Mahgoub Linda, Kaddoura Tarek, Kameny A Rebecca, Lopez Ortego Paloma, Vanderlaan Rachel D, Kakadekar Ashok, Dicke Frank, Rebeyka Ivan, Calderone Christopher A, Redington Andrew, Del Cerro Maria Jesus, Fineman Jeff, Adatia Ian
Stollery Children's Hospital, University of Alberta, Edmonton, Canada.
Electrical and Computer Engineering, University of Alberta, Edmonton, Canada.
Pediatr Pulmonol. 2017 Aug;52(8):1063-1070. doi: 10.1002/ppul.23679. Epub 2017 Feb 2.
Pulmonary vein stenosis is emerging as an important clinical problem in ex-premature infants.
We sought to describe the epidemiology of pulmonary vein stenosis affecting ex-premature infants by a multicenter retrospective cohort study of patients from seven children's hospitals diagnosed between 2000-2014.
We identified 39 ex-premature patients (26 males, median gestational age 28 weeks range 22-36 weeks, birth weight 1.1 kg range 433-2645-g) with pulmonary vein stenosis. Median age at diagnosis was 6.5 months (1 month-6 years). Presentation with pulmonary hypertension occurred in 26/39 (67%) and 29/39 (74%) had bronchopulmonary dysplasia, 15 (39%) were born of twin pregnancies with unaffected twin siblings. A median of 5 (range 1-25) echocardiograms was performed prior to diagnosis. The diagnosis was made using echocardiography in 22/39 (56%), by multi-detector contrast computed tomography scan (CT) in 8/39 (21%), cardiac catheterization in 6/39 (15%) patients, magnetic resonance imaging in 3/39 (8%). Freedom from death or re-stenosis was 73% at 1-year, 55% at 2, 5, and 10 years. Factors associated with shorter survival or re-stenosis were stenosis of ≥3 pulmonary veins (P < 0.01), bilateral pulmonary vein stenosis (P < 0.01) small for gestational age (P = 0.05), aged <6 months at diagnosis (P < 0.01).
Pulmonary vein stenosis of ex-premature infants is a complex problem with poor survival, delayed diagnosis, and unsatisfactory treatment. The lack of concordance in twins suggests epigenetic or environmental factors may play a role in the development of pulmonary vein stenosis. In ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia a focused echocardiographic assessment of the pulmonary veins is required with further imaging if the echocardiogram is inconclusive.
肺静脉狭窄正成为早产低体重儿的一个重要临床问题。
我们试图通过一项多中心回顾性队列研究来描述影响早产低体重儿的肺静脉狭窄的流行病学情况，该研究涉及来自7家儿童医院在2000年至2014年间确诊的患者。
我们确定了39例患有肺静脉狭窄的早产低体重儿患者（26例男性，中位胎龄28周，范围22 - 36周，出生体重1.1千克，范围433 - 2645克）。诊断时的中位年龄为6.5个月（1个月至6岁）。26/39（67%）的患者出现肺动脉高压，29/39（74%）患有支气管肺发育不良，15例（39%）为双胎妊娠，其未受影响的同胞为双胞胎。诊断前中位进行了5次（范围1 - 25次）超声心动图检查。22/39（56%）的患者通过超声心动图确诊，8/39（21%）通过多排对比计算机断层扫描（CT）确诊，6/39（15%）通过心导管检查确诊，3/39（8%）通过磁共振成像确诊。1年时无死亡或再狭窄的生存率为73%，2年、5年和10年时为55%。与较短生存期或再狭窄相关的因素包括≥3条肺静脉狭窄（P < 0.01）、双侧肺静脉狭窄（P < 0.01）、小于胎龄儿（P = 0.05）、诊断时年龄<6个月（P < 0.01）。
早产低体重儿的肺静脉狭窄是一个复杂问题，生存率低、诊断延迟且治疗效果不理想。双胞胎之间缺乏一致性表明表观遗传或环境因素可能在肺静脉狭窄的发生中起作用。对于患有肺动脉高压和支气管肺发育不良的早产低体重儿，如果超声心动图结果不明确，需要对肺静脉进行重点超声心动图评估并进一步成像检查。
