Outcomes in Hypoplastic Left Heart Syndrome

Pediatr Clin North Am. 2020 Oct;67(5):945-962. doi: 10.1016/j.pcl.2020.06.008.


Hypoplastic left heart syndrome (HLHS) is a complex form of congenital heart disease defined by anatomic and functional inadequacy of the left side of the heart with nonviability of the left ventricle to perform systemic perfusion. Lethal if not treated, a strategy for survival currently is well established, with continuing improvement in outcomes over the past 30 years. Prenatal diagnosis, good newborn care, improved surgical skills, specialized postoperative care, and unique strategies for interstage monitoring all have contributed to increasing likelihood of survival. The unique life with a single right ventricle and a Fontan circulation is a focused area of investigation.

Keywords: Congenital heart surgery; Fetal echocardiography; Hypoplastic left heart syndrome; Neurocognitive deficits in congenital heart disease; Outcomes for congenital heart disease.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Female
  • Fontan Procedure / methods*
  • Heart Ventricles / abnormalities*
  • Heart Ventricles / physiopathology
  • Humans
  • Hypoplastic Left Heart Syndrome / diagnosis*
  • Hypoplastic Left Heart Syndrome / physiopathology
  • Hypoplastic Left Heart Syndrome / surgery
  • Infant, Newborn
  • Palliative Care / methods*
  • Pregnancy
  • Prenatal Diagnosis / methods*