Hutson Shandee, Baerg Joanne, Deming Douglas, St Peter Shawn D, Hopper Andrew, Goff Donna A
Department of Neonatology, Loma Linda University Children's Hospital, Loma Linda, CA, USA.
Neonatology. 2017;112(3):281-286. doi: 10.1159/000477535. Epub 2017 Jul 14.
Omphalocele is one of the most common abdominal wall defects. Many newborn infants born with omphalocele present with significant respiratory distress at birth, requiring mechanical ventilatory support, and have clinical evidence of pulmonary hypertension. Little information exists on the prevalence of and risk factors associated with pulmonary hypertension in this cohort of infants.
To describe the prevalence of and risk factors associated with pulmonary hypertension among infants with omphalocele.
This is a multicenter retrospective chart review of demographic data and clinical characteristics of infants with omphalocele admitted to the neonatal intensive care units of Loma Linda University Children's Hospital and Children's Mercy Hospital between 1994 and 2011. Echocardiogram images were reviewed for pulmonary hypertension, and statistical analyses were performed to identify risk factors associated with the presence of pulmonary hypertension.
Pulmonary hypertension was diagnosed in 32/56 (57%) infants with omphalocele. Compared to infants without pulmonary hypertension, infants with pulmonary hypertension were more likely to have a liver-containing defect (16/32 [50%] vs. 5/24 [21%], p = 0.03), require intubation at birth (18/32 [56%] vs. 6/24 [17%], p = 0.03), and die during initial hospitalization (12/32 [38%] vs. 2/24 [8%], p = 0.01).
The majority of infants with omphalocele have evidence of pulmonary hypertension which is associated with increased mortality. Echocardiograms to screen for pulmonary hypertension should be obtained at ≥2 days of life in infants with omphalocele, especially in those with liver within the omphalocele sac and/or in those infants who require intubation at birth to screen for pulmonary hypertension.
脐膨出是最常见的腹壁缺陷之一。许多患有脐膨出的新生儿在出生时即出现严重呼吸窘迫，需要机械通气支持，并有肺动脉高压的临床证据。关于这组婴儿中肺动脉高压的患病率及相关危险因素的信息较少。
描述脐膨出婴儿中肺动脉高压的患病率及相关危险因素。
这是一项多中心回顾性图表研究，回顾了1994年至2011年间入住洛马林达大学儿童医院和儿童慈善医院新生儿重症监护病房的脐膨出婴儿的人口统计学数据和临床特征。对超声心动图图像进行肺动脉高压评估，并进行统计分析以确定与肺动脉高压存在相关的危险因素。
56例脐膨出婴儿中有32例（57%）被诊断为肺动脉高压。与无肺动脉高压的婴儿相比，有肺动脉高压的婴儿更可能有包含肝脏的缺损（16/32 [50%] 对5/24 [21%]，p = 0.03），出生时需要插管（18/32 [56%] 对6/24 [17%]，p = 0.03），以及在初次住院期间死亡（12/32 [38%] 对2/24 [8%]，p = 0.01）。
大多数脐膨出婴儿有肺动脉高压证据，这与死亡率增加相关。对于脐膨出婴儿，应在出生≥2天时进行超声心动图检查以筛查肺动脉高压，尤其是那些脐膨出囊内有肝脏和/或出生时需要插管的婴儿，以筛查肺动脉高压。
