Abstract：
UNASSIGNED: Ebstein anomaly (EA) is a cardiac malformation with highly variable presentation and severity with limited perinatal management options. We present incorporation of fetal lung measurements into a multidisciplinary evaluation for counseling and predicting postnatal outcomes in patients with severe EA.
UNASSIGNED: Five fetuses with severe fetal EA were reviewed. Third trimester sonographic observed/expected total lung area (O/E TLA) and lung to head ratio (O/E LHR), fetal MRI total fetal lung volume ratio (O/E-TFLV), echocardiographic cardio-thoracic ratio (CT ratio), sonographic estimated fetal weight (EFW) by Hadlock formula and presence of hydrops, were used to guide perinatal management.
UNASSIGNED: Three of five had appropriate fetal growth, were delivered at term in a cardiac operative suite, and underwent immediate intervention with good neonatal outcomes. Two had severe fetal growth restriction (FGR), CT ratios > 0.8 and O/E LHR and TLA < 25%. One of which delivered prematurely with neonatal demise and one suffered in utero demise at 34 weeks.
UNASSIGNED: FGR, hydrops, increased CT ratio and reduced O/E LHR and TFLV are potential prognosticators of poor outcomes in severe EA, and should be validated in larger cohorts that would allow for a statistical analysis of the predictive utility of these measurements.
Pulmonary hypoplasia is associated with severe morbidityThere are limited prognosticating tools to risk stratify and guide management in cases of severe prenatal Ebstein anomaliesFetal MRI may improve prognostication for fetuses with EA.
UNASSIGNED: Five fetuses with severe fetal EA were reviewed. Third trimester sonographic observed/expected total lung area (O/E TLA) and lung to head ratio (O/E LHR), fetal MRI total fetal lung volume ratio (O/E-TFLV), echocardiographic cardio-thoracic ratio (CT ratio), sonographic estimated fetal weight (EFW) by Hadlock formula and presence of hydrops, were used to guide perinatal management.
UNASSIGNED: Three of five had appropriate fetal growth, were delivered at term in a cardiac operative suite, and underwent immediate intervention with good neonatal outcomes. Two had severe fetal growth restriction (FGR), CT ratios > 0.8 and O/E LHR and TLA < 25%. One of which delivered prematurely with neonatal demise and one suffered in utero demise at 34 weeks.
UNASSIGNED: FGR, hydrops, increased CT ratio and reduced O/E LHR and TFLV are potential prognosticators of poor outcomes in severe EA, and should be validated in larger cohorts that would allow for a statistical analysis of the predictive utility of these measurements.
Pulmonary hypoplasia is associated with severe morbidityThere are limited prognosticating tools to risk stratify and guide management in cases of severe prenatal Ebstein anomaliesFetal MRI may improve prognostication for fetuses with EA.
摘要：
■Ebstein异常（EA）是一种心脏畸形，具有高度可变的表现和严重程度，围产期管理选择有限。我们提出将胎儿肺测量纳入多学科评估，以指导和预测重度EA患者的产后结局。
■对5例严重胎儿EA的胎儿进行了回顾。妊娠晚期超声观察/预期总肺面积（O/ETLA）和肺头比（O/ELHR），胎儿MRI胎儿肺总容积比（O/E-TFLV），超声心动图心胸比（CT比），通过Hadlock公式进行超声检查估计胎儿体重（EFW）和积水的存在，用于指导围产期管理。
■五分之三的胎儿发育良好,在心脏手术套件中分娩，并立即接受干预,新生儿结局良好.两个有严重的胎儿生长受限（FGR），CT比值>0.8，O/ELHR和TLA<25%。其中一个因新生儿死亡而过早分娩，另一个在34周时在子宫内死亡。
■FGR，积水,CT比率升高和O/ELHR和TFLV降低是严重EA不良结局的潜在预测因素,并且应该在更大的队列中进行验证，以便对这些测量的预测效用进行统计分析。
肺发育不全与严重病态相关在严重产前Ebstein异常病例中，风险分层和指导管理的预测工具有限。
■对5例严重胎儿EA的胎儿进行了回顾。妊娠晚期超声观察/预期总肺面积（O/ETLA）和肺头比（O/ELHR），胎儿MRI胎儿肺总容积比（O/E-TFLV），超声心动图心胸比（CT比），通过Hadlock公式进行超声检查估计胎儿体重（EFW）和积水的存在，用于指导围产期管理。
■五分之三的胎儿发育良好,在心脏手术套件中分娩，并立即接受干预,新生儿结局良好.两个有严重的胎儿生长受限（FGR），CT比值>0.8，O/ELHR和TLA<25%。其中一个因新生儿死亡而过早分娩，另一个在34周时在子宫内死亡。
■FGR，积水,CT比率升高和O/ELHR和TFLV降低是严重EA不良结局的潜在预测因素,并且应该在更大的队列中进行验证，以便对这些测量的预测效用进行统计分析。
肺发育不全与严重病态相关在严重产前Ebstein异常病例中，风险分层和指导管理的预测工具有限。
