Fetal aortic valve stenosis: a critique of case selection criteria for fetal intervention. (3rd September 2015)

• Record Type: Journal Article
• Authors: Hunter, Lindsey E.; Chubb, Henry; Miller, Owen; Sharland, Gurleen; Simpson, John M.
• Is Part Of: Prenatal diagnosis. Volume 35:Number 12(2015:Dec.)
• Page Start: 1176
• Page End: 1181
• Publication Date: 2015-09-03
• Subjects: Prenatal diagnosis -- Periodicals; Fetus -- Diseases -- Diagnosis -- Periodicals; Electronic journals; 618.32075
• DOI: 10.1002/pd.4661
• Languages: English
• ISSNs: 0197-3851

Abstract

Abstract: Objective: Selection of fetuses with aortic stenosis (AS) for prenatal intervention has been influenced by published scoring systems. This study aimed to test these scoring systems by retrospective application to consecutive cases of fetal AS. Methods: Retrospective analysis of the echocardiographic findings of 31 consecutive fetuses with AS evaluated at a tertiary fetal cardiology centre. Published 'eHLHS' scores and threshold scores were applied to the group and compared to postnatal management, in terms of biventricular repair versus single ventricle palliation. Results: Thirty‐one fetuses were identified with AS, and eHLHS was identified in 17 at the initial echocardiogram. No fetus with a full eHLHS score (3/3 or 4/4) achieved a biventricular repair. Three fetuses had a favourable threshold score (≥4), one of whom had a successful biventricular outcome. Seven fetuses had an unfavourable threshold score (<4) and underwent a univentricular pathway. Conclusion: The eHLHS score is a reliable predictor for the progression to HLHS at term. The score identifies those who would achieve a biventricular repair postnatally without prenatal intervention. A minority of fetuses with favourable threshold scores may achieve a biventricular repair postnatally without prenatal intervention, but eHLHS and an unfavourable threshold score (<4) predict a single ventricle pathway postnatally. © 2015 John Wiley & Sons, Ltd. Abstract : What's already known about this topic? Fetal aortic valve stenosis is a progressive disease that can result in hypoplastic left heart syndrome (HLHS) by term. Published scoring systems are available to predict progression to HLHS and identify fetuses who may benefit from fetal intervention. What does this study add? The published score for predicting evolving HLHS is accurate. A favourable 'threshold score' in the absence of fetal intervention results in a biventricular repair only in a minority of cases.