Contemporary prognosis of adult congenital heart disease

Congenital heart disease (CHD), encompassing a spectrum of structural abnormalities of the heart or major vessels at birth that can be mild, moderately severe or severe, is the most prevalent congenital defect in new-borns with a birth prevalence of 8-9 per 1000 live births. Advances in diagnosis and treatment of CHD during the past six decades have improved survival of new-borns with CHD. Consequently, the adult CHD (ACHD) population is growing and ageing, with the disease spectrum shifting toward proportionately more severe CHD. Contemporary estimates of ACHD prevalence in the developed world are 3-6 per 1000 adults. Despite advancements in treatment, most defects cannot be cured and majority of ACHD patients face delayed cardiac complications of residual CHD lesions and prior cardiac surgery. Consequently, lifelong surveillance and care is indicated. Providing effective care for the growing, ageing and increasingly complex ACHD population is a major challenge, that requires insight into the long-term prognosis in order to determine appropriate allocation of resources and optimal surveillance strategies. Multi-centre, region-wide ACHD databases are necessary to collect such comprehensive lifelong follow-up data. In this thesis, data from the Dutch nationwide CONgenital CORvitia (CONCOR) registry of ACHD patients is used to determine the risk of specific outcomes in ACHD patients. In the different sections, we focus on survival of patients with an atrial septal defect, aortic dissections and -surgery in patients with aortopathy-associated CHD types, and risk of infective endocarditis and coronary artery disease in patients with the full spectrum of CHD.