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The Ins and Outs of Adult Congenital Heart Disease

Portrait of Yuli Kim, MD
Dr. Yuli Kim

40,000. That’s how many new patients are diagnosed with congenital heart disease (CHD) each year. From the moment a diagnosis is made, usually at a prenatal ultrasound, families question what this will mean for the present and the future. The good news is that specialized care is available to CHD patients, letting them live long, healthy lives. The field of adult congenital heart disease (ACHD) has grown exponentially, and finding programs that are able to follow a CHD patient throughout their childhood and as they transition into adulthood is incredibly important.

Dr. Yuli Kim is the director of the Philadelphia Adult Congenital Heart Center, a joint program between two premiere institutions—The Children's Hospital of Philadelphia and Penn Medicine—that provides this continuity of care. Here she answers some common questions about adult congenital heart disease.

Adult Congenital Heart Disease Q&A

What is adult congenital heart disease?

The field of adult congenital heart disease is a relatively new specialty, so let’s break it down a bit. “Congenital heart disease” means you are born with a defect of the heart. No two patients are the same: the defect can range from simple to complex and everywhere in between.

An example of a simple form of congenital heart disease would be a ventricular or atrial septal defect, more commonly known as a hole in the heart. A patient with Tetralogy of Fallot, a combination of four defects that affect the overall structure of the heart, would be considered moderate or complex. These are the patients that should be followed on a regular basis. In the past, when these patients had their corrective surgery, they were told to go and live a normal life because they were "fixed”. We know now that that it’s not that simple. These patients require lifelong congenital care, ideally in a center that specializes in adult congenital heart disease.

Why is ACHD just being talked about now?

ACHD was not formally recognized until 1990, so the field is relatively new. In the past, babies who were born with congenital heart disease often did not survive infancy or childhood. With advances in surgery, medicine and ICU care, we now expect that over 95% of babies born with congenital heart disease survive to adulthood, which is why we have this new specialty that really wasn't around a few decades ago.

What are the types of patients you see, and what kind of treatment are they getting?

Most of our patients have undergone some type of corrective heart surgery in childhood. There are, of course, adults who are referred to our clinics who were recently diagnosed with congenital heart disease, but these are usually of milder forms.1,000,000 adults are living with CHD in the United States

New, innovative techniques have been developed that allow for minimally invasive heart valve repairs and replacements. This approach saves our patients who may have already had open heart surgery from undergoing another open heart surgery.

Are there any other common conditions you treat?

Another common condition we treat is pregnancy. It is really gratifying being able to counsel women who want to get pregnant and have congenital heart disease. These patients may have been told in the past that they are not able to have children or it's not safe for them to have children. We know now that many of these patients can safely have children and go through pregnancy as long as they are followed in a high-risk adult congenital heart center that partners with a maternal fetal medicine specialty clinic. This is a patient population that we specialize in as adult congenital cardiologists. It's very gratifying for the patients or families to know that they can have a safe pregnancy as long as they are supervised properly.

What is one thing you think everyone should know about ACHD?

We do believe that there are patients out there that we call “lost to care”. There was a point in time when patients were told that they did not need to come back, that they were "fixed”. This has actually resulted in a lost patient population of adults with congenital heart disease who are out there who might not even realize that they need adult congenital heart disease care.

In my opinion, there are two patient populations that get lost. The first being the older ones who were told not to come back because they were “fixed”. The second patient population are those at a vulnerable age. This is the 18 to 21-year-old age bracket and they’re just starting to become independent – they're going off to school, they feel invincible, they feel good, they feel healthy, they don't need to go to a doctor – and they do not go back.

I think it's really important that these patients get back into congenital care because it's really going to be the best for them in the long-term.

Learn more about the Philadelphia Adult Congenital Heart Center or make an appointment today.

About this Blog

The Penn Heart and Vascular blog provides the latest information on heart disease prevention, nutrition and breakthroughs in cardiovascular care.

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