Abstract：
A favorable postnatal prognosis in cases of pulmonary atresia/critical stenosis with intact ventricular septum (PA/CS-IVS) is generally equated with the possibility of achieving biventricular (BV) repair. Identification of fetuses that will have postnatal univentricular (UV) circulation is key for prenatal counseling, optimization of perinatal care and decision-making regarding fetal therapy. We aimed to evaluate the accuracy of published models for predicting postnatal circulation in PA/CS-IVS using a large internationally derived validation cohort.
This was a systematic review of published uni- and multiparametric models for the prediction of postnatal circulation based on echocardiographic findings at between 20 and 28 weeks of gestation. Models were externally validated using data from the International Fetal Cardiac Intervention Registry. Sensitivity, specificity, predictive values, area under the receiver-operating-characteristics curves (AUCs) and proportion of cases with true vs predicted outcome were calculated.
Eleven published studies that reported prognostic parameters of postnatal circulation were identified. Models varied widely in terms of the main outcome (UV (n = 3), non-BV (n = 3), BV (n = 3), right-ventricle-dependent coronary circulation (n = 1) or tricuspid valve size at birth (n = 1)) and in terms of the included predictors (single parameters only (n = 6), multiparametric score (n = 4) or both (n = 1)), and were developed on small sample sizes (range, 15-38). Nine models were validated externally given the availability of the required parameters in the validation cohort. Tricuspid valve diameter Z-score, tricuspid regurgitation, ratios between right and left cardiac structures and the presence of ventriculocoronary connections (VCC) were the most commonly evaluated parameters. Multiparametric models including up to four variables (ratios between right and left structures, right ventricular inflow duration, presence of VCC and tricuspid regurgitation) had the best performance (AUC, 0.80-0.89). Overall, the risk of UV outcome was underestimated and that of BV outcome was overestimated by most models.
Current prenatal models for the prediction of postnatal outcome in PA/CS-IVS are heterogeneous. Multiparametric models for predicting UV and non-BV circulation perform well in identifying BV patients but have low sensitivity, underestimating the rate of fetuses that will ultimately have UV circulation. Until better discrimination can be achieved, fetal interventions may need to be limited to only those cases in which non-BV postnatal circulation is certain. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
This was a systematic review of published uni- and multiparametric models for the prediction of postnatal circulation based on echocardiographic findings at between 20 and 28 weeks of gestation. Models were externally validated using data from the International Fetal Cardiac Intervention Registry. Sensitivity, specificity, predictive values, area under the receiver-operating-characteristics curves (AUCs) and proportion of cases with true vs predicted outcome were calculated.
Eleven published studies that reported prognostic parameters of postnatal circulation were identified. Models varied widely in terms of the main outcome (UV (n = 3), non-BV (n = 3), BV (n = 3), right-ventricle-dependent coronary circulation (n = 1) or tricuspid valve size at birth (n = 1)) and in terms of the included predictors (single parameters only (n = 6), multiparametric score (n = 4) or both (n = 1)), and were developed on small sample sizes (range, 15-38). Nine models were validated externally given the availability of the required parameters in the validation cohort. Tricuspid valve diameter Z-score, tricuspid regurgitation, ratios between right and left cardiac structures and the presence of ventriculocoronary connections (VCC) were the most commonly evaluated parameters. Multiparametric models including up to four variables (ratios between right and left structures, right ventricular inflow duration, presence of VCC and tricuspid regurgitation) had the best performance (AUC, 0.80-0.89). Overall, the risk of UV outcome was underestimated and that of BV outcome was overestimated by most models.
Current prenatal models for the prediction of postnatal outcome in PA/CS-IVS are heterogeneous. Multiparametric models for predicting UV and non-BV circulation perform well in identifying BV patients but have low sensitivity, underestimating the rate of fetuses that will ultimately have UV circulation. Until better discrimination can be achieved, fetal interventions may need to be limited to only those cases in which non-BV postnatal circulation is certain. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
摘要：
目的：室间隔完整的肺动脉闭锁/危重狭窄（PA/CS-IVS）存在异质性，良好的出生后预后通常等同于实现双心室（BV）修复的可能性。确定将以产后单室（UV）循环结束的胎儿是产前咨询的关键，优化围产期保健，并做出关于胎儿治疗的决定。几个小系列已经生成了预测产后循环的产前模型。我们的目标是使用大型国际衍生的验证队列来评估所述模型的准确性。
方法：这是对已发表的单参数和多参数模型的系统评价，这些模型基于妊娠20至28周的超声心动图结果预测出生后循环。使用来自国际胎儿心脏介入注册(IFCIR)的数据对模型进行外部验证。灵敏度,特异性，预测值，接收器工作特性曲线下的面积（AUC），并计算真实/预测结果。
结果：11项已发表的研究报告了出生后循环的预后参数。模型在报告的结果方面差异很大[UVn=3，非BVn=3，BV（n=3），右心室依赖性冠状动脉循环（n=1）或出生时三尖瓣大小（n=1）]，包含参数[单(n=7)，多参数评分(n=4)]，并在小样本量(范围15-38)上开发。九个模型进行了外部验证，考虑到他们在验证队列中所需参数的可用性。三尖瓣z值,三尖瓣反流,心室冠状动脉连接（VCC）的存在是最常用的评估参数。多参数模型，包括多达4个变量(右/左结构之间的比率，RV流入持续时间,存在VCC或三尖瓣返流）具有最佳性能（AUC0.80-0.89）。总的来说,紫外线的结果被低估了，和BV被所有模型高估了。
结论：目前用于预测PA/CS-IVS产后结局的产前模型是异质的。针对紫外线和非BV循环的多参数模型在识别BV患者方面表现良好，但敏感性较低。低估胎儿，最终将以紫外线循环结束。直到可以实现更好的歧视，胎儿干预措施可能应该有目的地仅限于那些非BV出生后循环确定的患者.本文受版权保护。保留所有权利。
方法：这是对已发表的单参数和多参数模型的系统评价，这些模型基于妊娠20至28周的超声心动图结果预测出生后循环。
结果：11项已发表的研究报告了出生后循环的预后参数。
结论：目前用于预测PA/CS-IVS产后结局的产前模型是异质的。
