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Hypoplastic left heart syndrome.

W I Norwood1

  • 1Department of Surgery, University of Pennsylvania School of Medicine, Philadelphia.

The Annals of Thoracic Surgery
|September 11, 1991
PubMed
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This study outlines a three-stage surgical approach for hypoplastic left heart syndrome, achieving 109 early survivors from 151 patients. The hemi-Fontan procedure shows promising results for these complex congenital heart cases.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Surgery
  • Neonatal Critical Care

Background:

  • Hypoplastic left heart syndrome (HLHS) presents complex surgical challenges in newborns.
  • Current management strategies have evolved significantly over time.
  • Maintaining ductal patency and balancing blood flow are critical preoperatively.

Purpose of the Study:

  • To outline the evolution of a three-stage surgical approach for HLHS.
  • To describe the operative details of palliative and definitive procedures.
  • To evaluate the outcomes of the intermediate-stage hemi-Fontan procedure.

Main Methods:

  • Preoperative management including prostaglandin E1 infusion.
  • Description of first-stage palliation and second-stage definitive procedures.

Related Experiment Videos

  • Implementation and assessment of the intermediate hemi-Fontan procedure.
  • Main Results:

    • 151 patients treated with a three-stage approach since January 1989, with 109 early survivors.
    • 78 patients underwent hemi-Fontan operation with 9 deaths; 5 were nonelective.
    • 27 of 78 hemi-Fontan patients proceeded to definitive Fontan with no mortality.

    Conclusions:

    • The three-stage surgical approach, including the hemi-Fontan procedure, offers a viable strategy for HLHS.
    • Early outcomes demonstrate significant survival rates.
    • Further evaluation of the hemi-Fontan stage is warranted for nonelective cases.