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Introduction: Neurocognitive disorders (NCD) are a growing concern in congenital heart disease (CHD) patients. We hypothesized that early life cardiopulmonary bypass (CBP) as well as age at intervention (AAI) were risk factors for NCDs on long-term follow-up in CHD patients. Methods: Data source was the Quebec CHD database. We defined NCD based on ICD 9 and 10 codes including autism spectrum disorder, global developmental disorder, specific developmental disorder, attention deficit hyperactive disorder, intellectual disability or specific learning deficit. We compared NCD risk from 1983-2010 in 3 cohorts of patients undergoing procedures in childhood: percutaneous atrial septal defect (ASD) closure (no CBP group), surgical ASD closure (short CBP group), and complex CHD lesion repair surgeries (long CBP group). Patients were followed from intervention until NCD diagnosis, death, or administrative censoring whichever came first. Results: By 27 years of follow-up, the cumulative risk of NCD was significantly higher in the complex surgery group compared to the ASD surgery group (crude, 24.9% and 13.4%; p Conclusions: In long-term follow-up of CHD patients, younger AAI of CBP and male sex were associated with increased risk of NCD. Our findings suggest that CBP duration in a wide range of CHD lesions and cohort effects are important predictors of NCD.

This article reviews the changing epidemiology of congenital heart disease summarizing its impact on the demographics of the congenital heart disease population and the progress made in order to improve outcomes in this patient population. Birth prevalence of congenital heart disease can be modified by many factors. As a result of decreasing mortality and increasing survival in all forms of congenital heart disease, the median age of patients has increased and adults now compose two-thirds of patients with congenital heart disease. Disease burden and resulting health services utilization increase significantly across the lifespan. Bridging the gap between policy and quality of care can be improved by referral to specialized adult congenital heart disease centers and planning delivery of specialized services that are commensurate with population needs, program accreditation criteria and certified training of designated workforce.

The number of adults with congenital heart disease (CHD) has increased markedly over the past few decades as a result of astounding successes in pediatric cardiac care. Nevertheless, it is now well understood that CHD is not cured but palliated, such that life-long expert care is required to optimize outcomes. All countries in the world that experience improved survival in CHD must face new challenges inherent to the emergence of a growing and aging CHD population with changing needs and medical and psychosocial issues. Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with CHD worldwide. Recognizing the unique and varied issues involved in caring for adults with CHD, ISACHD established a task force to assess the current status of care for adults with CHD across the globe, highlight major challenges and priorities, and provide future direction. The writing committee consisted of experts from North America, South America, Europe, South Asia, East Asia, and Oceania. The committee was divided into subgroups to review key aspects of adult CHD (ACHD) care. Regional representatives were tasked with investigating and reporting on relevant local issues as accurately as possible, within the constraints of available data. The resulting ISACHD position statement addresses changing patterns of worldwide epidemiology, models of care and organization of care, education and training, and the global research landscape in ACHD.