Specialized Adult Congenital Heart Disease Care: The Impact of Policy on Mortality
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.
Methods and 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 (CHD) database (n=71,467). This period covers several years before and after publication of guidelines endorsing specialized care for ACHD patients. A time-series design, based on Joinpoint and Poisson regression analyses, was used to assess 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 [RR] +7.4%; 95% CI +6.6% to +8.2%). In parallel, a significant reduction in expected ACHD patient mortality was observed after year 2000 (RR -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 (HR), 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 CHD (HR, 0.38;95% CI 0.22-0.67).
Conclusions—A significant increase in referrals to specialized-ACHD centers followed the introduction of 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.
- Received August 21, 2013.
- Revision received December 14, 2013.
- Accepted January 17, 2014.