Management of hypoplastic left heart syndrome with intact atrial septum

A two-centre experience

Shyam Sathanandam, Ranjit Philip, David Gamboa, Andrew Van Bergen, Michel N. Ilbawi, Christopher Knott-Craig, B. Rush Waller, Alexander J. Javois, Bettina F. Cuneo

Research output: Contribution to journalArticle

2 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)1072-1081
Number of pages10
JournalCardiology in the young
Volume26
Issue number6
DOIs
StatePublished - Aug 1 2016

Fingerprint

Atrial Septum
Hypoplastic Left Heart Syndrome
Survival
Pulmonary Veins
Decompression
Communication
Heart Transplantation
Cardiac Catheterization
Prenatal Diagnosis
Newborn Infant
Lung
Incidence

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

Cite this

Sathanandam, S., Philip, R., Gamboa, D., Van Bergen, A., Ilbawi, M. N., Knott-Craig, C., ... Cuneo, B. F. (2016). Management of hypoplastic left heart syndrome with intact atrial septum: A two-centre experience. Cardiology in the young, 26(6), 1072-1081. https://doi.org/10.1017/S1047951115001791

Management of hypoplastic left heart syndrome with intact atrial septum : A two-centre experience. / Sathanandam, Shyam; Philip, Ranjit; Gamboa, David; Van Bergen, Andrew; Ilbawi, Michel N.; Knott-Craig, Christopher; Waller, B. Rush; Javois, Alexander J.; Cuneo, Bettina F.

In: Cardiology in the young, Vol. 26, No. 6, 01.08.2016, p. 1072-1081.

Research output: Contribution to journalArticle

Sathanandam, S, Philip, R, Gamboa, D, Van Bergen, A, Ilbawi, MN, Knott-Craig, C, Waller, BR, Javois, AJ & Cuneo, BF 2016, 'Management of hypoplastic left heart syndrome with intact atrial septum: A two-centre experience', Cardiology in the young, vol. 26, no. 6, pp. 1072-1081. https://doi.org/10.1017/S1047951115001791
Sathanandam, Shyam ; Philip, Ranjit ; Gamboa, David ; Van Bergen, Andrew ; Ilbawi, Michel N. ; Knott-Craig, Christopher ; Waller, B. Rush ; Javois, Alexander J. ; Cuneo, Bettina F. / Management of hypoplastic left heart syndrome with intact atrial septum : A two-centre experience. In: Cardiology in the young. 2016 ; Vol. 26, No. 6. pp. 1072-1081.
@article{18b8f3097665473aa7d8facaab9e504d,
title = "Management of hypoplastic left heart syndrome with intact atrial septum: A two-centre experience",
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.",
author = "Shyam Sathanandam and Ranjit Philip and David Gamboa and {Van Bergen}, Andrew and Ilbawi, {Michel N.} and Christopher Knott-Craig and Waller, {B. Rush} and Javois, {Alexander J.} and Cuneo, {Bettina F.}",
year = "2016",
month = "8",
day = "1",
doi = "10.1017/S1047951115001791",
language = "English (US)",
volume = "26",
pages = "1072--1081",
journal = "Cardiology in the Young",
issn = "1047-9511",
publisher = "Cambridge University Press",
number = "6",

}

TY - JOUR

T1 - Management of hypoplastic left heart syndrome with intact atrial septum

T2 - A two-centre experience

AU - Sathanandam, Shyam

AU - Philip, Ranjit

AU - Gamboa, David

AU - Van Bergen, Andrew

AU - Ilbawi, Michel N.

AU - Knott-Craig, Christopher

AU - Waller, B. Rush

AU - Javois, Alexander J.

AU - Cuneo, Bettina F.

PY - 2016/8/1

Y1 - 2016/8/1

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=84940920758&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84940920758&partnerID=8YFLogxK

U2 - 10.1017/S1047951115001791

DO - 10.1017/S1047951115001791

M3 - Article

VL - 26

SP - 1072

EP - 1081

JO - Cardiology in the Young

JF - Cardiology in the Young

SN - 1047-9511

IS - 6

ER -