An update to the first-ever guideline on adult congenital heart disease, released today, provides new recommendations and a more nuanced classification system based on data and expertise accrued in the field over the past decade.
Recommendations for more than two dozen specific lesion types are included in the 2018 American Heart Association/American College of Cardiology Guideline for the Management of Adults With Congenital Heart Disease.
The 172-page document, published online in the Journal of the American College of Cardiology and Circulation, also includes recommendations on general principles ranging from evaluation of suspected disease to palliative care and end-of-life issues.
“The original guidelines, I think everyone would agree, were just a lot more best practice and expert consensus, whereas now we have at least some data to support our recommendations,” said guideline-writing committee vice chair Curt J. Daniels, MD.
Better road map
The document is intended to provide a “better road map” for all providers who will see such patients in their practice, said Dr. Daniels, director of the adult congenital heart disease and pulmonary hypertension program at Ohio State University Heart Center and Nationwide Children’s Hospital, Columbus.
“There are not enough adult congenital heart disease cardiologists and programs in the country to care for the almost 1.5 million adults with congenital heart disease in the United States, so we know these patients are cared for by general cardiologists,” Dr. Daniels said in an interview. “Having some guidelines about when to refer to those patients was a huge part of the purpose of these updated guidelines.”
The revamped classification system underlying the new guidelines seeks to better characterize disease severity based on the complexity of its anatomy and physiology, according to Dr. Daniels.
Previous documents, including the original 2008 AHA/ACC guideline (Circulation. 2008 Nov 7;118:e714-833), focused mainly on anatomic classifications to rank severity, he said.
“Traditionally, we’ve based the severity of congenital heart disease based on the complexity of anatomy they were born with, but that goes only so far,” Dr. Daniels said in an interview.
More than just anatomy
In the new system, anatomy is classified as simple (I, e.g., isolated small atrial septal defect), moderately complex (II, e.g., coarctation of the aorta), or greatly complex (III, e.g., cyanotic congenital heart defect), while physiologic stages range from A to D, increasing along with the severity of physiologic variables such as New York Heart Association functional class; exercise capacity; aortic enlargement; arrhythmias; renal, hepatic, or pulmonary function; and venal or arterial stenosis.
A normotensive patient with repaired coarctation of the aorta would be classified as IIA if she had normal end-organ function and exercise capacity, whereas a similar patient with an ascending aortic diameter of 4.0 cm would be classified as IIB, and with the addition of moderate aortic stenosis, would be classified as IIIC, according to an example provided in the guidelines.
Congenital heart disease specialist Robert “Jake” Jaquiss, MD, said in an interview that consideration of physiology alongside anatomy is one of the most important features of the new guidelines.
“This is a way of thinking about patients that involves not just their anatomy, but also considering a variety of domains in which there may be physiologic dysfunction that can modify the underlying anatomy,” said Dr. Jaquiss, chief of pediatric and congenital heart surgery at Children’s Medical Center/University of Texas Southwestern Medical Center, Dallas. “I think that is a more clinically relevant way to think about patients and patient evaluation management, and I commend the authors for that focus.”
For example, one patient who has undergone a Fontan procedure may be fully functional, whereas another with nearly identical anatomy may be burdened by arrhythmias, fluid retention, and impaired exercise function. “If we don’t grasp the physiologic difference, we treat the two patients the same way, which is obviously inappropriate,” Dr. Jaquiss said.