Adult Congenital Heart Disease Myths

Note from ACHA: The guest post below by Dr. Tsai, an ACHA medical professional member, was originally written for The Nebraska Medical Center Blog. ACHA has been granted permission to republish the post.

Today there are more adults than children in the United States with congenital heart disease. Just a few decades ago, many infants born with heart defects would not be expected to survive to adulthood. Now the long term survival rate is almost 90 percent. Unfortunately, there are few specialists trained to care for adults with congenital heart disease (ACHD) and many survivors fail to follow up with any cardiovascular specialist.

In my line of work, we like to say that when it relates to congenital heart disease, it’s not what you don’t know. It’s what you don’t know that you don’t know. Confused? There are a lot of myths and misconceptions about ACHD that contribute to the mismanagement of the condition.

Five common ACHD myths:

Myth: ACHD patients who have been fixed do not require close cardiovascular follow-up.

Avoid use of the “F-word” as many adults remain at risk for long term complications as a result of palliative repairs in childhood, including heart failure and arrhythmia. In fact, the life time risk for atrial fibrillation and flutter is greater than 50 percent. Further, some groups are at 25 to 100 times increased risk for sudden cardiac death. While surgical and transcatheter procedures can correct and improve abnormalities, practitioners often wait too long to intervene, and the development of symptoms may actually herald irreversible hemodynamic compromise.

Myth: Patients with repaired congenital heart disease have no restrictions in activities.

Most patients can lead relatively normal lives with few restrictions in activities of daily living. Aerobic exercise is often encouraged, including walking, running, swimming and biking. Nevertheless, some individuals are at increased risk for heart failure symptoms such as shortness of breath with activities. Other patients have abnormal aortic tissue and are at increased risk for aneurysm and spontaneous dissection. These individuals should avoid isometric activities, including heavy lifting and resistance training.

Myth: Women with ACHD should not be allowed to become pregnant.

Many women with ACHD can deliver a healthy baby successfully. Collaboration between cardiovascular specialists and high-risk obstetrics is recommended to navigate the increased risk for heart failure and arrhythmia, as well as preferred method of delivery. However, there are some women who should be discouraged from becoming pregnant, including those with cyanosis and pulmonary hypertension.

Myth: Eisenmenger syndrome is an untreatable condition.

Once fatal, pulmonary hypertension can now be treated with several different types of medications, including phosphodiesterase inhibitors, endothelin receptor blockers, and prostacyclin. However, although quality of life can be significantly improved, many patients remain undertreated.

Myth: All patients should receive antimicrobial prophylaxis.

Most patients today do not require antibiotics to prevent bacterial endocarditis. Those that do include patients with artificial heart valves or unrepaired cyanotic (blue) lesions.