New Guidelines Released on the Management of Congenital Heart Disease in Adults

On Friday, November 7, the American College of Cardiology and the American Heart Association announced the joint release of a comprehensive set of new practice guidelines on the management of adults with congenital heart disease (CHD). The American Society of Echocardiography, Canadian Cardiovascular Society, Heart Rhythm Society, International Society for Adult Congenital Cardiac Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons also collaborated in the development of the guidelines.

According to a news release from the American College of Cardiology (ACC), the guidelines are “the first of their kind in the United States” and will appear in the December 2, 2008, issue of the Journal of the American College of Cardiology (JACC) and the December 2, 2008, issue of Circulation.

Because of advances in the treatment and repair of congenital pediatric heart defects, there is a common misconception that children who have undergone surgical procedures to correct a defect are completely cured and do not require follow-up treatment for CHD as they age. Not so, according to Carole A. Warnes, MD, co-chair of the guidelines writing committee, professor of medicine at the Mayo Clinic in Rochester, MN, and director of Mayo’s adult congenital heart disease clinic. “Some patients leave their pediatric cardiologist and think they don’t need to see a physician anymore, and others are seen by a cardiologist with no training in congenital heart disease. We really need to focus on how to better care for this population,” Warnes said in the ACC news release announcing the new guidelines.

The reality is that “nearly all patients need follow-up throughout adult life. Some will eventually need additional surgery to correct complications that arise over time. Most will need guidance on how their heart condition affects the important milestones of adulthood, such as employment, pregnancy and physical activity,” according to the ACC news release, which also provides examples of complications that could arise in the absence of adequate follow-care: “Worsening of a leaky heart valve may be overlooked until the patient is in heart failure, making surgery highly risky or even impossible. A young woman may be told she cannot have children, when in fact, pregnancy might be possible if managed at a center with expertise in congenital heart disease. A seemingly routine appendectomy may turn into a life-threatening crisis.”

Accordingly, the guidelines “provide comprehensive information on the clinical features, diagnosis, and medical, surgical and interventional therapy of a range of congenital heart defects. They also point out common problems and pitfalls, highlight key issues to look for during follow-up, and make recommendations on genetic testing, pregnancy, contraception and physical activity.”

The full text of the guidelines was also posted on the ACC website. An executive summary of the new guidelines is also available.