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Author Spotlight: Effect of Left Atrial Ligation on Avian Embryonic Hearts and HLHS Implications
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Hypoplastic left ventricle and scimitar syndrome

Aaron W Eckhauser1, Michael L O'Byrne, Paul M Weinberg

  • 1Primary Children's Medical Center and Division of Pediatric Cardiothoracic Surgery, Department of Surgery, University of Utah, Salt Lake City, Utah.

The Annals of Thoracic Surgery
|December 4, 2013
PubMed

Insights

Hypoplastic left ventricle with scimitar syndrome is a rare congenital heart defect. Survivors have undergone heart transplant, Norwood, or Fontan operations, showing varied treatment success.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • Hypoplastic left ventricle (HLV) and scimitar syndrome (SS) are rare, complex congenital heart defects.
  • The combined anatomy presents unique diagnostic and therapeutic challenges.

Observation:

  • This study reports on five patients with the co-occurring HLV and SS.
  • Follow-up data were collected for these patients at a single institution.

Findings:

  • Four out of five patients with HLV and SS are currently alive.
  • Survivors achieved this through diverse surgical interventions: two underwent heart transplantation, one had a Norwood operation, and one completed staged palliation with a Fontan operation.

Implications:

  • This rare combination of congenital heart defects can be managed with various advanced surgical strategies.
  • Successful outcomes highlight the importance of tailored, staged interventions for complex pediatric cardiac conditions.

Keywords:
11, 21

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