Epidemiology of noncomplex left ventricular outflow tract obstruction malformations (aortic valve stenosis, coarctation of the aorta, hypoplastic left heart syndrome) in Texas, 1999–2001
Article first published online: 8 JUL 2005
Copyright © 2005 Wiley-Liss, Inc.
Birth Defects Research Part A: Clinical and Molecular Teratology
Volume 73, Issue 8, pages 555–561, August 2005
How to Cite
McBride, K. L., Marengo, L., Canfield, M., Langlois, P., Fixler, D. and Belmont, J. W. (2005), Epidemiology of noncomplex left ventricular outflow tract obstruction malformations (aortic valve stenosis, coarctation of the aorta, hypoplastic left heart syndrome) in Texas, 1999–2001. Birth Defects Research Part A: Clinical and Molecular Teratology, 73: 555–561. doi: 10.1002/bdra.20169
- Issue published online: 5 AUG 2005
- Article first published online: 8 JUL 2005
- Manuscript Accepted: 4 APR 2005
- Manuscript Received: 10 DEC 2004
- March of Dimes
- National Institutes of Health (NIH). Grant Numbers: K23 HL70823, K12 HD43372, K12 HD41648, R01 HD39056
- Centers for Disease Control and Prevention via Texas Center for Birth Defects Research and Prevention, Birth Defects Epidemiology and Surveillance Branch, Texas Department of State Health Services. Grant Number: U50/CCU613232
- congenital heart disease;
- Poisson distribution;
- regression analysis;
- southwestern United States;
The left ventricular outflow tract (LVOT) malformations aortic valve stenosis (AVS), coarctation of the aorta (CoA), and hypoplastic left heart syndrome (HLHS) contribute significantly to infant mortality due to birth defects. Previous epidemiology data showed rate differences between male and female and white and black ethnic groups. The Texas Birth Defects Registry, an active surveillance program, enables study in a large, diverse population including Hispanics.
Records of children up to 1 year old with AVS, CoA, and HLHS born in Texas from 1999 to 2001, were collected from the registry. Those including additional heart defects or a chromosomal anomaly were excluded. Multivariate analysis included: infant sex; United States–Mexico border county residence; and maternal age, race/ethnicity, birthplace, and education.
There were 910 cases among 1.08 million live births, of which 499 met inclusion criteria. Multivariate modeling of all LVOT malformations combined demonstrated lower prevalence rate ratios (PRRs) for black males (0.26) and Hispanic males (0.70). Similar results were found for CoA but not AVS or HLHS. Higher PRRs were noted for increased maternal age for LVOT (1.3 for 24–34 years; 1.7 for >34 years), AVS, and HLHS, but not CoA, and higher PRRs across all diagnoses for males (LVOT PRR, 2.4) were noted. CoA PRRs were higher in border county vs. non–border county residents (PRR, 2.1). Maternal education and birthplace were not significant factors.
There are rate differences for males among all 3 ethnic groups. Sex and ethnic differences suggest genetic etiologies, where the ethnic differences could be used to find susceptibility loci with mapping by admixture linkage disequilibrium. Increased CoA rates along the U.S.–Mexico border suggest environmental causes that will require further monitoring. Birth Defects Research (Part A), 2005. © 2005 Wiley-Liss, Inc.