Population-based analysis of survival for hypoplastic left heart syndrome

J Pediatr. 2011 Jul;159(1):57-63. doi: 10.1016/j.jpeds.2010.12.054. Epub 2011 Feb 24.


Objective: To analyze survival patterns among infants with hypoplastic left heart syndrome (HLHS) in the State of Michigan.

Study design: Cases of HLHS prevalent at live birth were identified and confirmed within the Michigan Birth Defects Registry from 1992 to 2005 (n=406). Characteristics of infants with HLHS were compared with a 10:1 random control sample.

Results: Compared with 4060 control subjects, the 406 cases of HLHS were more frequently male (62.6% vs 51.4%), born prematurely (<37 weeks gestation; 15.3% vs 8.7%), and born at low birth weight (LBW) (<2.5 kg; 16.0% vs 6.6%). HLHS 1-year survival rate improved over the study period (P=.041). Chromosomal abnormalities, LBW, premature birth, and living in a high poverty neighborhood were significantly associated with death. Controlling for neighborhood poverty, term infants versus preterm with HLHS or LBW were 3.2 times (95% CI: 1.9-5.3; P<.001) more likely to survive at least 1 year. Controlling for age and weight, infants from low-poverty versus high-poverty areas were 1.8 times (95% CI: 1.1-2.8; P=.015) more likely to survive at least 1 year.

Conclusions: Among infants with HLHS in Michigan, those who were premature, LBW, had chromosomal abnormalities, or lived in a high-poverty area were at increased risk for early death.

MeSH terms

  • Case-Control Studies
  • Chromosome Aberrations / statistics & numerical data
  • Female
  • Humans
  • Hypoplastic Left Heart Syndrome / mortality*
  • Infant
  • Infant, Low Birth Weight
  • Infant, Newborn
  • Infant, Premature
  • Linear Models
  • Male
  • Michigan / epidemiology
  • Patient Transfer
  • Poverty
  • Premature Birth
  • Registries
  • Sex Distribution