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Age at referral and mortality from critical congenital heart disease
David E Fixler1, Ping Xu2, Wendy N Nembhard2
1Division of Pediatric Cardiology, Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas; david.fixler@childrens.com.
Insights
Late or no referral for critical congenital heart disease (CCHD) in newborns is linked to increased mortality. Early diagnosis and timely cardiac center referral are crucial for improving survival rates in CCHD infants.
Area of Science:
- Neonatal cardiology
- Public health
- Pediatric screening
Background:
- Critical congenital heart disease (CCHD) screening aims for early detection and referral.
- The impact of delayed referral on CCHD mortality requires further definition.
Purpose of the Study:
- To examine the association between referral timing and mortality in neonates with CCHD.
- To describe referral patterns in a population-based cohort.
Main Methods:
- Population-based study of 2360 neonates with CCHD born between 1996-2007.
- Data linkage with Texas Birth Defects Registry and state vital records.
- Logistic regression, Cox proportional hazards, and Kaplan-Meier methods analyzed referral timing and mortality.
Main Results:
- Median referral age was 1 day; 27.5% were referred after 4 days, and 7.5% had no referral record.
- Mortality rates were 18.1% (0-4 days referral), 9.0% (5-27 days referral), and 38.8% (no referral).
- No significant improvement in referral timing was observed across the study period.
Conclusions:
- A substantial number of CCHD neonates face late or no referral, contributing to preventable deaths.
- Further research is needed to quantify the benefits of pulse oximetry screening on CCHD outcomes.
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.
