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Survival of Children With Hypoplastic Left Heart Syndrome
Csaba Siffel1, Tiffany Riehle-Colarusso2, Matthew E Oster3
1National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia; College of Allied Health Sciences, Georgia Regents University, Augusta, Georgia;
Insights
Survival rates for infants with hypoplastic left heart syndrome (HLHS) have significantly improved. Factors like full-term birth and normal weight positively impact long-term survival for children with HLHS.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Defects Research
- Population Health Studies
Background:
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect.
- Understanding factors influencing HLHS survival is crucial for improving outcomes.
- Population-based data provides insights into long-term survival trends.
Purpose of the Study:
- To analyze survival rates of infants diagnosed with hypoplastic left heart syndrome (HLHS).
- To investigate the impact of demographic and clinical factors on HLHS survival.
- To assess survival trends using population-based data from Metropolitan Atlanta.
Main Methods:
- Identified 212 infants with nonsyndromic HLHS born between 1979-2005.
- Utilized the Metropolitan Atlanta Congenital Defects Program data.
- Ascertained vital status through 2009 via vital records linkage.
- Estimated Kaplan-Meier survival probabilities stratified by characteristics.
Main Results:
- Overall survival probability to 2009 was 24%, showing significant improvement over time (0% in 1979-1984 to 42% in 1999-2005).
- Survival was 66% in the first week, 27% in the first year, and 24% in 10 years.
- Poorer survival observed in preterm, low birth weight, and infants from high-poverty neighborhoods.
- Survival for surgically treated children was 52%; long-term survival beyond 1 year was ~90%.
Conclusions:
- Survival to adolescence for nonsyndromic HLHS has improved, particularly for full-term, normal birth weight infants in lower-poverty areas.
- Long-term survival for HLHS survivors beyond infancy is notably high.
- Further research is needed to understand the growing population of HLHS survivors for resource planning.
Objective:
To examine the survival of infants with hypoplastic left heart syndrome (HLHS) and potential influence of demographic and clinical characteristics on survival using population-based data.
Methods:
Infants with nonsyndromic HLHS (n = 212) born between 1979 and 2005 were identified through the Metropolitan Atlanta Congenital Defects Program. Vital status was ascertained through 2009 based on linkage with vital records. We estimated Kaplan-Meier survival probabilities stratified by select demographic and clinical characteristics.
Results:
The overall survival probability to 2009 was 24% and significantly improved over time: from 0% in 1979-1984 to 42% in 1999-2005. Survival probability was 66% during the first week, 27% during the first year of life, and 24% during the first 10 years. Survival of very low and low birth weight or preterm infants and those born in high-poverty neighborhoods was significantly poorer. For children with information on surgical intervention (n = 88), the overall survival was 52%, and preterm infants had significantly poorer survival (31%) compared with term infants (56%). For children who survived to 1 year of age, long-term survival was ∼90%.
Conclusions:
Survival to adolescence of children with nonsyndromic HLHS born in metropolitan Atlanta has significantly improved in recent years, with those born full term, with normal birth weight, or in a low-poverty neighborhood having a higher survival probability. Survival beyond infancy to adolescence is high. A better understanding of the growing population of survivors with HLHS is needed to inform resource planning.
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