Abstract 2765: How Many Infants with Critical Congenital Heart Disease Are Missed?
Objectives: To estimate the number of infants with critical congenital heart disease (CCHD) who died and in whom the diagnoses were missed, and to evaluate the clinical and demographic characteristics of missed CCHD cases.
Methods: We used 1989 –2004 statewide death registry data from the Death Statistics Master File at the California Department of Health Services. Initial case selection criteria included: >24 hours old and < 1 year old at the age of death; cause of death as one of the CCHD diagnoses; no surgery was performed. Based on the information available in the death registry, we defined ``missed” CHD as cases in which autopsy results were used to establish the cause of death; ``probable missed”cases as those deaths which occurred at home, out-of-hospital or in the hospital emergency department (ED) and were reported to a coroner; and ``possible missed” cases as all selected cases except for infants with hypoplastic left heart syndrome (HLHS) who were likely to be receiving comfort care.
Results: The initial case selection resulted in 898 deaths. A slight male predominance (55%) was noted. 152 missed, 37 probable missed, and an additional 262 possible missed cases of infants with CCHD were found. Median age at death was 9 days for all selected cases, and was 13.5 days for 152 missed cases. Among 152 missed cases, 6.6% deaths occurred at home and 45% died in the hospital ED. HLHS and coarctation of aorta were the most common diagnoses missed, accounting for 2/3 of missed cases and 46% all missed, probable and possible missed cases. We estimated that 10 missed cases and an additional 20 probable and possible missed cases occurred in California per year. Analysis of the time trend showed the number of missed cases decreased between 1989 and 1999, and stayed unchanged after 1999.
Conclusions: In 1989 –2004, up to 30 infants died of missed CCHD diagnoses in California per year. The median age of death in the missed cases was younger than two weeks, therefore, more than half of these infants did not have a chance to be re-examined by a pediatrician at a 2-week-old well child visit. HLHS and coarctation of aorta account for the majority of deaths from missed CHD.