In 1996, building off the work of the Canadian Adult Congenial Heart Network when it was established in 1992, the Canadian Cardiac Society brought together a panel of international experts for a conference on adult congenital heart disease (ACHD). Among the proposals resulting from the forum was a recommendation that all ACHD patients be referred to specialized centers for their ongoing care. Despite this recommendation, more than a decade later, less than a third of eligible patients actively receive specialized ACHD care in Canada—a place where health insurance is universal. According to previous research, in the U.S. the proportion seen at specialty centers may be even lower.

In a revealing study published March 3 in Circulation, Ariane Marelli, MD, FACC, McGill Adult Unit for Congenital Heart Disease, McGill University Health Centre, and her team of researchers provided the first empirical evidence demonstrating the benefits of the specialized center model and its ability to prolong mortality.

Utilizing data from the Quebec Congenital heart Disease Database spanning 1990 through 2005, covering several years before and after the publication of guidelines endorsed specialized care for ACHD patients, Marelli et al examined the referral rates to specialized-ACHD centers and ACHD patient morality rates. Their time-series analysis demonstrated that a significant increase in referral rates to specialized-ACHD centers occurred in 1997 after the introduction of the clinical guidelines (Rate Ratio [RR] +7.4 percent; 95 percent CI +6.6 percent to +8.2 percent). By 2000 a noticeable reduction in expected ACHD patient mortality was observed (RR -5.0 percent; 95 percent CI -10.8 percent to -0.8 percent). In further post-hoc cohort and case-control analyses, specialized-ACHD care was independently associated with reduced mortality (Hazard Ratio (HR), 0.78; 95 percent CI, 0.65-0.94) and a reduced odds of death (adjusted odds ratio: 0.82; 95 percent CI 0.08-0.97), respectively.

Ultimately the findings of the study reinforce the ACC/AHA 2008 guidelines for the management of adults with congenital heart disease, the recommendations from the Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease, and the European Society of Cardiology guidelines for the management of grown-up congenital heart disease — that adult patients with moderate or complex congenital heart disease find regular treatment at specialized ACHD centers in collaboration with their other providers, and that cardiologists should be informed of the survival advantage and encouraged to refer the appropriate patients to facilities dedicated to coordinated ACHD care.

"The authors suggest multiple reasons why increasing patient referrals to ACHD centers of expert care might improve outcomes," writes Gary Webb, MD, FACC, the Heart Institute at Cincinnati Children's Hospital Medical Center, University of Cincinnati Medical School, and colleagues, in a responding editorial. "Team members within such a system of health care delivery have a special interest, training and experience with ACHD patients, and as such, may be more likely to utilize practice guidelines, reduce variability in care and maximize the use of medical advances than other practitioners who may be less familiar with this information. They also discuss the ‘practice makes perfect' hypothesis to explain how such higher volume specialized centers allow the accumulation of skills and knowledge, the acquisition of specialized equipment and the development of service models that have been demonstrated in other cardiovascular patient groups to improve outcomes."

"The results from the current study provide legitimacy to prior expert guideline statements that can continue to form the basis for the continued development of systems of ACHD care delivery in the U.S. and elsewhere," concludes Webb. "The belief by specialized ACHD cardiologists that has led them to advance such care is now supported by data. The imperative now becomes the achievement of changes in practice that builds a specialized health care delivery system to engender fiscally responsible and patient-centered quality care in an atmosphere of collaboration and coordination within the multidisciplinary team of specialized health care providers needed for the many and varied needs of ACHD patients. Fortunately, the time to move forward on behalf of these patients is now."

Keywords: Child, Cooperative Behavior, Cardiology, Delivery of Health Care, Referral and Consultation, Health Services Needs and Demand, Insurance, Health, Patient-Centered Care