Sandoval J, Bauerle O, Gomez A, Palomar A, Martínez Guerra M L, Furuya M E
Cardiopulmonary Department, Instituto Nacional de Cardiología Ignacio Chávez, México DF, México.
J Am Coll Cardiol. 1995 Feb;25(2):466-74. doi: 10.1016/0735-1097(94)00391-3.
This study characterized mortality in a group of Mexican children (n = 18, mean [+/- SD] age 9.9 +/- 3 years) with primary pulmonary hypertension and investigated the factors associated with their survival.
Primary pulmonary hypertension is a progressive, fatal disease of unknown cause. Establishing the diagnosis earlier in life may influence prognosis.
A dynamic cohort of children with primary pulmonary hypertension were enrolled between December 1977 and May 1991 and followed up through September 1992. Measurements included hemodynamic and pulmonary function variables in addition to demographic data, medical history and response to vasodilator treatment. We also compared the survival estimates of these children with those of our adult patients with primary pulmonary hypertension (n = 42, mean age 27.9 +/- 8.5 years).
Baseline mean (+/- SD) pulmonary artery pressure was similar in children and adults (66 +/- 15 vs. 65 +/- 18 mm Hg, p = NS), but a higher cardiac index resulted in a lower mean pulmonary vascular resistance index in children (18 +/- 7 vs. 26 +/- 12 U/m2, p < 0.01). The proportion of patients who had a positive hemodynamic response to vasodilator treatment was higher in children than in adults (41% vs. 25%). Estimated median survival in children was 4.12 years (95% confidence interval [CI] 0.75 to 8.66) and 3.12 years in adults (95% CI 0.5 to 13.25, chi-square log-rank 0.81, p = NS). Elevated right atrial pressure (rate ratio 10.2) and decreased stroke volume index (rate ratio 32.9) were the only significant predictors of mortality (Cox proportional hazards model).
Children with primary pulmonary hypertension have a poor survival expectancy, which does not appear to differ from that in adults with primary pulmonary hypertension. Mortality in childhood primary pulmonary hypertension is also associated with variables that assess right ventricular dysfunction.
本研究对一组患有原发性肺动脉高压的墨西哥儿童（n = 18，平均[±标准差]年龄9.9±3岁）的死亡率进行了特征描述，并调查了与他们生存相关的因素。
原发性肺动脉高压是一种病因不明的进行性致命疾病。在生命早期确诊可能会影响预后。
1977年12月至1991年5月纳入了一组原发性肺动脉高压儿童动态队列，并随访至1992年9月。测量包括血流动力学和肺功能变量，以及人口统计学数据、病史和对血管扩张剂治疗的反应。我们还将这些儿童的生存估计值与我们成年原发性肺动脉高压患者（n = 42，平均年龄27.9±8.5岁）的生存估计值进行了比较。
儿童和成人的基线平均（±标准差）肺动脉压相似（66±15对65±18 mmHg，p =无显著性差异），但较高的心指数导致儿童的平均肺血管阻力指数较低（18±7对26±12 U/m2，p < 0.01）。对血管扩张剂治疗有血流动力学阳性反应的患者比例儿童高于成人（41%对25%）。儿童的估计中位生存期为4.12年（95%置信区间[CI] 0.75至8.66），成人为3.12年（95%CI 0.5至13.25，卡方对数秩检验0.81，p =无显著性差异）。右心房压力升高（率比10.2）和每搏量指数降低（率比32.9）是死亡率的唯一显著预测因素（Cox比例风险模型）。
原发性肺动脉高压儿童的生存预期较差，这似乎与成年原发性肺动脉高压患者无异。儿童原发性肺动脉高压的死亡率也与评估右心室功能障碍的变量相关。
