ACC/AHA Release Updated Guideline for Treatment of Adult Congenital Heart Disease

August 20, 2018—The American College of Cardiology (ACC) and the American Heart Association (AHA) recently announced the release of an updated guideline for the management of adult congenital heart disease (ACHD) patients.

According to a joint press release by the ACC/AHA, the prevalence of ACHD is growing due to the success of treating these patients during childhood. Survival to age 18 years is now expected in 90% of children diagnosed with severe congenital heart disease.

Karen K. Stout, MD, Chair of the writing committee for the guideline, commented in the societies’ press release, “Although the prevalence of ACHD is increasing, the population of patients with a given congenital abnormality or specific repair may be relatively small, which can make accruing evidence to guide treatment challenging.” Dr. Stout is Professor of Medicine and Pediatrics in Cardiology at the University of Washington in Seattle, Washington.

The guideline update is a full revision of the original guideline published in 2008. The AHA/ACC press release noted that this revised version incorporates new data from the growing body of high-quality data from the ACHD experience to guide the care of this relatively new population.

Additions to the guideline include a new classification system for ACHD patients that retains the traditional classification based on the structural complexity of the disease while considering the patient’s functional status and other factors. The new system provides the basis for making lesion-specific recommendations for interval clinical follow-up and testing modalities such as electrocardiography, transthoracic echocardiography, cardiovascular magnetic resonance, and exercise testing.

These recommendations are intended to provide guidance to a wide variety of providers caring for patients with ACHD, including general, pediatric, and ACHD cardiologists, as well as surgeons, primary care providers, and other health care providers.

“As new data become available, we expect changes to the relative weights attributed to the existing components of the classification system and perhaps new components, resulting in a system that ever more precisely tracks the overall severity of the patient’s disease and need for more or less intensive follow-up and management,” Dr. Stout said.

The “2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease” is available online in both Journal of the American College of Cardiology and Circulation.