Hypoplastic left heart syndrome: Valuing the survival

Objective: To examine the survival. developmental status, qualify 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 Wealth Questionnaire Parent Fomat-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.