Dal Col Alexis K, Bhombal Shazia, Tacy Theresa A, Hintz Susan R, Feinstein Jeffrey, Altit Gabriel
Division of Cardiology, Department of Pediatrics, Stanford University School of Medicine, Lucile Packard Children's Hospital, Stanford, California.
Division of Neonatal and Developmental Medicine, Department of Pediatrics, Stanford University School of Medicine, Lucile Packard Children's Hospital, Stanford, California.
Am J Perinatol. 2021 Aug;38(S 01):e109-e115. doi: 10.1055/s-0040-1708048. Epub 2020 Mar 20.
Pulmonary hypertension (PH) has been described in the neonatal omphalocele population. This study was aimed to describe cardiac function and PH severity using echocardiography in newborns with giant omphalocele (GO) and with non-GO and determine if right ventricular (RV) dysfunction is associated with mortality.
Retrospective, single-center analysis of first echocardiography among neonatal omphalocele patients born between 2004 and 2017 was conducted. Multivariate logistic and univariate Cox's regression was constructed to measure hazard ratio (HR) for death outcome.
There were 32 newborns, of whom 18 were GO and 7 died. GO had increased systolic pulmonary arterial to systolic systemic blood pressure ratio (97% [isosystemic] vs. 73% [three-fourths systemic] = 0.03). RV performance parameters (tricuspid annular plane excursion, HR = 0.40; fractional area change, HR = 0.90; and RV peak global longitudinal strain, HR = 1.39) were associated with mortality. These RV performance parameters remained associated in a multiple logistic regression accounting for gestational age and GO status. The overall population had abnormal eccentricity index and pulmonary artery acceleration time to RV ejection time ratio, two markers of PH.
Patients with omphalocele have increased pulmonary pressure, with GO being worse than non-GO. RV dysfunction at initial echocardiography was significantly associated with mortality.
新生儿脐膨出人群中已出现肺动脉高压（PH）的描述。本研究旨在通过超声心动图描述巨大脐膨出（GO）和非巨大脐膨出新生儿的心脏功能和PH严重程度，并确定右心室（RV）功能障碍是否与死亡率相关。
对2004年至2017年间出生的新生儿脐膨出患者的首次超声心动图进行回顾性单中心分析。构建多变量逻辑回归和单变量Cox回归以测量死亡结局的风险比（HR）。
共有32例新生儿，其中18例为GO，7例死亡。GO的收缩期肺动脉与收缩期体循环血压比值升高（97%[等体循环]对73%[四分之三体循环]，P=0.03）。RV性能参数（三尖瓣环平面偏移，HR=0.40；面积变化分数，HR=0.90；以及RV峰值整体纵向应变，HR=1.39）与死亡率相关。在考虑胎龄和GO状态的多变量逻辑回归中，这些RV性能参数仍然相关。总体人群的偏心率指数和肺动脉加速时间与RV射血时间比值异常，这是PH的两个指标。
脐膨出患者的肺动脉压力升高，GO患者比非GO患者更严重。初始超声心动图显示的RV功能障碍与死亡率显著相关。
