Age at referral and mortality from critical congenital heart disease

Pediatrics. 2014 Jul;134(1):e98-105. doi: 10.1542/peds.2013-2895.


Background and objective: Newborn pulse oximetry screening is recommended to promote early referral of neonates with critical congenital heart disease (CCHD) and reduce mortality; however, the impact of late referral on mortality is not well defined. The purpose of this population-based study was to describe the association between timing of referral to a cardiac center and mortality in 2360 liveborn neonates with CCHD.

Methods: Neonates with CCHD born before pulse oximetry screening (1996-2007) were selected from the Texas Birth Defects Registry and linked to state birth and death records. Age at referral was ascertained from date of first cardiac procedure at a cardiac center. Logistic and Cox proportional hazards regression models were used to estimate factors associated with late referral and mortality; the Kaplan-Meier method was used to estimate 3-month survival.

Results: Median age at referral was 1 day (25th-75th percentile: 0-6 days). Overall, 27.5% (649 of 2360) were referred after age 4 days and 7.5% (178 of 2360) had no record of referral. Neonatal mortality was 18.1% (277 of 1533) for those referred at 0 to 4 days of age, 9.0% (34 of 379) for those referred at 5 to 27 days of age, and 38.8% (69 of 178) for those with no referral. No improvement in age at referral was found across the 2 eras within 1996-2007.

Conclusions: A significant proportion of neonates with CCHD experienced late or no referral to cardiac specialty centers, accounting for a significant number of the deaths. Future population-based studies are needed to determine the benefit of pulse oximetry screening on mortality and morbidity.

Keywords: congenital heart disease; epidemiology; health policy and outcome research; neonatal mortality.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Age Factors
  • Critical Illness
  • Female
  • Heart Defects, Congenital / diagnosis
  • Heart Defects, Congenital / mortality*
  • Heart Diseases / congenital*
  • Heart Diseases / diagnosis
  • Heart Diseases / mortality*
  • Humans
  • Infant, Newborn
  • Male
  • Oximetry
  • Referral and Consultation / statistics & numerical data*