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Impact of prenatal haemodynamic and functional abnormalities in Ebstein’s anomaly on survival

Published online by Cambridge University Press:  29 December 2014

Grace Freire ,
Thieu Nguyen ,
Priya Sekar ,
Marilyn Wilhm ,
Kathy Arnold ,
Jenny Leshko ,
Ernest K. Amankwah  and
James Huhta
Grace Freire*
Affiliation:
Johns Hopkins All Children’s Heart Institute, All Children’s Hospital, St Petersburg, Florida, United States of America
Thieu Nguyen
Affiliation:
Johns Hopkins All Children’s Heart Institute, All Children’s Hospital, St Petersburg, Florida, United States of America
Priya Sekar
Affiliation:
Department of Cardiology, Johns Hopkins University, Baltimore, Maryland, United States of America
Marilyn Wilhm
Affiliation:
Johns Hopkins All Children’s Heart Institute, All Children’s Hospital, St Petersburg, Florida, United States of America
Kathy Arnold
Affiliation:
Johns Hopkins All Children’s Heart Institute, All Children’s Hospital, St Petersburg, Florida, United States of America
Jenny Leshko
Affiliation:
Clinical and Translational Research Office, Johns Hopkins All Children’s Hospital, St Petersburg, Florida, United States of America
Ernest K. Amankwah
Affiliation:
Clinical and Translational Research Office, Johns Hopkins All Children’s Hospital, St Petersburg, Florida, United States of America
James Huhta
Affiliation:
Department of Perinatal Cardiology, All Children’s Hospital, St Petersburg, Florida, United States of America Department of Pediatrics, University of Florida, Gainesville, Florida, United States of America
*
Correspondence to: Dr G. Freire, MD, Johns Hopkins All Children’s Heart Institute, 2nd Floor Outpatient Care Center # 206, 601 5th Street South, Saint Petersburg, FL 33701, United States of America. Tel: +727 767 6483; Fax: +727 767 8990; E-mail: gfreire1@jhmi.edu
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Abstract

Predicting outcomes of foetuses with Ebstein’s anomaly and tricuspid valve dysplasia continues to be challenging. Limited data exist on the prognostic significance of prenatal haemodynamic and functional parameters in this population. Our aim was to investigate the prognostic significance of haemodynamic and ventricular functional parameters in addition to associated morphometric parameters in patients with Ebstein’s anomaly. We reviewed medical records of foetuses with Ebstein’s anomaly and tricuspid valve dysplasia at All Children’s Hospital Johns Hopkins Medicine and Johns Hopkins University between 2005 and 2012. The main outcome was survival past 30 days from birth; participants who died in utero or <30 days after birth were considered non-survivors. There were 13 survivors and seven non-survivors. We found that participants with abnormal right ventricular function predicted by low tricuspid regurgitation velocity (<2.3 m/second) (p=0.012) and low estimated right ventricular pressure (<24 mmHg) (p=0.029), a low (<7) cardiovascular profile score (p=0.029) and high (>0.53) cardiothoracic ratio (p=0.008) at the first foetal echocardiogram were less likely to survive. In addition, participants with a fossa ovalis/atrial septal length ratio <0.36 at the last foetal echocardiogram (p=0.051) were more likely to die, albeit of borderline statistical significance. Low tricuspid regurgitation velocity and low right ventricular estimated pressure, or a low cardiovascular profile score could be potential prognostic factors for Ebstein’s anomaly and tricuspid valve dysplasia. However, future larger prospective studies are needed to confirm these initial findings.

Keywords

Ebstein’s anomalycardiovascular profile scorecardiothoracic ratiofossa ovalis/atrial septal length ratiocerebroplacental ratioright ventricular pressure
Type
Original Article
Information
Cardiology in the Young , Volume 24 , Issue 6: HeartWeek 2014 , December 2014 , pp. 1049 - 1056
Copyright
© Cambridge University Press 2014 

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