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Prevalence of Associated Extracardiac Malformations in the Congenital Heart Disease Population

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Abstract

The authors hypothesized that changes in prenatal factors such as termination of pregnancy for fetal anomalies and prenatal vitamin supplementation have altered the epidemiology of patients with multiple congenital anomalies and may have had an impact on their prevalence in the current era. This study reviewed the Nationwide Inpatient Sample database from 1998 to 2008 and compared the prevalence of ECM among live births with a CHD diagnosis (case) and that among live births without a CHD diagnosis (control). For this study, 42 ECM and 10 CHD diagnoses were selected for subanalysis. Longitudinal analysis also was performed to determine temporal variation of ECM prevalence in the CHD population during the 11-year study period. The cohort in this study consisted of 97,154 patients in the case group and 12,078,482 subjects in the control group. The prevalences in the CHD population were 11.4 % for nonsyndromic congenital malformation (NSCM), 2.2 % for genetic syndrome (GS), and 13.6 % for overall extracardiac congenital malformation (ECM). The prevalences in the control group were 6.7 % for NSCM, 0.3 % for GS, and 7.0 % for ECM. The findings showed a strong association of NSCM [odds ratio (OR) 1.88; 95 % confidence interval (CI) 1.73–1.94], GS (OR 2.52; 95 % CI 2.44–2.61), and overall ECM (OR 2.01; 95 % CI 1.97–2.14) with CHD. The prevalences of GS and multiple organ system CM decreased significantly during the study period. This study was the largest and most comprehensive population-based study to evaluate the association between CHD and ECM in newborns.

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Acknowledgments

We acknowledge the Nationwide Inpatient Sample (NIS), the Healthcare Cost and Utilization Project (HCUP), and the Agency for Healthcare Research and Quality for granting us unlimited access to their database. We also thank Jen Yau and Ugochi Egbe for their contribution during data mining, formatting, statistical analysis, and proofreading.

Conflict of interest

The authors have no relevant financial interests, affiliations, or potential conflicts of interest.

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Authors and Affiliations

  1. Division of Pediatric Cardiology, Mount Sinai Medical Center, One Gustave L. Levy Place, Box 1201, New York, NY, 10029, USA

    Alexander Egbe, Santosh Uppu, Simon Lee & Shubhika Srivastava

  2. Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY, USA

    Deborah Ho

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  1. Alexander Egbe
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  2. Santosh Uppu
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  3. Simon Lee
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  4. Deborah Ho
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  5. Shubhika Srivastava
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Correspondence to Alexander Egbe.

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Egbe, A., Uppu, S., Lee, S. et al. Prevalence of Associated Extracardiac Malformations in the Congenital Heart Disease Population. Pediatr Cardiol 35, 1239–1245 (2014). https://doi.org/10.1007/s00246-014-0922-6

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  • DOI: https://doi.org/10.1007/s00246-014-0922-6

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