title: [Preliminary results and future perspectives of the Piedmont Adult Congenital Heart Disease Registry].
abstract: 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.
authors.affiliation: S.C. Cardiologia Pediatrica, Ospedale Regina Margherita, A.O.U. Città della Salute e della Scienza, Torino.
authors.forename: Raffaella
authors.initials: R
authors.lastname: Marzullo
authors.affiliation: S.C. Cardiologia Pediatrica, Ospedale Regina Margherita, A.O.U. Città della Salute e della Scienza, Torino.
authors.forename: Roberto
authors.initials: R
authors.lastname: Bordese
authors.affiliation: U.O. Cardiologia, Presidio Ospedaliero di Savigliano, Savigliano (CN).
authors.forename: Antonia
authors.initials: A
authors.lastname: Bassignana
authors.affiliation: S.C. Cardiologia Pediatrica, Ospedale Regina Margherita, A.O.U. Città della Salute e della Scienza, Torino.
authors.forename: Gaetana
authors.initials: G
authors.lastname: Ferraro
authors.affiliation: U.O. Cardiologia, Ospedale di Bra, Bra (CN).
authors.forename: Giovanni
authors.initials: G
authors.lastname: Dall'Orto
authors.affiliation: S.C. Cardiologia, Ospedale Maggiore della Carità, Novara.
authors.forename: Lorena
authors.initials: L
authors.lastname: Ferrarotti
authors.affiliation: Dipartimento Cardiovascolare e Toracico, A.O.U. Città della Salute e della Scienza, Torino.
authors.forename: Daniela
authors.initials: D
authors.lastname: Libertucci
authors.affiliation: U.O. Cardiologia, Ospedale Martini, Torino.
authors.forename: Laura
authors.initials: L
authors.lastname: Rissone
authors.affiliation: U.O. Cardiologia, Ospedale di Mondovì, Mondovì (CN).
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Amoroso
authors.affiliation: Dipartimento di Cardiochirurgia, Ospedale Ordine Mauriziano, Torino.
authors.forename: Guglielmo
authors.initials: G
authors.lastname: Actis Dato
authors.affiliation: S.C. Pneumologia, A.O.U. San Luigi Gonzaga, Orbassano (TO).
authors.forename: Carlo
authors.initials: C
authors.lastname: Albera
authors.affiliation: Dipartimento di Scienze Mediche e Chirurgiche, A.O.U. Città della Salute e della Scienza, Torino.
authors.forename: Laura
authors.initials: L
authors.lastname: Bergamasco
authors.affiliation: S.C. Cardiologia Pediatrica, Ospedale Regina Margherita, A.O.U. Città della Salute e della Scienza, Torino.
authors.forename: Gabriella
authors.initials: G
authors.lastname: Agnoletti
mesh_terms: D000328:Adult; D000800:Angioplasty, Balloon / Q000639:trends; D006348:Cardiac Surgical Procedures / Q000639:trends; D002309:Cardiology / Q000639:trends; D057286:Electronic Health Records / Q000639:trends; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000401:mortality* / Q000601:surgery* / Q000628:therapy; D006343:Heart Septal Defects / Q000401:mortality / Q000601:surgery; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011446:Prospective Studies; D012042:Registries; D012086:Reoperation / Q000639:trends; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends