Epidemiology of nonsyndromic conotruncal heart defects in Texas, 1999–2004


  • This publication was supported in part through a cooperative agreement (U01DD000494) between the Centers for Disease Control and Prevention and the Texas Department of State Health Services (DSHS), Austin, TX. Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the Centers for Disease Control and Prevention. This publication was also supported in part by Title V Maternal and Child Health Block Grants Funds from the Office of Title V and Family Health, Texas DSHS, Austin, TX.


INTRODUCTION: Congenital heart defects (CHDs) are the most common structural birth defects, yet their etiology is poorly understood. As there is heterogeneity within the group of CHDs, epidemiologic studies often focus on subgroups, of conditions, such as conotruncal heart defects (CTDs). However, even within these subgroups there may be etiologic heterogeneity. The aim of the present study was to identify and compare maternal and infant characteristics associated with three CTDs: truncus arteriosus (TA), dextro-transposition of the great arteries (d-TGA), and tetralogy of Fallot (TOF). METHODS: Data for cases with nonsyndromic TA (n = 78), d-TGA (n = 438), and TOF (n = 529) from the Texas Birth Defects Registry, 1999–2004, were used to estimate crude and adjusted prevalence ratios, separately for each condition, using Poisson regression. Polytomous logistic regression was used to determine whether the observed associations were similar across the two largest case groups (d-TGA and TOF). RESULTS: In Texas, 1999–2004, the prevalence of nonsyndromic TA, d-TGA, and TOF was 0.35, 1.98, and 2.40 per 10,000 live births, respectively. There was evidence of a significant linear increase in the risk of each condition with advancing maternal age (p < 0.01). Significant associations were observed for TA and maternal residence on the Texas-Mexico border; d-TGA and infant sex, maternal race/ethnicity, history of previous live birth, and birth year; and TOF and maternal race/ethnicity and education. Further, the associations with some, but not all, of the study variables were significantly different for d-TGA and TOF. CONCLUSION: These findings add to our limited understanding of the epidemiology of CTDs. Birth Defects Research (Part A), 2010. © 2010 Wiley-Liss, Inc.