Fu Li-jun, Zhou Ai-qing, Guo Ying, Zhao Peng-jun, Huang Mei-rong, Li Fen
Department of Cardiology, Shanghai Children's Medical Center, Medical College of Shanghai Jiao Tong University, Shanghai 200127, China.
Zhonghua Er Ke Za Zhi. 2011 Dec;49(12):886-9.
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.
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.
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·m(2). 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.
Adenosine is an effective vasodilator in children with IPAH and can be used for safe and rapid assessment of vasodilator reserve in these patients.
评估肺血管反应性在特发性肺动脉高压(IPAH)的管理中起着重要作用。本研究的目的是探讨IPAH患儿肺血管扩张试验的适应证和方法。
2009年10月至2011年6月,将一组世界卫生组织功能分级为II至III级的IPAH儿科患者纳入研究。所有患者均进行了右心导管检查。在获得基线血流动力学数据后,开始以50μg/(kg·min)的剂量输注腺苷,每隔2分钟以25μg/(kg·min)的幅度增加,最大剂量为250μg/(kg·min),或直至出现阳性急性反应。
共有15例IPAH患者纳入研究。患者的平均年龄为6.3岁。平均肺动脉压(mPAP)为(67.1±15.9)mmHg。肺毛细血管楔压(PCWP)为(9.7±2.9)mmHg。肺血管阻力指数(PVRI)为(17.9±7.5)Wood U·m²。3例患者为反应者,定义为mPAP至少下降10mmHg至40mmHg或更低的压力水平。根据相同标准,12例患者为无反应者。15例患者中有5例出现不良反应,包括胸部不适(n = 1)、全身性低血压(n = 3)和心动过缓(n = 1)。所有副作用在停止输注腺苷后30 - 60秒内消退。
腺苷是IPAH患儿有效的血管扩张剂,可用于安全、快速评估这些患者的血管扩张储备。