Original ArticlePopulation-Based Analysis of Survival for Hypoplastic Left Heart Syndrome
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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2019, American Journal of Obstetrics and Gynecology MFMCitation Excerpt :Racial and ethnic differences in mortality rates were most notably observed during the postnatal period and in early childhood,29 which would have significant implications in the cohort that goes on to get pregnant. Additionally, factors that are associated with an increased risk of neonatal death include delivery in a location of increased distance to a cardiac surgical center,30 concomitant noncardiac congenital anomalies and genetic disorders, particularly chromosomal defects,31 and living in a high-poverty neighborhood.32 Our data provide a longitudinal picture of the pregnancy maladaptation cardiac output in patients with Fontan circulation and the associated perinatal morbidity.
The authors declare no conflicts of interest.