Related Experiment Video
Updated: Aug 12, 2026
Left Lung Orthotopic Transplantation in a Juvenile Porcine Model for ESLP
Published on: February 14, 2022
Hypoplastic left heart syndrome: valuing the survival
D L Williams1, A C Gelijns, A J Moskowitz
1International Center for Health Outcomes and Innovation Research, Department of Surgery, Columbia University, College of Physicians and Surgeons, New York Presbyterian Hospital, New York, NY, USA.
Insights
Staged surgical repair for hypoplastic left heart syndrome (HLHS) shows 5-year survival of 54%, with developmental delays in early stages. Long-term outcomes require further study.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Developmental Pediatrics
Background:
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect requiring complex surgical palliation.
- Staged reconstructive surgery aims to improve survival and quality of life for affected children.
Purpose of the Study:
- To evaluate survival rates, developmental status, quality of life, and direct medical costs in children with HLHS undergoing staged surgical repair (Stages I, II, and III).
Main Methods:
- Kaplan-Meier survival analysis was performed on 106 children with HLHS who underwent staged repair between 1990 and 1999.
- Quality of life and developmental progress were assessed using validated parent-reported questionnaires (CHQ-PF28, ASQ).
- Hospital costs were calculated using the ratio-of-costs-to-charges method; physician and outpatient medication costs were also captured.
Main Results:
- Institutional 1- and 5-year survival rates were 58% and 54%, respectively. Predictors of survival included birth weight, need for inotropic drugs, and surgical experience.
- Children undergoing Norwood Stage I repair showed fewer developmental milestones compared to those surviving to later stages.
- Median inpatient costs for Stages I, II, and III were $51,000, $33,892, and $52,183, with outpatient/readmission costs <10% of total.
Conclusions:
- Staged surgical repair for HLHS yields moderate survival rates but highlights potential developmental challenges in early stages.
- Further prospective research is necessary to comprehensively assess long-term developmental and quality-of-life outcomes, alongside cost-effectiveness of staged palliation and heart transplantation.
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.
More Related Videos
04:37Left Atrial Ligation in the Avian Embryo as a Model for Altered Hemodynamic Loading During Early Vascular Development
Published on: June 16, 2023
05:18Implantation of Left Ventricular Assist Device (LVAD) in Juvenile Landrace Swine: A LVAD Implantation Model of Pediatric Heart Failure
Published on: January 16, 2026
Related Concept Videos
01:22Mitral Stenosis I: Introduction
01:22Heart Failure VI: Adjunct Therapies
01:30Cardiomyopathy II: Dilated Cardiomyopathy
01:29Cardiomyopathy III: Hypertrophic Cardiomyopathy
01:29Cardiomyopathy V: Interprofessional Care
01:28Cardiomyopathy VII: Pre and Post Operative Nursing Management