Edwards Lindsay A, Arunamata Alisa, Maskatia Shiraz A, Quirin Amy, Bhombal Shazia, Maeda Katsuhide, Tacy Theresa A, Punn Rajesh
Division of Cardiology, Department of Pediatrics, Stanford University School of Medicine, 750 Welch Road, Suite 305, Palo Alto, CA, 94304, USA.
Division of Neonatology, Department of Pediatrics, Stanford University School of Medicine, Palo Alto, CA, USA.
Pediatr Cardiol. 2019 Aug;40(6):1304-1313. doi: 10.1007/s00246-019-02155-7. Epub 2019 Jul 23.
This study aimed to evaluate fetal echocardiographic parameters associated with neonatal intervention and single-ventricle palliation (SVP) in fetuses with suspected left-sided cardiac lesions. Initial fetal echocardiograms (1/2002-1/2017) were interpreted by the contemporary fetal cardiologist as coarctation of the aorta (COA), left heart hypoplasia (LHH), hypoplastic left heart syndrome (HLHS), mitral valve hypoplasia (MVH) ± stenosis, and aortic valve hypoplasia ± stenosis (AS). The cohort comprised 68 fetuses with suspected left-sided cardiac lesions (COA n = 15, LHH n = 9, HLHS n = 39, MVH n = 1, and AS n = 4). Smaller left ventricular (LV) length Z score, aortic valve Z score, ascending aorta Z score, and aorta/pulmonary artery ratio; left-to-right shunting at the foramen ovale; and retrograde flow in the aortic arch were associated with the need for neonatal intervention (p = 0.005-0.04). Smaller mitral valve (MV) Z score, LV length Z score, aortic valve Z score, ascending aorta Z score, aorta/pulmonary artery ratio, and LV ejection fraction, as well as higher tricuspid valve-to-MV (TV/MV) ratio, right ventricular-to-LV (RV/LV) length ratio, left-to-right shunting at the foramen ovale, abnormal pulmonary vein Doppler, absence of prograde aortic flow, and retrograde flow in the aortic arch were associated with SVP (p < 0.001-0.008). The strongest independent variable associated with SVP was RV/LV length ratio (stepwise logistical regression, p = 0.03); an RV/LV length ratio > 1.28 was associated with SVP with a sensitivity of 76% and specificity of 96% (AUC 0.90, p < 0.001). A fetal RV/LV length ratio of > 1.28 may be a useful threshold for identifying fetuses requiring SVP.
本研究旨在评估与疑似左侧心脏病变胎儿的新生儿干预及单心室姑息治疗（SVP）相关的胎儿超声心动图参数。最初的胎儿超声心动图（2002年1月至2017年1月）由当代胎儿心脏病专家解读为主动脉缩窄（COA）、左心发育不全（LHH）、左心发育不全综合征（HLHS）、二尖瓣发育不全（MVH）±狭窄以及主动脉瓣发育不全±狭窄（AS）。该队列包括68例疑似左侧心脏病变的胎儿（COA 15例、LHH 9例、HLHS 39例、MVH 1例、AS 4例）。较小的左心室（LV）长度Z评分、主动脉瓣Z评分、升主动脉Z评分以及主动脉/肺动脉比值；卵圆孔处的左向右分流；以及主动脉弓内的逆向血流与新生儿干预需求相关（p = 0.005 - 0.04）。较小的二尖瓣（MV）Z评分、LV长度Z评分、主动脉瓣Z评分、升主动脉Z评分、主动脉/肺动脉比值以及LV射血分数，以及较高的三尖瓣与MV（TV/MV）比值、右心室与LV（RV/LV）长度比值、卵圆孔处的左向右分流、异常肺静脉多普勒、主动脉前向血流缺失以及主动脉弓内的逆向血流与SVP相关（p < 0.001 - 0.008）。与SVP相关的最强独立变量是RV/LV长度比值（逐步逻辑回归，p = 0.03）；RV/LV长度比值> 1.28与SVP相关，敏感性为76%，特异性为96%（曲线下面积0.90，p < 0.001）。胎儿RV/LV长度比值> 1.28可能是识别需要SVP胎儿的有用阈值。
