New Guidelines for the Management of Adult Congenital Heart Disease

The American College of Cardiology and the American Heart Association have released the first full revision of their guidelines in a decade, including a new classification system.

A decade after the American College of Cardiology and the American Heart Association released its first guidelines for the management of adult congenital heart disease (ACHD), the two groups have produced a full revision – including a new way of classifying patients by CHD anatomy and physiological stage.

Curt Daniels, MD, member of The Heart Center at Nationwide Children’s Hospital and director of the Adolescent and Adult Congenital Heart Disease Program, is the vice chair of the guideline writing committee.

“Because of the success of pediatric cardiology and heart surgery, there are now more adults than children with congenital heart disease. It is imperative we provide high quality data-driven specialized care for these unique adult patients and the new ACHD care guidelines provides a means for evaluation and treatment based upon current research and expert opinion.” says Dr. Daniels.

Perhaps most widely applicable for ACHD providers is the new classification system that takes into account both anatomic complexity and physiological status. Anatomy is stratified from I (simple) to III (great complexity). Physiological status ranges from A (e.g: no hemodynamic or anatomic sequelae, etc.) to D (e.g. severe aortic enlargement, severe hypoxemia, etc).

The 2008 guidelines focused on anatomy to rank disease severity; the new guidelines recognize that patients with the same underlying anatomy may have different consequences of that anatomy and repairs they have undergone. The new classifications may help providers determine how closely some patients need to be followed.

For example, one patient with an atrial septal defect may be considered IIC, while another with the same anatomy may be considered IID with less stable physiology. The IID patient should have more regular follow-up with a specialist and more frequent exercise tests.  

(Collateral material for better understanding the guidelines, including a more detailed explanation of the case above, is available at the Journal of The American College of Cardiology). 

The 170-page guidelines span the breadth of ACHD knowledge, from the definition of terms to management practices for specific lesions. The guidelines also emphasize the importance of treatment as part of an integrated, collaborative ACHD program.

Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Journal of The American College of Cardiology . 2018 Aug 10. [Epub ahead of print]