Hypoplastic left heart syndrome

Lancet. 2009 Aug 15;374(9689):551-64. doi: 10.1016/S0140-6736(09)60563-8.


Hypoplastic left heart syndrome is a rare congenital heart defect in which the left side of the heart is underdeveloped. Surgical management of hypoplastic left heart syndrome has changed the prognosis of the condition that was previously regarded as fatal. We discuss surgical strategies based on staged procedures, with the right ventricle supporting both systemic and pulmonary circulation. We also discuss other management options, such as neonatal transplantation and the recent innovation of hybrid techniques. Surgical techniques and the understanding of the pathophysiology of this condition have been at the forefront of neonatal cardiac surgery and intensive care. The management of the syndrome remains a challenge because affected children grow into adolescence and adulthood posing various new problems and demands.

Publication types

  • Review

MeSH terms

  • Age Factors
  • Anastomosis, Surgical / adverse effects
  • Anastomosis, Surgical / methods
  • Coronary Angiography
  • Forecasting
  • Heart Bypass, Right / adverse effects
  • Heart Bypass, Right / methods*
  • Heart Bypass, Right / trends
  • Heart Transplantation
  • Heart Ventricles / surgery
  • Humans
  • Hypoplastic Left Heart Syndrome / diagnosis*
  • Hypoplastic Left Heart Syndrome / epidemiology
  • Hypoplastic Left Heart Syndrome / genetics
  • Hypoplastic Left Heart Syndrome / surgery*
  • Infant, Newborn
  • Intensive Care, Neonatal
  • Neonatal Screening
  • Palliative Care / methods*
  • Palliative Care / trends
  • Patient Care Team / organization & administration
  • Prognosis
  • Pulmonary Artery / surgery
  • Rare Diseases
  • Risk Factors
  • Treatment Outcome
  • Ultrasonography, Prenatal / methods
  • United Kingdom / epidemiology