Original Article
Population-Based Analysis of Survival for Hypoplastic Left Heart Syndrome

https://doi.org/10.1016/j.jpeds.2010.12.054Get rights and content

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.

Section snippets

Methods

This study was approved by human subject research review committees at both the University of Michigan and the Michigan Department of Community Health. Infants with HLHS diagnosis and included in the birth defects surveillance database were identified within the Michigan Birth Defects Registry from 1992 to 2005 by the Division of Vital Records & Health Statistics. The Michigan Birth Defects Registry is a statewide passive population-based registry covering all of Michigan. Case reports are

Results

From 1992 through 2005, 1 859 717 live births were registered in the State of Michigan. Of these, 697 infants were initially identified with HLHS through the Michigan Birth Defects Registry, and, for confirmatory purposes, hospital discharge data were available for assessment of 645 (93%) of these infants. Registry and discharge data were used to verify each HLHS case through individual case evaluation. This resulted in 416 infants confirmed as having HLHS (0.022% of live births) and, after

Discussion

This study from a population-based cohort of live births in Michigan illustrates that infants with HLHS differed from the general newborn population in that they were more likely to be born male, premature, or of low birth weight. However, race/ethnicity, neighborhood poverty level, access to care, and health insurance payer status did not differ between the HLHS and general newborn populations. Among children born with HLHS, we found that chromosomal anomalies, prematurity, low birth weight,

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    The authors declare no conflicts of interest.

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