Preoperative Stabilization of Infants With Hypoplastic Left Heart Syndrome Before Stage I Palliation

Author(s): Amy Donnellan, RN, DNP, CPNP-AC, Lindsey Justice, RN, DNP, CPNP-AC

Contact Hours 1.00

CERP A 1.00

Expires Feb 01, 2020

Topics: Cardiovascular

Population: Neonatal, Pediatric

Role: Staff

Member: Free
NonMember: $10.00

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Activity Summary

Hypoplastic left heart syndrome is a severe form of congenital heart disease that results in single-ventricle physiology. Although surgical palliation in infants with this syndrome is widely agreed on in practice, variation occurs in both surgical and medical management. Perinatal factors that affect preoperative management include the subtype of the syndrome and the patient’s birth weight, gestational age, and genetic abnormalities. The general aspects of perioperative stabilization and management are monitoring, use of vasoactive infusions, ventilatory support, and nutrition. Management strategies for balancing single-ventricle physiology are aimed at addressing the 3 major causes of desaturation: diminished pulmonary blood flow, low mixed venous oxygen saturation, and pulmonary venous desaturation.


  • Identify prenatal and perinatal factors that impact single ventricle management.
  • Describe strategies of perioperative stabilization and management.
  • Define three major causes of desaturation in single ventricle physiology.

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