Hypoplastic left heart syndrome: valuing the survival

J Thorac Cardiovasc Surg. 2000 Apr;119(4 Pt 1):720-31. doi: 10.1016/S0022-5223(00)70007-9.


Objective: To examine the survival, developmental status, quality of life, and direct medical costs of children with hypoplastic left heart syndrome who have undergone stage I, II, and III reconstructive surgery.

Methods: A total of 106 children underwent staged repair for classic hypoplastic left heart syndrome between February 1990 and March 1999 (stage I: 106; stage II: 49; stage III: 25; 4 converted to heart transplantation). Survival was analyzed by the Kaplan-Meier method. In a cross-sectional study, parents assessed quality of life by completing the Infant/Toddler Child Health Questionnaire or Child Health Questionnaire Parent Format-28; they assessed developmental progress by completing the Ages and Stages Questionnaire. The ratio-of-costs-to-charges method was used to derive hospital costs, and payments were used to capture physician time and wholesale pricing for outpatient medications.

Results: Institutional 1-year and 5-year actuarial survivals were 58% and 54%. Birth weight, the need for preoperative inotropic drugs, and surgical experience were predictors of survival. Norwood I patients achieved fewer developmental benchmarks than those who survived to subsequent stages. Child Health Questionnaire Parent Format-28 mean summary scores for physical and psychosocial health were 48.5 +/- 6.3 and 42.8 +/- 9.9. The median inpatient costs for stage I, II, and III repairs were $51,000, $33,892, and $52,183, respectively. Monthly outpatient and readmission costs were less than 10% of total costs.

Conclusion: A prospective, large-scale study of the comprehensive outcomes of staged repair and transplantation is needed. This study will need to address the longer-term developmental and quality-of-life outcomes, as well as the long-term cost effectiveness of these procedures.

MeSH terms

  • Child Development
  • Female
  • Health Care Costs
  • Humans
  • Hypoplastic Left Heart Syndrome / economics
  • Hypoplastic Left Heart Syndrome / mortality*
  • Hypoplastic Left Heart Syndrome / surgery*
  • Infant
  • Infant, Newborn
  • Male
  • Quality of Life
  • Surveys and Questionnaires
  • Survival Rate