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Background: Adults with congenital heart disease are a relatively new population that progressively increases in size and complexity. In Italy, there are no accurate data concerning the distribution of congenital defects and the long-term outcome relating to both congenital heart disease per se and comorbidities, due to the aging process., Methods: The Piedmont Adult Congenital Heart Disease Registry has been designed to investigate these aspects and to support a high quality healthcare development for grown-up congenital heart patients. Within 2 years, 459 consecutive patients routinely followed in 10 divisions of cardiology in Piedmont were included in the project. Electronic dedicated software has supported data collection., Results: Mean age of patients is 35 ± 16 years. Septal defects are the most common type of congenital heart disease (35.3%). At baseline evaluation, 71.7% of patients reported a previous surgical and/or percutaneous treatment and 6.3% an electrophysiological procedure. Freedom from intervention is 44%, 81% and 56% at the age of 18, 30 and 45 years, respectively. Patients who had a treatment during infancy show a better intervention-free survival (p=0.038) compared with patients treated during adulthood. Despite the majority of the population had an almost preserved functional status, 27.5% had ≥1 long-term sequelae (arrhythmias 27.5%; pulmonary hypertension 5.2%; neurological problems 4.1%; cyanosis 4.8%; liver dysfunction 2.4%; enteropathy 2.4%; lung disease 2.2%). During 2 years of follow-up, the estimated mortality rate is 0.88%., Conclusions: Adults with congenital heart disease are a heterogeneous population of relatively young patients with relevant clinical and social problems. The late sequelae related to both the underlying heart disease and the advancing age require continuous monitoring and lifelong specialized care.

Aim: To investigate the very long-term clinical outcomes of atrial septal defect (ASD) percutaneous closure in adult patients and to evaluate the 12-month effects of the device on aortic and mitral valve function., Methods: Over a 12-year period, a total of 110 consecutive patients underwent percutaneous ASD closure. A yearly clinical follow-up was conducted and any adverse event was recorded. In a 55-patient echocardiographic subgroup, the baseline and 12-month aortic and mitral regurgitation rate was recorded., Results: Mean age was 50.9 ± 17 years and 75% of patients were female. Mean ASD echocardiographic dimension was 17.6 ± 6.2 mm (range, 5-36 mm). Procedural success rate was 97%. After a mean follow-up of 61.8 ± 34.9 months (range, 6-167 months), all-cause death occurred in 2 patients (1.8%) and the composite primary outcome of major adverse cardiovascular event (MACE) occurred in 5 patients (4.5%): 2 non-device related cardiac deaths occurred and 3 surgeries were required. The Kaplan-Meier analysis showed an event-free survival at 140 months of 90%. In the 12-month echocardiographic substudy, no case of significant (moderate or severe) new-onset aortic regurgitation was detected, while 1 case (1.8%) of worsening mild-to-moderate aortic regurgitation was described (P=.90). No case of significant new-onset or worsening mitral regurgitation was noted. No patient needed aortic or mitral surgical repair at very long-term follow-up., Conclusions: Transcatheter ASD closure is a safe procedure with satisfactory very long-term clinical outcomes. The ASD device does not significantly affect aortic and mitral valve function.