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Hypoplastic left heart syndrome: from in-utero diagnosis to school age

Jack Rychik1

  • 1Fetal Heart Program, The Cardiac Center at The Children's Hospital of Philadelphia, and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA. rychik@email.chop.edu

Seminars in Fetal & Neonatal Medicine
|October 26, 2005
PubMed

Insights

Hypoplastic left heart syndrome (HLHS) is increasingly diagnosed prenatally and treated successfully. Ongoing research tracks surgical survivors, identifying morbidities and seeking new therapies for lifelong cardiovascular challenges.

Area of Science:

  • Pediatric Cardiology
  • Developmental Biology
  • Congenital Heart Disease Research

Background:

  • Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect.
  • Advances in prenatal diagnosis are improving early identification of HLHS.
  • Successful surgical interventions offer improved survival outcomes for affected infants.

Purpose of the Study:

  • To review the current understanding of HLHS development during gestation.
  • To highlight the ongoing monitoring and study of HLHS surgical survivors.
  • To identify existing morbidities and knowledge gaps in HLHS pathophysiology.

Main Methods:

  • Review of current literature on HLHS development and outcomes.
  • Analysis of follow-up data from HLHS surgical survivors.
  • Identification of pathophysiological mechanisms underlying long-term morbidities.

Main Results:

  • Prenatal diagnosis of HLHS is common and successful treatments lead to survival.
  • Long-term follow-up reveals various morbidities in HLHS survivors.
  • Significant questions remain regarding the pathophysiological basis of these morbidities.

Conclusions:

  • Continued research into HLHS pathophysiology is crucial.
  • Development of novel therapies is needed to address lifelong challenges in adult HLHS survivors.
  • Further understanding of this unique cardiovascular condition will guide future treatments.

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