Houde C, Bohn D J, Freedom R M, Rabinovitch M
Department of Paediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
Br Heart J. 1993 Nov;70(5):461-8. doi: 10.1136/hrt.70.5.461.
To describe the demographic and haemodynamic variables of children presenting with primary pulmonary hypertension or pulmonary hypertension appearing or persisting after surgical correction of congenital heart defects and to assess the acute effect of vasodilator drugs on their pulmonary vascular bed.
Retrospective review.
Paediatric cardiology department and intensive care unit of a large tertiary centre.
Fourteen consecutive patients presenting with primary pulmonary hypertension (group 1) or pulmonary hypertension persisting or appearing late after complete surgical repair (group 2).
Baseline haemodynamic measurements were taken in room air at rest and repeated in 100% oxygen. With constant monitoring of heart rate and pulmonary and systemic arterial pressures, patients were given serial intravenous, sublingual, or oral incremental doses of vasodilators (mean 4.1 trials per patient). The maximum effect of the drug was charted.
A positive response to acute vasodilator tests was defined as a decrease in mean pulmonary or mean systemic arterial pressure > 15% with the mean pulmonary artery pressure not reaching the systemic level and either no change or an increase in mean systemic arterial pressure. Haemodynamic variables between groups (1 v 2, responders v non-responders, patients experiencing or not experiencing adverse effects to vasodilators) were compared by a two tailed unpaired Student's t test, and their survival curves were compared by the log rank statistic.
Groups are small and definitive conclusions are difficult to draw, but the baseline haemodynamic assessments were not significantly different between group 1 and 2 or between responders and non-responders to vasodilators. Patients experiencing adverse effects had a higher pulmonary vascular resistance (p = 0.04) and wedge pressure (p = 0.02) than those without adverse effects. Of the vasodilators used, tolazoline, hydralazine, salbutamol, phentolamine, and phenoxybenzamine were ineffective. A positive response was seen in five of 13 patients given oxygen, in one of eight given prostacyclin, four of 12 given nifedipine, four of eight given diltiazem, one of six given captopril, and two of seven given glyceryl trinitrate. Estimates of survival of the population with primary pulmonary hypertension were 37% at one year and 12% at 2.5 years. Survival was significantly shorter in the non-responders than in the responders (p = 0.005).
Children with primary pulmonary hypertension present to the cardiologist at a young age (five of eight were younger than 7 years) but with advanced pulmonary vascular disease and have a poor prognosis. 64% of group 1 and group 2 patients had a positive response to acute treatment with at least one vasodilator. Calcium channel blockers were the most effective agents. There was a positive response to drugs despite a negative response to acute treatment with oxygen. The survival of non-responders was shorter than that of the responders.
描述原发性肺动脉高压患儿或先天性心脏病手术矫正后出现或持续存在肺动脉高压患儿的人口统计学和血流动力学变量，并评估血管扩张剂对其肺血管床的急性作用。
回顾性研究。
一家大型三级中心的儿科心脏病科和重症监护病房。
连续14例原发性肺动脉高压患儿（第1组）或完全手术修复后持续存在或晚期出现肺动脉高压的患儿（第2组）。
在静息室内空气中进行基线血流动力学测量，并在吸入100%氧气时重复测量。在持续监测心率、肺和体循环动脉压的情况下，给患者静脉、舌下或口服递增剂量的血管扩张剂（每位患者平均4.1次试验）。记录药物的最大效应。
急性血管扩张剂试验的阳性反应定义为平均肺动脉或平均体循环动脉压降低>15%，平均肺动脉压未达到体循环水平，且平均体循环动脉压无变化或升高。通过双尾非配对学生t检验比较组间（第1组与第2组、反应者与无反应者、对血管扩张剂有不良反应或无不良反应的患者）的血流动力学变量，并通过对数秩统计量比较其生存曲线。
样本量较小，难以得出明确结论，但第1组和第2组之间或血管扩张剂反应者与无反应者之间的基线血流动力学评估无显著差异。出现不良反应的患者比未出现不良反应的患者具有更高的肺血管阻力（p = 0.04）和楔压（p = 0.02）。所用血管扩张剂中，妥拉唑啉、肼屈嗪、沙丁胺醇、酚妥拉明和酚苄明无效。在13例吸氧患者中有5例、8例接受前列环素治疗的患者中有1例、12例接受硝苯地平治疗的患者中有4例、8例接受地尔硫卓治疗的患者中有4例、6例接受卡托普利治疗的患者中有1例以及7例接受硝酸甘油治疗的患者中有两例出现阳性反应。原发性肺动脉高压患者群体的1年生存率估计为37%，2.5年生存率为12%。无反应者的生存期明显短于反应者（p = 0.005）。
原发性肺动脉高压患儿就诊时年龄较小（8例中有5例小于7岁），但肺血管疾病已较严重，预后较差。第1组和第2组中64%的患者对至少一种血管扩张剂的急性治疗有阳性反应。钙通道阻滞剂是最有效的药物。尽管对吸氧急性治疗无反应，但对药物有阳性反应。无反应者的生存期短于反应者。
