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Effect of a selection and postoperative care protocol on survival of infants with hypoplastic left heart syndrome

Paul A Checchia1, Ranae Larsen, Ruchir Sehra

  • 1Department of Critical Care Medicine, Loma Linda University Children's Hospital, Loma Linda, California, USA. checchia_p@kids.wustl.edu

The Annals of Thoracic Surgery
|February 5, 2004
PubMed

Insights

A new scoring system improved survival for infants undergoing the Norwood procedure (a heart surgery) by guiding treatment decisions. This program enhanced care for hypoplastic left heart syndrome, reducing bias in procedure selection.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Surgery
  • Medical Decision Support Systems

Background:

  • Infants with hypoplastic left heart syndrome often require complex surgical interventions.
  • Preoperative risk stratification is crucial for optimizing outcomes in these high-risk neonates.
  • The Norwood procedure and heart transplantation are primary treatment options.

Purpose of the Study:

  • To develop and implement a program for assigning infants to either heart transplantation or the Norwood procedure.
  • To enhance postoperative care for infants undergoing the Norwood procedure.
  • To improve survival rates in infants with hypoplastic left heart syndrome.

Main Methods:

  • A weighted scoring system was developed using six risk factors: ventricular function, tricuspid regurgitation, ascending aortic diameter, atrial septal defect characteristics, blood type, and age.
  • The scoring system prospectively assigned mortality risk to guide recommendations for Norwood procedure or transplantation.
  • Postoperative care protocols were enhanced for infants undergoing the Norwood procedure.

Main Results:

  • Survival following the Norwood procedure significantly improved post-implementation (50% vs. 10% at 1 year, p < 0.0001).
  • High-risk infants (score ≤7) undergoing the Norwood procedure had a 33% survival rate at 1 year.
  • Lower-risk infants (score >7) had a 1-year survival rate of 80% after the Norwood procedure; transplant outcomes were unchanged.

Conclusions:

  • Implementation of an institutional management approach significantly improved survival after the Norwood procedure for infants with hypoplastic left heart syndrome.
  • The scoring system may help neutralize historical biases in selecting between the Norwood procedure and heart transplantation.
  • This program enhances the management of complex congenital heart disease in neonates.
Abstract

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