Hypoplastic left heart syndrome: "to treat or not to treat".
OBJECTIVE
To review our experience in managing infants with hypoplastic left heart syndrome (HLHS) and to establish a consistent approach in counseling families based on our experience and on review of medical literature.


METHODS
Infants were divided into three group periods based on significant changes in the approach to treatment. After a diagnosis of HLHS was made, a multidisciplinary team met the family to discuss different management options: compassionate care, active treatment by heart transplantation, or Norwood staged surgery.


RESULTS
Parents of 58 infants (58%) opted for compassionate care, and 41 (42%) opted for active treatment. Seven infants underwent heart transplantation and all remain alive. A total of 27 infants underwent Norwood stage I, and 19 (70%) survived. There was a significant increase in parental choice for active treatment over the three time periods.


CONCLUSIONS
These results indicate that HLHS can no longer be regarded as a uniformly fatal congenital anomaly. However, due to uncertainty about long-term outcome, discussion with parents should be open, and compassionate care should be presented as a management option until long-term data are available.
Osiovich
H
H
Neonatal Intensive Care Unit, University of Alberta Hospital, Edmonton, Canada.
Phillipos
E
E
Byrne
P
P
Robertson
M
M
D005066:Euthanasia, Passive; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality / Q000628:therapy*; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies