The ACC and the American Heart Association have released an updated guideline for the management of adult congenital heart disease (ACHD) patients. The 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease, published Aug. 16 in the Journal of the American College of Cardiology, replaces the original guideline published in 2008, and incorporates new data and growing ACHD expertise.

Specifically, the guideline presents a new classification system for ACHD patients that retains the traditional classification based on the structural complexity of the disease while taking into account the patient's functional status and other factors, including the presence of cardiovascular and non-cardiovascular problems. The new system is intended to capture the complexity of ACHD in terms of both anatomy and physiology, rather than anatomy alone. It also provides the basis for making lesion-specific recommendations for interval clinical follow-up and testing modalities such as electrocardiograms, transthoracic echocardiography, cardiovascular magnetic resonance and exercise testing. The need for specialized care is also highlighted throughout the guideline, with authors noting that patients with significant ACHD who are cared for in ACHD centers have better outcomes than those cared for in centers without ACHD expertise.

Overall, the new 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. "Patients with ACHD are a heterogeneous population," said Karen K. Stout, MD, FACC, chair of the writing committee for the guideline. "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. 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."

Keywords: Aortic Diseases, Arrhythmias, Cardiac, Cardiac Catheterization, Cardiac Imaging Techniques, Multidetector Computed Tomography, Cardiac Imaging Techniques, Magnetic Resonance Imaging, Cardiovascular Surgical Procedures, Delivery of Health Care, Delivery of Health Care, Integrated, Organization and Administration, Heart Defects, Congenital, Cyanosis, Echocardiography, Electrocardiography, Endocarditis, Exercise, Exercise Test, Functional Residual Capacity, Genetic Diseases, Inborn, Heart Failure, Heart Transplantation, Diagnostic Imaging, Palliative Care, Motor Activity, Pregnancy, Hypertension, Pulmonary, Sports, Surgical Procedures, Operative, Patient Care Team, Heart Valve Diseases, Child, Follow-Up Studies, Surgeons, Magnetic Resonance Spectroscopy, Primary Health Care

< Back to Listings