All patients performed a comprehensive 6-minute walk test (6-MWT), which measures 6-MWD and tcSO2 and HR before ("baseline"), during ("exercise") and 5 min after ("recovery") the walk test.Results: The 6-MWD expressed either in meters or in sex-and age-corrected z-scores, was associated with transplant-free survival, independently from sex, age, and the presence of a shunt-defect. Shorter 6-MWD correlated with higher WHO-FC and increased NT-pro-BNP. Absolute tcSO2 at exercise and tcSO2-decrease during 6-MWT were associated with transplant-free survival, independent from 6-MWD. Combining tcSO2-decrease with 6-MWD provided the strongest prognostic model. Patients with 6-MWD > 352 m (the median 6-MWD) had a better outcome than those with smaller 6-MWD. A large tcSO2-decrease during 6-MWT (>19% for patients with and >5% for patients without a shunt defect) identified patients with worse transplant-free survival both in patients with a 6-MWD above and below the median 6-MWD.Conclusions: The 6-MWD is an independent predictor of prognosis in pediatric PAH, that reflects disease severity and clinically relevant exercise-tolerance and therefore qualifies as a treatment goal. The magnitude of tcSO2-decrease during 6-MWT, adjusted for the presence of a shunt, indicates an additional risk factor for prognosis in children with PAH. (C) 2015 Elsevier Ireland Ltd. All rights reserved.