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Hypoplastic left heart syndrome: "to treat or not to treat"
H Osiovich1, E Phillipos, P Byrne
1Neonatal Intensive Care Unit, University of Alberta Hospital, Edmonton, Canada.
Insights
Hypoplastic left heart syndrome (HLHS) management has evolved, with increasing parental choice for active treatment like heart transplantation or Norwood surgery. Compassionate care remains a vital option for families facing this complex congenital heart defect.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Neonatal Surgery
Background:
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect.
- Historically, HLHS had a uniformly fatal prognosis, necessitating careful family counseling.
Purpose of the Study:
- To review institutional experience in managing infants with HLHS.
- To establish a consistent family counseling approach for HLHS based on clinical experience and literature review.
Main Methods:
- Infants with HLHS were categorized into three periods based on treatment approach changes.
- A multidisciplinary team discussed management options: compassionate care, heart transplantation, or Norwood staged surgery.
Main Results:
- Of 99 infants, 58% chose compassionate care, and 42% opted for active treatment.
- Seven infants underwent heart transplantation, all surviving; 27 underwent Norwood stage I, with a 70% survival rate.
- A significant trend towards increased parental selection of active treatment was observed over time.
Conclusions:
- HLHS is no longer uniformly fatal, offering improved outcomes with modern interventions.
- Open discussions with parents are crucial due to long-term outcome uncertainties.
- Compassionate care should be presented as a valid management option until more long-term data become available.
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.
Conclusion:
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.