The Unique Adult Congenital Heart Patient

Most people do not understand what I mean when I tell them I’m an adult congenital. I am part of a growing population of more than one million adults who live with a congenital heart defect (CHD). Thanks to the advances made in congenital cardiac surgery, many children are growing into adulthood.

Like most, after surgery, I thought I was “fixed” and would never have to worry about with my heart. No one told me or my parents about issues that could arise later in life. There wasn’t enough knowledge about long-term outcomes at the time of my heart repair to know with certainty what issues could arise.

I was born with a CHD known as complete atrioventricular (AV) canal defect. My repair in 1972 was successful, but I will always live with a CHD. For most of life, the only reminder of my CHD was the railroad-like scar on my chest. In the winter of 1999, I started to notice sudden episodes of rapid heartbeats and a very uncomfortable feeling in my neck and chest. Needless to say, these episodes concerned me. A friend of mine, a pediatric cardiologist, recommended that I go see an electrophysiologist—a cardiologist who specializes in the heart’s electrical system.

When I walked into the waiting room, it could have been the casting call for the sequel to the movie Cocoon. I was the youngest person in the room by about 30 years. The doctor did not conduct any diagnostic tests. He talked a lot about his education and seemed to be more interested in impressing me with his credentials. Once he listened to my heart, he asked, “What side of the heart is that AV canal located?”

I was shocked at this question. Even I knew the answer had nothing to do with either side of my heart. This was the second clue that I was at the wrong place for this condition. He encouraged me to allow him to do an EP (electrophysiology) study. I refused, and remember leaving the clinic as soon as possible.

Under the advice of my childhood pediatric cardiologist, I got a second opinion. Once I met with my new adult congenital cardiologist and his team, they ordered a cardiac event monitor, so they could help diagnose the arrhythmia. The results indicated I was experiencing an arrhythmia known as supraventricular tachycardia (SVT).

The good news is that my SVT can be managed by medication and, if needed, cardiac ablation. More importantly, the ACHD specialist knew exactly what to do for me. SVTs are common with patients with AV canal defect, since the physiology of the heart’s electrical system is close to the area of repair. Even with the best surgical repair, SVTs often occur and can be managed. Even though they are not lethal, they are serious and need specialized attention. The first doctor may have been familiar with SVTs, but not in the adult congenital patient. The congenital heart is unique, and even though repaired, is not a normal heart.

Without the specialized care of an adult congenital heart program, the first doctor could have caused me more harm. Today my SVTs are being managed with medication. There is comfort in knowing that more options are available, with specialists who understand the complexities of the congenital heart in the adult patient. My advice to anyone living with a CHD is to seek a specialized adult congenital program, even if there are no issues. Adults need regular heart checks to make sure their repairs are sustaining as they grow.