OBJECTIVE: Congenital heart disease (CHD) is a leading cause of premature death in infants and children. Currently limited data are available regarding lesion specific mortality over time. Our study aimed to describe pediatric mortality trends by CHD lesion in the United States. METHODS: We conducted a 19 year analysis (1999 to 2017) of publicly available, deidentified multiple cause of death data compiled and produced by the National Center for Health Statistics. Analysis was stratified by CHD diagnosis and age using 3 age categories (infants, 1 to 4 years, and 5 to 17 years). Temporal trends of CHD mortality and the effect of contributing risk factors were analyzed by using joinpoint regression. RESULTS: Mortality was highest for in infants for all CHD lesions, in particular for total anomalous pulmonary venous return. Significant declines in infant CHD mortality occurred for most other lesions. Contributing risk factors, including prematurity, extracardiac birth defects, and genetic conditions, occurred in 19% of infant CHD deaths and demonstrated worse mortality trends in the majority of lesions. Mortality rates remained highest for single ventricle lesions in all ages, with an infant mortality rate plateau in the later half of the study and progressive increasing mortality rates for children 5 to 17 years. CONCLUSIONS: CHD mortality is decreasing for most lesions. Because of the heterogenicity of CHD lesions, there is expected variability in mortality trends by lesion and age group. Single ventricle lesions continue to contribute most heavily to premature death because of CHD demonstrated by significant increases in mortality rate for children aged 5 to 17 years.

Introduction: The prevalence of attention deficit/hyperactivity disorder in the general population is common and is now diagnosed in 4%–12% of children. Children with CHD have been shown to be at increased risk for attention deficit/hyperactivity disorder. Case reports have led to concern regarding the use of attention deficit/hyperactivity disorder medications in children with underlying CHD. We hypothesised that medical therapy for patients with CHD and attention deficit/hyperactivity disorder is safe. Methods: A single-centre, retrospective chart review was performed evaluating for adverse events in patients aged 4–21 years with CHD who received attention deficit/hyperactivity disorder therapy over a 5-year span. Inclusion criteria were a diagnosis of CHD and concomitant medical therapy with amphetamines, methylphenidate, or atomoxetine. Patients with trivial or spontaneously resolved CHD were excluded from analysis. Results: In 831 patients with CHD who received stimulants with a mean age of 12.9 years, there was only one adverse cardiovascular event identified. Using sensitivity analysis, our median follow-up time was 686 days and a prevalence rate of 0.21% of adverse events. This episode consisted of increased frequency of supraventricular tachycardia in a patient who had this condition prior to initiation of medical therapy; the condition improved with discontinuation of attention deficit/hyperactivity disorder therapy. Conclusion: The incidence of significant adverse cardiovascular events in our population was similar to the prevalence of supraventricular tachycardia in the general population. Our single-centre experience demonstrated no increased risk in adverse events related to medical therapy for children with attention deficit/hyperactivity disorder and underlying CHD. Further population-based studies are indicated to validate these findings.

Background: Adults with congenital heart disease (ACHD) comprise an increasing proportion of individuals living with CHD in the United States (US) although little is known regarding lesion or age-specific mortality in the modern era. Our study aimed to describe current era ACHD mortality rates by age and CHD lesion and provide temporal mortality rate trends for the last two decades. Methods: We conducted a 19-year analysis (1999-2017) of publicly available, de-identified Multiple Cause of Death data compiled and produced by the National Center for Health Statistics. Age and lesion-specific mortality rates were calculated using joinpoint regression.Results: ACHD mortality rates decreased by an average of 2%-4% per year for all adults. CHD lesions resulting in the highest ACHD-related mortality varied by age. Unlike the other lesions, mortality attributed to single ventricle physiology failed to improve in early adulthood (average of 1.6% increase per year).In decedents age 65 years or older, simpler forms of CHD like shunt lesions became more prominent contributors, accounting for 46% of deaths. Conclusion:Rates of mortality due to ACHD have declined significantly for adults with CHD, however, continued mortality due to single ventricle physiology remains an area requiring improved strategies to increase survival.