Population-Based Analysis of Survival for Hypoplastic Left Heart Syndrome

• Published in: The Journal of pediatrics Vol. 159; no. 1; pp. 57 - 63
• Main Authors: Hirsch, Jennifer C., Copeland, Glenn, Donohue, Janet E., Kirby, Russell S., Grigorescu, Violanda, Gurney, James G.
• Format: Journal Article
• Language: English
• Published: Maryland Heights, MO Elsevier Inc 01.07.2011; Mosby, Inc; Elsevier
• Subjects: Biological and medical sciences; Cardiology. Vascular system; Case-Control Studies; Chromosome Aberrations; Chromosome Aberrations - statistics & numerical data; congenital abnormalities; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; death; epidemiology; Female; General aspects; Heart; Humans; Hypoplastic Left Heart Syndrome; Hypoplastic Left Heart Syndrome - mortality; Infant; Infant, Low Birth Weight; Infant, Newborn; Infant, Premature; infants; Linear Models; Male; Medical sciences; Michigan; Michigan - epidemiology; Miscellaneous; mortality; Patient Transfer; Pediatrics; Poverty; Premature Birth; Public health. Hygiene; Public health. Hygiene-occupational medicine; Registries; risk; Sex Distribution; statistics & numerical data; survival rate; Michigan; LBW; ICD-9; HLHS; International Classification of Diseases; Hypoplastic left heart syndrome; Low birth weight; Pediatrics; Heart disease; Analysis; Cardiovascular disease; Left Heart Hypoplasia; Population; Congenital disease; Survival; Public health; Syndrome
• ISSN: 0022-3476; 1097-6833; 1097-6833
• DOI: 10.1016/j.jpeds.2010.12.054

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