CAJ | 학술논문

目的探讨儿童特发性肺动脉高压(IPAH)患者急性肺血管扩张试验的指征、方法学.方法 2009年10月到2011年6月收治的WHO心功能Ⅱ～Ⅲ级的IPAH患儿参与了该项研究.在右心导管检查获取了基线血流动力学资料后,从中心静脉泵入腺苷,起始剂量为50 μg/(kg·min),每2分钟递增25 μg/(kg·min),直至达到最大剂量250μg/(kg·min)或达到阳性反应.结果 15例IPAH患儿纳入了该项研究,平均年龄为6.3岁,平均肺动脉压(mPAP)为(67.1±15.9)mm Hg (1 mm Hg =0.133 kPa),肺毛细血管楔压(PCWP)为(9.7±2.9) mm Hg,肺血管阻力指数(PVRI)为(17.9±7.5) Wood U·m2.在急性肺血管扩张试验中,3例mPAP下降幅度超过10 mm Hg,并且绝对值≤40 mm Hg,为阳性反应；另外12例为阴性反应.15例中有5例在试验中出现不良反应,包括胸闷不适(n=1)、体循环低血压(n=3)、心动过缓(n=1).停用腺苷后30～60 s,上述不良反应均迅速消失.结论静脉用腺苷是一种安全、有效的肺血管扩张剂,可作为儿童IPAH患者急性肺血管扩张试验的试验药物.
목적 탐토인동특발성폐동맥고압(IPAH)환자급성폐혈관확장시험적지정、방법학.방법 2009년10월도2011년6월수치적WHO심공능Ⅱ～Ⅲ급적IPAH환인삼여료해항연구.재우심도관검사획취료기선혈류동역학자료후,종중심정맥빙입선감,기시제량위50 μg/(kg·min),매2분종체증25 μg/(kg·min),직지체도최대제량250μg/(kg·min)혹체도양성반응.결과 15례IPAH환인납입료해항연구,평균년령위6.3세,평균폐동맥압(mPAP)위(67.1±15.9)mm Hg (1 mm Hg =0.133 kPa),폐모세혈관설압(PCWP)위(9.7±2.9) mm Hg,폐혈관조력지수(PVRI)위(17.9±7.5) Wood U·m2.재급성폐혈관확장시험중,3례mPAP하강폭도초과10 mm Hg,병차절대치≤40 mm Hg,위양성반응；령외12례위음성반응.15례중유5례재시험중출현불량반응,포괄흉민불괄(n=1)、체순배저혈압(n=3)、심동과완(n=1).정용선감후30～60 s,상술불량반응균신속소실.결론 정맥용선감시일충안전、유효적폐혈관확장제,가작위인동IPAH환자급성폐혈관확장시험적시험약물.
Objective The assessment of pulmonary vascular reactivity plays an important role in the management of idiopathic pulmonary arterial hypertension (IPAH).The aim of this study was to explore the indications and methodology of pulmonary vasodilator testing in children with IPAH.Methods From October 2009 to June 2011,a cohort of pediatric patients with IPAH in WHO functional classes Ⅱ to Ⅲ were enrolled in the study.Right heart catheterization was performed in all patients.After baseline hemodynamics were obtained,adenosine infusions were started at a dose of 50 μg/( kg · min),increased by 25 μg/(kg · min) at 2 min intervals to a maximum of 250 μg/(kg · min) or until a positive acute response.Results A total of 15 patients with IPAH were enrolled in the study.The mean age of the patients was 6.3 yrs.Mean pulmonary artery pressure (mPAP) was (67.1 ± 15.9) mm Hg.Pulmonary capillary wedge pressure (PCWP) was (9.7 ±2.9) mm Hg.Pulmonary vascular resistance index (PVRI)was (17.9 ±7.5) Wood U · m2.Three patients were responders,defined as a fall in mPAP of at least 10 mm Hg to a pressure level of 40 mm Hg or lower.Twelve patients were nonresponders according to the same criteria.Five out of the 15 patients experienced adverse effects,including chest discomfort (n =1 ),systemic hypotension ( n =3 ) and bradycardia ( n =1 ).All side effects abated within 30-60 s of the discontinuation of the adenosine infusion.Conclusion Adenosine is an effective vasodilator in children with IPAH and can be used for safe and rapid assessment of vasodilator reserve in these patients.