Specialized Adult Congenital Heart Disease Care: The Impact of Policy on Mortality

Study Questions:

What is the impact of specialized care on adult congenital heart disease (ACHD) patient mortality?


A retrospective review was performed using the population-based Quebec Congenital Heart Disease (CHD) database. A time-series design, based on Joinpoint and Poisson regression analyses, was used to determine changes in annual referral and patient mortality rates. Both case-control and cohort studies were used to assess the association between specialized ACHD care and all-cause mortality.


There was a significant increase in referral rates to specialized ACHD centers in 1997 (rate ratio [RR], + 7.4%; 95% confidence interval [CI], + 6.6% to + 8.2%). This was in the setting of a recommendation that all ACHD patients be referred to specialized ACHD centers, which was presented in 1996, and published in 1998. A significant reduction in ACHD patient mortality was seen after the year 2000 (RR, -5.0%; 95% CI, -10.8% to -0.8%). In the cohort and case-control studies, specialized ACHD care was independently associated with reduced mortality (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).


Referrals to specialty ACHD centers increased significantly after the introduction of guidelines recommending referral of this patient population for specialty care. Referral to specialized ACHD care was independently associated with a significant mortality reduction.


This landmark study is the first to demonstrate a survival benefit for patients receiving care at specialized ACHD centers. The study provides data to support existing expert opinion recommendations in previously published consensus statements and clinical guidelines. It will be vitally important in the future to identify what factors lead to improved survival of patients at these specialized centers. Candidate factors include patient volume, adherence to guidelines/standards of care, improved procedural/surgical outcomes, and improved access to other subspecialty services. Knowledge of these contributing factors will help to ensure the generalizability of these results to other geographic areas and systems of care.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Congenital Heart Disease, CHD and Pediatrics and Quality Improvement

Keywords: Odds Ratio, Expert Testimony, Case-Control Studies, Heart Defects, Congenital, Referral and Consultation, Reference Standards, Standard of Care, Confidence Intervals, Regression Analysis

< Back to Listings