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Hypoplastic left heart syndrome: current considerations and expectations

Jeffrey A Feinstein1, D Woodrow Benson, Anne M Dubin

  • 1Department of Pediatrics, Stanford University School of Medicine, Lucile Salter Packard Children's Hospital, Palo Alto, California 94304, USA. jeff.feinstein@stanford.edu

Journal of the American College of Cardiology
|December 24, 2011
PubMed

Insights

Hypoplastic Left Heart Syndrome (HLHS) management has dramatically improved, with 70% of newborns now expected to reach adulthood. This paper details the current state of HLHS care across all stages.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Neonatal Care

Background:

  • Hypoplastic Left Heart Syndrome (HLHS) is a severe congenital heart defect with historically poor outcomes.
  • Significant advancements in diagnosis and treatment have transformed HLHS management and survival rates.
  • Current survival expectations indicate that approximately 70% of newborns with HLHS may reach adulthood.

Purpose of the Study:

  • To present the current state of the art in understanding and treating Hypoplastic Left Heart Syndrome (HLHS).
  • To outline the comprehensive management strategies across all stages of HLHS care.
  • To address critical aspects including genetics, developmental outcomes, and quality of life for HLHS patients.

Main Methods:

  • Review of current diagnostic approaches and management strategies for HLHS.
  • Detailed description of the multi-stage treatment protocol: pre-Stage I, Stage I, Stage II, and Stage III (Fontan surgery).
  • Inclusion of long-term follow-up considerations, genetics, developmental outcomes, and quality of life.

Main Results:

  • Survival rates to age 5 have improved, with expectations for 70% of newborns with HLHS to reach adulthood.
  • A well-established 3-stage treatment approach exists, though center-specific variations are noted.
  • Comprehensive care strategies encompass fetal assessment through long-term follow-up.

Conclusions:

  • Hypoplastic Left Heart Syndrome (HLHS) management has seen a dramatic positive shift in outcomes.
  • A standardized, multi-stage approach is crucial for optimizing HLHS patient care.
  • Holistic management, including genetics and quality of life, is essential for complex HLHS patients.

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