Age at Referral and Mortality From Critical Congenital Heart Disease

Abstract

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.

Doctor please feel my pulses! An aid to diagnosis in the newborn

“…The diagnosis however will require additional history, further examination findings and appropriate investigations. Hopefully, such an approach will allow for earlier recognition of illness in the newborn leading to better outcomes …”

Creating Awareness for Early Identification of Congenital Heart Disease

“…11 Methods to improve early detection of CCHD appear warranted. Evidence is there that routine pulse oximetry performed on asymptomatic newborns after 24 hours of life, but before hospital discharge, may detect CCHD.…”

Implications of Using Pulse Oximetry to Screen for Critical Congenital Heart Disease in Newborns

“…Prior to pulse oximetry screening, as many as 22 percent to 43 percent of newborns with CCHD were diagnosed 3 days or more after discharge (CDC, ). Late diagnosis of CCHD resulted in 8 percent of these newborns eventually dying in the first year of life (Fixler, Xu, Nembhard, Ethen, & Canfield, ). Even with special ultrasounds, called fetal echocardiograms, which aren't available universally, diagnosis of CCHD during pregnancy is difficult (Ailes et al., ).…”