Division of Pediatric Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, ON, Canada, Toronto.
Division of Cardiology, Capital Institute of Pediatrics, Beijing, China.
Cardiol Young. 2022 Nov;32(11):1768-1779. doi: 10.1017/S1047951121004935. Epub 2022 Jan 6.
To evaluate the impact of fetal haemodynamics on surgical and neurodevelopmental outcomes in severe Ebstein anomaly and tricuspid valve dysplasia.
Thirty-four fetuses with Ebstein anomaly/tricuspid valve dysplasia were referred from 2013 to 2019 for fetal echocardiography and clinical management. Nineteen fetuses with Ebstein anomaly/tricuspid valve dysplasia and 30 controls underwent cardiovascular magnetic resonance to quantify the fetal blood flow and to calculate cerebral oxygen delivery (cDO2) and consumption (cVO2). The 3D steady-state free precession acquisition was used to measure fetal brain volume. Surgical outcome, brain MRI, and neurodevelopmental follow-up were reviewed.
Twenty-six fetuses were live born (76%) and survival (65%) at a mean follow-up of 4 years. Nine fetuses had a brain MRI before discharge, and all had clinically silent injuries and volume loss. At 18 months, five single-ventricle patients had a neurodevelopmental delay in cognition and language (mean percentile: 11th), with gross-motor skills more affected than fine-motor skills (mean percentiles: 4th and 34th). Fetuses with Ebstein anomaly/tricuspid valve dysplasia had smaller brains, lower combined ventricular output, ascending aorta, superior caval vien and umbilical vein flows, lower oxygen saturation in ascending aorta and superior caval vien, lower cDO2 and cVO2 (p < 0.05). Superior caval vien/combined ventricular output and descending aorta/combined ventricular output ratios were lower in fetuses with circular shunt (p < 0.05). Fetuses requiring the Starnes procedure tended to have smaller brains, lower combined ventricular output, superior caval vien, descending aorta, and umbilical vein flows.
All patients with Ebstein anomaly/tricuspid valve dysplasia are at high risk of neurodevelopmental delay and warrant follow-up. Fetal cardiovascular magnetic resonance revealed impaired brain growth with diminished cerebral blood flow and cDO2, the extenting dependent on the severity of the haemodynamic compromise.
评估胎儿血液动力学对重度埃布斯坦畸形和三尖瓣发育不良的手术和神经发育结局的影响。
2013 年至 2019 年，34 例埃布斯坦畸形/三尖瓣发育不良胎儿因胎儿超声心动图和临床管理而转诊。19 例埃布斯坦畸形/三尖瓣发育不良胎儿和 30 例对照胎儿接受心血管磁共振成像（CMR）以量化胎儿血流并计算脑氧输送（cDO2）和消耗（cVO2）。使用三维稳态自由进动采集测量胎儿脑容量。回顾手术结果、脑 MRI 和神经发育随访情况。
26 例胎儿（76%）存活分娩（65%），平均随访 4 年。9 例胎儿在出院前进行了脑 MRI 检查，所有胎儿均有临床无症状性损伤和脑容量损失。18 个月时，5 例单心室患者在认知和语言方面存在神经发育迟缓（平均百分位数：第 11 位），粗运动技能比精细运动技能受影响更大（平均百分位数：第 4 位和第 34 位）。埃布斯坦畸形/三尖瓣发育不良胎儿的脑容量较小，合并心室输出、升主动脉、上腔静脉和脐静脉流量较低，升主动脉和上腔静脉氧饱和度较低，cDO2 和 cVO2 较低（p < 0.05）。存在环形分流的胎儿上腔静脉/合并心室输出和降主动脉/合并心室输出比值较低（p < 0.05）。需要进行斯泰恩斯手术的胎儿往往脑容量较小，合并心室输出、升主动脉、降主动脉和脐静脉流量较低。
所有埃布斯坦畸形/三尖瓣发育不良患者均有发生神经发育迟缓的高风险，需要进行随访。胎儿心血管磁共振成像显示，脑生长受损，脑血流和 cDO2 减少，其程度取决于血液动力学损害的严重程度。
