Altit Gabriel, Bhombal Shazia, Van Meurs Krisa, Tacy Theresa A
Division of Neonatology, Department of Pediatrics, McGill University - Montreal Children's Hospital, 1001 Decarie, Montreal, QC, H4A 3J1, Canada.
Division of Neonatal and Developmental Medicine, Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA.
Pediatr Cardiol. 2018 Jun;39(5):993-1000. doi: 10.1007/s00246-018-1850-7. Epub 2018 Mar 9.
Newborns with congenital diaphragmatic hernia (CDH) have varying degrees of pulmonary hypoplasia and pulmonary hypertension (PH), and there is limited evidence that cardiac dysfunction is present. We sought to study early neonatal biventricular function and performance in these patients by reviewing early post-natal echocardiography (ECHO) measurements and comparing them to normal term newborns.
Retrospective case-control study reviewing clinical and ECHO data on term newborns with CDH and normal controls born between 2009 and 2016. Patients were excluded if major anomalies, genetic syndromes, or no ECHO available. PH was assessed by ductal shunting and tricuspid regurgitant jet velocity. Speckle-tracking echocardiography was used to assess myocardial deformation using velocity vector imaging.
Forty-four patients with CDH and 18 age-matched controls were analyzed. Pulmonary pressures were significantly higher in the CDH cohort (systolic pulmonary arterial pressure to systolic blood pressure of 103 ± 13 vs. 78 ± 29%, p = 0.0001). CDH patients had decreased RV fractional area change (FAC - 28.6 ± 11.1 vs. 36.2 ± 9.6%, p = 0.02), tricuspid annular plane of systolic excursion (TAPSE-5.6 ± 1.6 vs. 8.6 ± 1.6 mm, p = 0.0001), and RV outflow tract stroke distance (8.6 ± 2.7 vs. 14.0 ± 4.5 cm, p = 0.0001) compared with controls. The left ventricular (LV) ejection fraction was similar in both groups, but CDH patients had a decreased LV end-diastolic volume by Simpson's rule (2.7 ± 1.0 vs. 5.0 ± 1.8 mL, p = 0.0001) and LVOT stroke distance (9.7 ± 3.4 vs. 12.6 ± 3.6 cm, p = 0.004). Biventricular global longitudinal strain (GLS) was markedly decreased in the CDH population compared to controls (RV-GLS: - 9.0 ± 5.3 vs. - 19.5 ± 1.4%, p = 0.0001; LV GLS: - 13.2 ± 5.8 vs. - 20.8 ± 3.5%, p = 0.0001).
CDH newborns have evidence of biventricular dysfunction and decreased cardiac output. Abnormal function may be a factor in the non-response to pulmonary arterial vasodilators in CDH patients. A two-pronged management strategy aimed at improving cardiac function, as well as reducing pulmonary artery pressure in CDH newborns, may be warranted.
患有先天性膈疝（CDH）的新生儿存在不同程度的肺发育不全和肺动脉高压（PH），而关于存在心脏功能障碍的证据有限。我们试图通过回顾出生后早期超声心动图（ECHO）测量结果并将其与足月正常新生儿进行比较，来研究这些患者的早期新生儿双心室功能和表现。
回顾性病例对照研究，分析2009年至2016年间出生的患有CDH的足月新生儿和正常对照的临床及ECHO数据。如果存在重大畸形、遗传综合征或无ECHO数据，则将患者排除。通过导管分流和三尖瓣反流喷射速度评估PH。使用斑点追踪超声心动图通过速度矢量成像评估心肌变形。
分析了44例患有CDH的患者和18例年龄匹配的对照。CDH队列中的肺动脉压力显著更高（收缩期肺动脉压与收缩压之比为103±13%对78±29%，p = 0.0001）。与对照组相比，CDH患者的右心室面积变化分数（FAC - 28.6±11.1%对36.2±9.6%，p = 0.02）、三尖瓣环收缩期位移平面（TAPSE - 5.6±1.6对8.6±1.6mm，p = 0.0001）以及右心室流出道搏出距离（8.6±2.7对14.0±4.5cm，p = 0.0001）降低。两组的左心室射血分数相似，但根据辛普森法则，CDH患者的左心室舒张末期容积降低（2.7±1.0对5.0±1.8mL，p = 0.0001），左心室流出道搏出距离降低（9.7±3.4对12.6±3.6cm，p = 0.004）。与对照组相比，CDH人群的双心室整体纵向应变（GLS）显著降低（右心室GLS： - 9.0±5.3%对 - 19.5±1.4%，p = 0.0001；左心室GLS： - 13.2±5.8%对 - 20.8±3.5%，p = 0.0001）。
CDH新生儿有双心室功能障碍和心输出量降低的证据。功能异常可能是CDH患者对肺动脉血管扩张剂无反应的一个因素。可能需要一种旨在改善心脏功能以及降低CDH新生儿肺动脉压力的双管齐下的管理策略。
