title: Specialized adult congenital heart disease care: the impact of policy on mortality.
abstract: BACKGROUND
  Clinical guidelines recommend specialized care for adult congenital heart disease (ACHD) patients. In reality, few patients receive such dedicated care. We sought to examine the impact of specialized care on ACHD patient mortality.
  
  
  RESULTS
  We examined referral rates to specialized ACHD centers and ACHD patient mortality rates between 1990 and 2005 in the population-based Quebec Congenital Heart Disease database (n=71 467). This period covers several years before and after the publication of guidelines endorsing specialized care for ACHD patients. A time-series design, based on Joinpoint and Poisson regression analyses, was used to assess the changes in annual referral and patient mortality rates. The association between specialized ACHD care and all-cause mortality was assessed in both case-control and cohort studies. The time-series analysis demonstrated a significant increase in referral rates to specialized ACHD centers in 1997 (rate ratio, +7.4%; 95% confidence interval [CI], +6.6% to +8.2%). In parallel, a significant reduction in expected ACHD patient mortality was observed after year 2000 (rate ratio, -5.0%;95% CI, -10.8% to -0.8%). In exploratory post hoc cohort and case-control analyses, specialized ACHD care was independently associated with reduced mortality (hazard ratio, 0.78; 95% CI, 0.65-0.94) and a reduced odds of death (adjusted odds ratio, 0.82; 95% CI, 0.08-0.97), respectively. This effect was predominantly driven by patients with severe congenital heart disease (hazard ratio, 0.38; 95% CI, 0.22-0.67).
  
  
  CONCLUSIONS
  A significant increase in referrals to specialized ACHD centers followed the introduction of the clinical guidelines. Moreover, referral to specialized ACHD care was independently associated with a significant mortality reduction. Our findings support a model of specialized care for all ACHD patients.
authors.affiliation: McGill Adult Unit for Congenital Heart Disease, McGill University Health Centre, Montreal, Canada (D.M., R.I.-I., J.T., A.M.); Department of Epidemiology & Biostatistics, McGill University, Montreal, Canada (L.P., M.A.); Division of General Internal Medicine, Department of Medicine, McGill University, Montreal, Canada (L.P.); Division of Clinical Epidemiology, Research Institute McGill University Health Center, Montreal, Canada (L.P., M.A.); Department of Cardiology, Montreal Heart Institute, Université de Montréal, Montreal, Canada (P.K.); and Stollery Children's Hospital and University of Alberta, Edmonton, Alberta, Canada (A.S.M.).
authors.forename: Darren
authors.initials: D
authors.lastname: Mylotte
authors.forename: Louise
authors.initials: L
authors.lastname: Pilote
authors.forename: Raluca
authors.initials: R
authors.lastname: Ionescu-Ittu
authors.forename: Michal
authors.initials: M
authors.lastname: Abrahamowicz
authors.forename: Paul
authors.initials: P
authors.lastname: Khairy
authors.forename: Judith
authors.initials: J
authors.lastname: Therrien
authors.forename: Andrew S
authors.initials: AS
authors.lastname: Mackie
authors.forename: Ariane
authors.initials: A
authors.lastname: Marelli
mesh_terms: D000328:Adult; D002309:Cardiology / Q000706:statistics & numerical data*; D016022:Case-Control Studies; D016208:Databases, Factual / Q000706:statistics & numerical data; D005260:Female; D006291:Health Policy; D006330:Heart Defects, Congenital / Q000401:mortality* / Q000628:therapy*; D017052:Hospital Mortality; D006801:Humans; D015994:Incidence; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care; D016016:Proportional Hazards Models; D011792:Quebec / Q000453:epidemiology; D012017:Referral and Consultation / Q000706:statistics & numerical data*; D055815:Young Adult
keywords: congenital; heart diseases; mortality; referral and consultation; treatment outcome