Hypoplastic Left Heart Syndrome and Other Shunt-Dependent Single Ventricles

Pediatr Crit Care Med. 2016 Aug;17(8 Suppl 1):S318-22. doi: 10.1097/PCC.0000000000000783.


Objectives: In this review, we discuss the pathophysiology, treatment, and outcomes of patients with the hypoplastic left heart syndrome and other single ventricle variants prior to and following surgery.

Data source: MEDLINE and PubMed.

Conclusions: Patients with shunted single ventricle physiology are at increased risk for acute hemodynamic decompensation owing to the increased myocardial workload, the dynamic balance between systemic and pulmonary circulations, and the potential for shunt obstruction. Understanding of the physiology and anticipatory management are critical to prevent hemodynamic compromise and cardiac arrest.

Publication types

  • Review

MeSH terms

  • Cardiac Surgical Procedures / adverse effects
  • Cardiac Surgical Procedures / methods*
  • Cardiac Surgical Procedures / mortality
  • Heart Ventricles / physiopathology*
  • Hemodynamics / physiology
  • Humans
  • Hypoplastic Left Heart Syndrome / physiopathology
  • Hypoplastic Left Heart Syndrome / surgery*
  • Infant
  • Infant, Newborn