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The Miracle Baby Grows Up: Hypoplastic Left Heart Syndrome in the Adult

Matthew Lewis1, Marlon Rosenbaum2

  • 1Herbert Irving Pavilion, 161 Fort Washington Ave Suite 627, New York, NY, 10032, USA. ml3329@cumc.columbia.edu.

Current Cardiology Reports
|July 27, 2017
PubMed

Insights

Hypoplastic left heart syndrome (HLHS) patients now survive to adulthood but face lifelong complications from single-ventricle palliation. Recognizing and addressing these challenges is crucial for improving long-term outcomes and quality of life.

Area of Science:

  • Adult congenital heart disease
  • Single-ventricle palliation
  • Hypoplastic left heart syndrome (HLHS)

Background:

  • Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect characterized by underdevelopment of the left heart.
  • Historically fatal in infancy, modern surgical techniques now enable HLHS patients to reach adulthood.
  • Despite improved survival, significant long-term morbidity and mortality persist in the adult HLHS population.

Purpose of the Study:

  • To review common sources of morbidity and mortality in adult patients with Hypoplastic left heart syndrome (HLHS).
  • To identify areas requiring further research to improve the prognosis and quality of life for this growing patient group.
  • To emphasize the importance of understanding lifelong challenges associated with single-ventricle repair.

Main Methods:

  • This review synthesizes current literature on the long-term outcomes of Hypoplastic left heart syndrome (HLHS).
  • Analysis focuses on complications arising from single-ventricle palliation, particularly the Fontan procedure.
  • Identifies patient-specific risks and areas for future clinical investigation.

Main Results:

  • Adults with palliated Hypoplastic left heart syndrome (HLHS) experience significant lifelong morbidity due to elevated systemic venous pressures post-Fontan procedure.
  • Downstream organ dysfunction is a major concern, influencing management and organ allocation decisions.
  • Patients with a morphologic systemic right ventricle, common in HLHS, may face even higher risks.

Conclusions:

  • The growing adult Hypoplastic left heart syndrome (HLHS) population requires increased recognition of their unique lifelong health challenges.
  • A collaborative approach between patients and healthcare providers is essential to address clinical uncertainties.
  • Continued research and coordinated care are vital for enhancing patient prognosis and quality of life.
Abstract

Keywords:
Adult congenital heart diseaseFontanHypoplastic left heart syndromeSingle ventricle

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