Novel approach to prenatal predictors of outcomes for fetuses with severe Ebstein anomaly

Abstract

Objective

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.

Methods

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.

Results

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.

Conclusions

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.

KEY POINTS

• Pulmonary hypoplasia is associated with severe morbidity

• There are limited prognosticating tools to risk stratify and guide management in cases of severe prenatal Ebstein anomalies

• Fetal MRI may improve prognostication for fetuses with EA

Keywords:

• Ebstein anomaly
• cardiothoracic ratio
• fetal magnetic resonance imaging
• fetal echocardiography
• fetal lungs

Disclosure statement

No potential conflict of interest was reported by the author(s).

Additional information

Funding

There are no funding sources to disclose