Guidelines

Guideline for the Management of Adults With Congenital Heart Disease (2018)

The American College of Cardiology and American Heart Association have released new guidelines for treating congenital heart disease. Congenital heart disease occurs when there is a defect in the heart’s structure present at birth.

Thanks to advances in surgery and better medical care, the majority of children born with a heart defect now survive into adulthood. These guidelines review the latest evidence to help guide doctors caring for adults living with congenital heart disease. The publication is a major update of recommendations released in 2008.

Here are highlights from the guidelines that patients should know.

New Classifications

The guidelines set up a new way to group adults with congenital heart disease. There are more than 30 types of heart defects, and some are minor while others are life-threatening. At the same time, many of the 1.4 million adults affected in the United States have no symptoms or complications. All patients are different, so it’s important to capture the severity of their conditions to ensure the best treatment.

Based on the new system, patients will be grouped as class 1, 2 or 3 (ranging from simple to complex), depending on the type of problems with their heart structure.

MORE: View the congenital heart disease anatomy table 

Also, patients will be grouped as stage A, B, C or D based on their overall health and way their heart functions. The new labels will describe patients’ health more accurately and help lead to specific recommendations for each type of patient, experts say. 

Treating Patients Without Symptoms

It can be hard to decide when to treat patients with congenital heart disease who have no symptoms. The guidelines talk about two conditions that don’t always cause symptoms:

  • Secundum atrial septal defect (a hole in the wall of the upper two chambers of the heart)
  • Right ventricular dilatation (enlarged right chamber of the heart)

Experts used evidence to create a guide on how to treat these patients. The chart should help inform decisions, particularly for patients without symptoms who are considering treatment.

Role of Medicine

The guidelines also discuss the role of medication. Some medications have been shown to improve heart health, such as beta blockers and angiotensin-converting enzyme (ACE) inhibitors. These medicines lower blood pressure and reduce risk for heart events. However, most clinical trials for these drugs did not involve patients with congenital heart disease. As a result, deciding whether to use them should be made on a case-by-case basis, depending on the needs of each patient. 

Experts urge more research to explore the role of medication and to help improve the overall outcomes for adults living with congenital heart disease.

Go to www.CardioSmart.org/ACHD to learn more.

Read the full text of the guidelines in the Journal of the American College of Cardiology