Pulmonary artery hypertension in formerly premature infants with bronchopulmonary dysplasia: Clinical features and outcomes in the surfactant era
Pulmonary artery hypertension in formerly premature infants with bronchopulmonary dysplasia: Clinical features and outcomes in the surfactant era
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DOI：
10.1542/peds.2007-0971
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发表时间：
2007-12-01
期刊：
影响因子：
8
通讯作者：
Mullen, Mary P.
中科院分区：
文献类型：
--
作者：
Khemani, Ekta;McElhinney, Doff B.;Mullen, Mary P.
背景尽管支气管肺发育不良早产儿肺血管结构和功能异常可能使婴儿易患肺动脉高压，但在肺表面活性物质时代，支气管肺发育不良相关肺动脉高压的特征和结局知之甚少。我们研究了1998年至2006年期间42名患有支气管肺发育不良的早产儿（< 32周），他们在出生后≥ 2个月被诊断为肺动脉高压，中位年龄为4.8个月。通过超声心动图对所有患者的肺动脉高压进行分级; 13例患者还接受了心脏导管插入术。42例患者中有18例（43%）患有重度肺动脉高压（系统性或系统上性右心室压）。在13名接受导管插入术的患者中，平均肺动脉压为43 +/- 8 mm Hg，肺血管阻力指数为9.9 +/- 2.8 Wood单位。在12名患者中，100%氧气和80 ppm吸入一氧化氮改善了肺动脉压和肺血管阻力，但仍然升高。肺血管阻力指数在100%氧气中降至7.9 +/- 3.8 Wood单位，在添加一氧化氮后降至6.4 +/- 3.1 Wood单位。16名患者（38%）在随访期间死亡。诊断肺动脉高压后6个月的估计生存率为64% +/- 8%，2年的估计生存率为53% +/- 11%。在多变量分析中，重度肺动脉高压和小于胎龄儿出生体重与较差的存活率相关。在26名幸存者中（中位随访时间：9.8个月），24名患者（89%）的肺动脉高压相对于其最严重水平有所改善。患有支气管肺发育不良和严重肺动脉高压的早产儿死亡风险很高，特别是在诊断肺动脉高压后的前6个月内。
BACKGROUND. Although abnormal pulmonary vascular structure and function in preterm infants with bronchopulmonary dysplasia may predispose infants to pulmonary artery hypertension, little is known about the characteristics and outcomes of bronchopulmonary dysplasia-associated pulmonary artery hypertension in the surfactant era.METHODS. We studied 42 premature infants ( < 32 weeks of gestation) with bronchopulmonary dysplasia who were diagnosed as having pulmonary artery hypertension >= 2 months after birth, between 1998 and 2006, at a median age of 4.8 months. Pulmonary artery hypertension was graded through echocardiography for all patients; 13 patients also underwent cardiac catheterization.RESULTS. Eighteen ( 43%) of 42 patients had severe pulmonary artery hypertension ( systemic or suprasystemic right ventricular pressure). Among 13 patients who underwent catheterization, the mean pulmonary artery pressure was 43 +/- 8 mm Hg and the pulmonary vascular resistance index was 9.9 +/- 2.8 Wood units. In 12 patients, pulmonary artery pressure and pulmonary vascular resistance improved with 100% oxygen and 80 ppm inhaled nitric oxide but remained elevated. The pulmonary vascular resistance index decreased to 7.9 +/- 3.8 Wood units in 100% oxygen and to 6.4 +/- 3.1 Wood units with the addition of nitric oxide. Sixteen patients ( 38%) died during the follow-up period. Estimated survival rates were 64% +/- 8% at 6 months and 53% +/- 11% at 2 years after diagnosis of pulmonary artery hypertension. In multivariate analyses, severe pulmonary artery hypertension and small birth weight for gestational age were associated with worse survival rates. Among 26 survivors ( median follow-up period: 9.8 months), pulmonary artery hypertension was improved, relative to its most severe level, in 24 patients ( 89%).CONCLUSION. Premature infants with bronchopulmonary dysplasia and severe pulmonary artery hypertension are at high risk of death, particularly during the first 6 months after diagnosis of pulmonary artery hypertension.