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Congenital Heart Disease Patients Require Lifetime Care and Management

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 were not expected to survive to adulthood. Now, with advances in surgery, medications and heart assist devices, long-term survival rate is almost 90 percent and many are living well into adulthood.

"Unfortunately, many congenital heart disease patients enter adulthood with complex complications that have developed over the years", says Shane Tsai, MD, cardiologist at Nebraska Medicine.

Shane Tsai, MD
Shane Tsai, MD

"In addition, 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. There also are a lot of myths and misconceptions about ACHD that can contribute to the mismanagement of the condition."

The first of these is the belief that ACHD patients who have received treatment do not require close cardiovascular follow-up. "On the contrary, many adults remain at risk for long-term complications as a result of palliative repairs in childhood, including heart failure and arrhythmia", says Dr. Tsai. "In fact, the life time risk for atrial fibrillation and flutter is greater than 50 percent. In addition, 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, he says.

Another misconception is the belief that patients with repaired congenital heart disease require no restrictions in activities. While most patients can lead relatively normal lives with few restrictions in activities of daily living, some individuals are at increased risk for heart failure symptoms such as shortness of breath with activities, he says. 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. If complications are not an issue, aerobic exercise such as walking, running, swimming and biking are encouraged.

It is often thought that women with ACHD should not be allowed to become pregnant. This is simply not the case. 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 the preferred method of delivery," says Dr. Tsai. However, there are some women who should be discouraged from becoming pregnant, including those with cyanosis and pulmonary hypertension.

Another myth is that Eisenmenger Syndrome is an untreatable condition. Unfortunately, many patients remain undertreated, even though quality of life can be significantly improved with the appropriate medications, notes Dr. Tsai. Once fatal, pulmonary hypertension can now be treated with several different types of medications, including phosphodiesterase inhibitors, endothelin receptor blockers and prostacyclin.

It is often believed that all patients should receive antimicrobial prophylaxis. However, most patients today do not require antibiotics to prevent bacterial endocarditis, he says. Those that do include patients with artificial heart valves or unrepaired cyanotic (blue) lesions.

Dr. Tsai is the only cardiologist in Nebraska board certified in both pediatric and adult cardiology. He will be joined later this summer by Jonathan Cramer, MD, a pediatric cardiologist, who also has advanced subspecialty training in adult congenital heart disease.

"We often say, 'It's not what you don't know, it's what you don't know that you don't know that causes problems,'" says Dr. Tsai.

To learn more, make a referral or connect with Dr. Tsai or a member of the Heart Center team, call, 877-647-7497 or visit at