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Management of hypoplastic left heart syndrome with intact atrial septum: a two-centre experience

Published online by Cambridge University Press:  08 September 2015

Shyam K. Sathanandam ,
Ranjit Philip ,
David Gamboa ,
Andrew Van Bergen ,
Michel N. Ilbawi ,
Christopher Knott-Craig ,
B. Rush Waller ,
Alexander J. Javois  and
Bettina F. Cuneo
Shyam K. Sathanandam
Affiliation:
Advocate Children’s Hospital, Oak Lawn, Illinois, United States of America University of Tennessee Health Science Center, LeBonheur Children’s Hospital Memphis, Tennessee, United States of America
Ranjit Philip*
Affiliation:
University of Tennessee Health Science Center, LeBonheur Children’s Hospital Memphis, Tennessee, United States of America
David Gamboa
Affiliation:
Advocate Children’s Hospital, Oak Lawn, Illinois, United States of America School of Medicine, The University of Utah, Salt Lake City, Utah, United States of America
Andrew Van Bergen
Affiliation:
Advocate Children’s Hospital, Oak Lawn, Illinois, United States of America
Michel N. Ilbawi
Affiliation:
Advocate Children’s Hospital, Oak Lawn, Illinois, United States of America
Christopher Knott-Craig
Affiliation:
University of Tennessee Health Science Center, LeBonheur Children’s Hospital Memphis, Tennessee, United States of America
B. Rush Waller
Affiliation:
University of Tennessee Health Science Center, LeBonheur Children’s Hospital Memphis, Tennessee, United States of America
Alexander J. Javois
Affiliation:
Advocate Children’s Hospital, Oak Lawn, Illinois, United States of America
Bettina F. Cuneo
Affiliation:
Advocate Children’s Hospital, Oak Lawn, Illinois, United States of America Children’s Hospital Colorado, Aurora, Colorado, United States of America
*
Correspondence to: R. Philip, MD, Division of Pediatric Cardiology, Le Bonheur Children’s Hospital, Heart Institute, 3rd Floor, 50 N. Dunlap Street, Memphis, TN 38103, United States of America. Tel: +901- 287-5092; Fax: +901-287-4646; E-mail: rphilip@uthsc.edu
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Abstract

Introduction

Hypoplastic left heart syndrome with an intact atrial septum is a poor predictor of outcomes. Prenatal assessment of pulmonary venous Doppler and emergent postnatal cardiac intervention may be associated with better outcomes.

Materials and methods

A retrospective review of all hypoplastic left heart syndrome patients in two centres over a 5-year period was performed. Group 1 included patients with adequate inter-atrial communication. Group 2 included patients with prenatal diagnosis with an intact atrial septum who had immediate transcatheter intervention. Group 3 included patients with intact atrial septum who were not prenatally diagnosed and underwent either delayed intervention or no intervention before stage 1 palliation. Primary outcome was survival up to stage 2 palliation.

Results

The incidence of hypoplastic left heart syndrome with a restrictive atrial communication was 11.2% (n=19 of 170). Overall survival to stage 2 or heart transplantation was 85% and 67% for Groups 1 and 2, respectively (n=129/151, n=8/12; p=0.03), and 0% (n=0/7) for Group 3. Survival benefits were observed between Groups 2 and 3 (p<0.001). Foetal pulmonary vein Doppler reverse/forward velocity time integral ratio of ⩾18% (sensitivity, 0.99, 95% CI, 0.58–1; specificity, 0.99, 95% CI, 0.96–1) was predictive of the need for emergent left atrial decompression.

Conclusion

Using a multidisciplinary approach and foetal pulmonary vein Doppler, time-saving measures can be instituted by delivering prenatally diagnosed neonates with hypoplastic left heart syndrome with intact atrial septum close to the cardiac catheterisation suite where left atrial decompression can be performed quickly and safely that may improve survival.

Keywords

Hypoplastic left heart syndromeintact atrial septumcongenital heart disease
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Issue 6 , August 2016 , pp. 1072 - 1081
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Copyright
© Cambridge University Press 2015 

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