Drossner David M, Kim Dennis W, Maher Kevin O, Mahle William T
Children's Healthcare of Atlanta, Emory University School of Medicine, 1405 Clifton Rd, NE, Atlanta, GA 30322-1062, USA.
Pediatrics. 2008 Sep;122(3):e656-61. doi: 10.1542/peds.2008-0075.
Pulmonary vein stenosis is a rare, although often lethal, anomaly. Risk factors for the diagnosis of pulmonary vein stenosis are poorly characterized. In this study we sought to identify factors associated with pulmonary vein stenosis, paying particular attention to preterm birth.
By review of the cardiac database we identified all of the subjects with pulmonary vein stenosis over a 10-year period at our institution. Those children with anomalous pulmonary venous connection were not included. Patient-related variables were analyzed for their association with pulmonary vein stenosis. Pulmonary vein stenosis was diagnosed by spectral Doppler interrogation of the pulmonary veins (continuous, turbulent flow with calculated mean gradient > 5 mm Hg) and confirmed by cardiac catheterization in nearly all of the cases.
Twenty-six patients with pulmonary vein stenosis were identified. The median age at diagnosis was 7.4 months; range: 1 day to 35 months. Congenital heart defects were present in the majority of subjects. Associated genetic syndromes were present in 8 subjects (31%). The 2-year survival rate from diagnosis was 43%. The majority of subjects (16 [61%]) were preterm. Gestational ages ranged from 24.2 to 41.0 weeks, and birth weights ranged from 460 to 4445 g. Preterm birth was strongly associated with the diagnosis of pulmonary vein stenosis, odds ratio 10.2 (95% CI 4.7-22.6), p < .001. Eleven (42%) of the 26 subjects were treated for bronchopulmonary dysplasia before being diagnosed with pulmonary vein stenosis.
Prematurity is associated with the diagnosis of pulmonary vein stenosis. It is interesting to note that many of these patients also have intracardiac shunt lesions, which may act in concert with preterm endothelium to produce pulmonary vein stenosis.
肺静脉狭窄是一种罕见但往往致命的畸形。肺静脉狭窄诊断的危险因素特征尚不明确。在本研究中，我们试图确定与肺静脉狭窄相关的因素，尤其关注早产情况。
通过查阅心脏数据库，我们确定了本机构10年间所有患有肺静脉狭窄的受试者。那些有异常肺静脉连接的儿童未被纳入。分析与患者相关的变量与肺静脉狭窄的关联。通过对肺静脉进行频谱多普勒检查（连续性、湍流，计算平均梯度>5 mmHg）诊断肺静脉狭窄，几乎所有病例均通过心导管检查得以证实。
共确定26例肺静脉狭窄患者。诊断时的中位年龄为7.4个月；范围：1天至35个月。大多数受试者存在先天性心脏缺陷。8名受试者（31%）存在相关的遗传综合征。诊断后2年生存率为43%。大多数受试者（16例[61%]）为早产儿。胎龄范围为24.2至41.0周，出生体重范围为460至4445 g。早产与肺静脉狭窄的诊断密切相关，比值比为10.2（95%可信区间4.7 - 22.6），p <.001。26名受试者中有11名（42%）在被诊断为肺静脉狭窄之前接受过支气管肺发育不良的治疗。
早产与肺静脉狭窄的诊断相关。值得注意的是，这些患者中有许多还存在心内分流病变，它们可能与早产的内皮共同作用导致肺静脉狭窄。
