Hypoplastic left heart syndrome

Pediatr Nurs. 2001 May-Jun;27(3):245-8, 251-2, 286.


Sixteen years ago, hypoplastic left heart syndrome (HLHS) was a fatal diagnosis. Following birth and closing of the ductus arteriosus, HLHS infants developed metabolic acidosis, decreased systemic perfusion, circulatory collapse, and death. Currently, HLHS can be detected in fetuses in the first trimester of pregnancy. Today's families may choose from four HLHS treatment options, which include (a) medical termination of pregnancy (MTP), (b) supportive care, (c) Norwood (3 Staged) surgical procedure, and/or (d) cardiac transplantation. Presently, pharmacologic therapy with prostaglandin E1 stabilizes HLHS infants until the initiation of a treatment modality. As with many new treatment technologies, the availability of four options forces parents to make difficult decisions. Therefore, parents of HLHS infants require ongoing emotional support, information, and continual family teaching. Nurses caring for these critically ill infants must possess specific skills and knowledge.

Publication types

  • Review

MeSH terms

  • Abortion, Therapeutic
  • Acidosis / etiology
  • Choice Behavior
  • Fontan Procedure
  • Heart Transplantation
  • Humans
  • Hypoplastic Left Heart Syndrome / complications
  • Hypoplastic Left Heart Syndrome / diagnosis*
  • Hypoplastic Left Heart Syndrome / mortality
  • Hypoplastic Left Heart Syndrome / therapy*
  • Infant
  • Palliative Care
  • Parents / psychology
  • Patient Selection
  • Pediatric Nursing / methods
  • Perioperative Care / nursing
  • Risk Factors
  • Shock, Cardiogenic / etiology
  • Survival Rate
  • Treatment Outcome
  • Ultrasonography, Prenatal