Skip to main content

Advertisement

Log in

Congenital Heart Disease and Myelomeningocele in the Newborn: Prevalence and Mortality

  • Original Article
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

Myelomeningocele (MMC) and congenital heart disease (CHD) are independent risk factors for increased morbidity and mortality in the newborn period and each can require significant operations shortly after birth. Few studies have examined the impact of these combined lesions. We sought to examine the incidence of CHD in patients with MMC, and to evaluate length of stay (LOS), hospital charges, and mortality. Using the Texas Inpatient Public Use Data File, ~ 6.9 million newborn records between 1/1999 and 12/2016 were examined. Hospitalizations were classified as MMC without CHD (n = 3054), CHD without MMC (n = 72,266), and MMC with CHD (n = 171). The birth prevalence of CHD with MMC was 0.3/10,000 live hospital births, with 5% of patients with MMC having CHD, and 0.2% of those with CHD having MMC. There was increased LOS in patients with both MMC and CHD (median 15 days, IQR 5–31), compared to CHD without MMC (median 6 days, IQR 2–20) and MMC without CHD (median 8 days, IQR 1–14) and higher total hospital charges (median $95,007, IQR $26,731–$222,660) compared to CHD without MMC (median $27,726, $6463–$118,370) and MMC without CHD (median $40,066, IQR $5744–$97,490). Mortality was significantly higher in patients with MMC and CHD (22.2% compared to 3.1% in MMC without CHD and 4.1% in CHD without MMC). Significance remained when limiting for patients without genetic conditions or additional major birth defects. MMC with CHD in the newborn compared to either CHD or MMC alone is associated with longer LOS, higher charges, and increased mortality.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

Data Availability

The Texas inpatient public use data file is available by request from the Texas Department of State Health Services.

Code Availability

International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) or Tenth Revision, Clinical Modification (ICD-10) codes.

References

  1. Parker SE, Mai CT, Canfield MA et al (2010) Updated national birth prevalence estimates for selected birth defects in the United States, 2004–2006. Birth Defects Res Part A Clin Mol Teratol 88:1008–1016. https://doi.org/10.1002/bdra.20735

    Article  CAS  Google Scholar 

  2. Blom HJ, Smulders Y (2011) Overview of homocysteine and folate metabolism. With special references to cardiovascular disease and neural tube defects. J Inherit Metab Dis 34:75–81. https://doi.org/10.1007/s10545-010-9177-4

    Article  CAS  PubMed  Google Scholar 

  3. Williams J, Mai CT, Mulinare J (2015) National birth defects prevention month and folic acid awareness week—January 2015 updated estimates of neural tube defects prevented by mandatory folic acid fortification—United States, 1995–2011. Centers Dis Control Prev Morb Mortal Wkly Rep 64:1–6

    Google Scholar 

  4. Canfield MA, Honein MA, Yuskiv N et al (2006) National estimates and race/ethnic-specific variation of selected birth defects in the United States, 1999–2001. Birth Defects Res Part A Clin Mol Teratol 76:747–756. https://doi.org/10.1002/bdra.20294

    Article  CAS  Google Scholar 

  5. Liu S, Joseph KS, Luo W et al (2016) Effect of folic acid food fortification in Canada on congenital heart disease subtypes. Circulation 134:647–655. https://doi.org/10.1161/CIRCULATIONAHA.116.022126

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Abouk R, Grosse SD, Ailes EC, Oster ME (2017) Association of US State implementation of newborn screening policies for critical congenital heart disease with early infant cardiac deaths. JAMA 318:2111. https://doi.org/10.1001/jama.2017.17627

    Article  PubMed  PubMed Central  Google Scholar 

  7. Shin M, Kucik JE, Siffel C et al (2012) Improved survival among children with spina bifida in the United States. J Pediatr 161:1132-1137.e3. https://doi.org/10.1016/j.jpeds.2012.05.040

    Article  PubMed  PubMed Central  Google Scholar 

  8. Adzick NS, Thom EA, Spong CY et al (2011) A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 364:993–1004. https://doi.org/10.1056/NEJMoa1014379

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Moon-Grady AJ, Morris SA, Belfort M et al (2015) International fetal cardiac intervention registry: a worldwide collaborative description and preliminary outcomes. J Am Coll Cardiol 66:388–399. https://doi.org/10.1016/j.jacc.2015.05.037

    Article  PubMed  Google Scholar 

  10. Deptartment of State Health (2019) Texas inpatient public use data file. https://www.dshs.texas.gov/thcic/hospitals/Inpatientpudf.shtm. Accessed 7 Jan 2019

  11. Yoon PW, Rasmussen SA, Lynberg MC et al (2001) The national birth defects prevention study. Public Health Rep 116:32–40. https://doi.org/10.1093/phr/116.s1.32

    Article  PubMed  PubMed Central  Google Scholar 

  12. U.S B of L and S (2019) Consumer Price Index inflation calculator. https://data.bls.gov/cgi-bin/cpicalc.pl. Accessed 17 Feb 2019

  13. American Institute of Ultrasound in Medicine (2013) Practice parameter for the performance of fetal echocrdiography

  14. Vernon MM, Powell D, Schultz AH et al (2015) Is routine preoperative transthoracic echocardiography necessary in newborns with myelomeningocele? J Perinatol 35:842–845. https://doi.org/10.1038/jp.2015.74

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Ritter S, Tani LY, Shaddy RE, Minich LL (1999) Are screening echocardiograms warranted for neonates with meningomyelocele? Arch Pediatr Adolesc Med 153:1264–1266

    Article  CAS  Google Scholar 

  16. Moeini Naghani I, Hashemi Zonouz T, Shahjouei S et al (2014) Congenital cardiac anomalies in myelomeningocele patients. Acta Med Acad 43:160–164. https://doi.org/10.5644/ama2006-124.115

    Article  PubMed  Google Scholar 

  17. Koçak G, Onal C, Koçak A et al (2008) Prevalence and outcome of congenital heart disease in patients with neural tube defect. J Child Neurol 23:526–530. https://doi.org/10.1177/0883073807309789

    Article  PubMed  Google Scholar 

Download references

Funding

No specific support.

Author information

Authors and Affiliations

Authors

Contributions

JKW and SAM conceptualized and designed the study, carried out data analysis, drafted the initial manuscript, and reviewed and revised the manuscript. WEW critically reviewed and revised the manuscript for important intellectual content. YW assisted in data analysis. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

Corresponding author

Correspondence to J. Kevin Wilkes.

Ethics declarations

Conflict of interest

The authors have no conflicts of interest relevant to this article to disclose.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary Information

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Wilkes, J.K., Whitehead, W.E., Wang, Y. et al. Congenital Heart Disease and Myelomeningocele in the Newborn: Prevalence and Mortality. Pediatr Cardiol 42, 1026–1032 (2021). https://doi.org/10.1007/s00246-021-02576-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-021-02576-3

Keywords

Navigation