Congenital Heart Disease

For some, heart disease develops over time. For others, it is a part of life from the beginning. Congenital heart disease (CHD) refers to a heart problem that occurs when the heart does not form properly during a baby’s development during pregnancy. This leads to heart defects that are present at birth. We offer comprehensive, lifelong care for congenital heart disease. We care for people who have undergone corrective surgeries in childhood for their CHD and those who first discover their CHD when they are adults.

Whether you are an adult with congenital heart disease or caring for a child who has been diagnosed, The University of Kansas Health System offers complete care to support you.

Congenital heart disease happens during pregnancy when the heart does not form properly, usually within the first 8 weeks of a baby's development. Almost 1 in 100 babies is born with CHD. The condition is present at birth, but it may not be detected right away. Due to advances in medical and surgical care, most people born with CHD now survive into adulthood.

The only way to know for sure if you have congenital heart disease is to be evaluated by a cardiologist. Adults with CHD continue to need care throughout their lives and are best treated by physicians who understand their complex and unique needs. The University of Kansas Health System adult congenital heart program makes a lifelong commitment to its patients with CHD. In partnership with Children’s Mercy Hospital, we offer a full scope of inpatient and outpatient clinical services to adults with CHD.

Congenital heart disease can take many forms, from simple to complex. Some of the most common types of congenital heart disease include:

• Artery and vessel problems
• Chamber problems
• Holes in the heart
• Valve problems

Congenital heart defects, or problems with the heart's structure, are the most common type of congenital heart disease. Our congenital heart disease care team helps manage repaired or unrepaired congenital heart defects in adolescence and adulthood.

A variety of syndromes are commonly associated with CHD in adolescence and adulthood, including:

• Down syndrome
• Kabuki syndrome
• Klinefelter syndrome
• Noonan syndrome
• Turner syndrome
• Williams syndrome

Congenital heart disease symptoms will vary from person to person, depending on the number, type and severity of defects. For some, no symptoms are present. Others may develop symptoms later in life, while some will have symptoms at birth or in early childhood.

Common signs of congenital heart disease include:

• Abnormal heart rhythm
• Cyanosis (bluish coloring of the skin, lips and fingernails)
• Fatigue that is brought on easily
• Heart murmur
• Poor blood circulation
• Rapid breathing
• Shortness of breath
• Slow weight gain or growth (in children)

Congenital heart disease can be diagnosed during pregnancy, in infancy or later in life. There are a number of cardiac diagnostic tools that can be used to identify a congenital heart problem. Once an accurate diagnosis is made, your cardiologist may or may not recommend treatment, depending on the severity of your condition.

Some people can go without treatment for their congenital heart disease symptoms and live normal lives with no long-term effects to their heart health. Others with more serious conditions may need one or more treatment options right away:

• Artificial heart valves
• Heart pacing devices
• Heart surgery
• Heart transplant
• Lifestyle changes
• Medications
• Minimally invasive procedures

Our congenital heart disease care team also offers several programs specific to people with CHD.

• Transition of care: The University of Kansas Health System partners with Children’s Mercy Hospital to provide transition care to people who are moving from pediatric to adult CHD care. The transition telehealth visit includes transition coordinators from the health system and Children’s Mercy who introduce patients to the adult CHD program. They learn about the maintenance their condition may require as they move into adulthood and schedule their first appointment with the health system.
• Single ventricle/Fontan surveillance: This program offers complete evaluations, monitoring and long-term care for adolescents and adults with single ventricle defects after the Fontan procedure. We provide a comprehensive, multidisciplinary program with adult CHD specialists working closely with hepatologists, nutritionists, exercise physiologists and social workers, to ensure patients receive the most advanced, integrated and comprehensive care.
• Cardiovascular genetics counseling: We recognize the critical importance of understanding the genetic factors that can influence your cardiac condition. That’s why we offer specialized genetic counseling services as part of our comprehensive care. Our expert genetic counselors work closely with patients and their families to help them make informed decisions about their health and provide support for managing hereditary risk.
• Adult CHD/electrophysiology : Our adult CHD/electrophysiology program offers combined visits with an adult CHD specialist and an adult CHD-focused electrophysiologist for our patients with complex heart rhythm needs. This allows for maximum collaboration between experts with complementary skill sets.
• Pulmonary valve care: Deciding when, how and where to get your pulmonary valve replaced can be a daunting decision. That’s why we offer a dedicated pulmonary valve program, where you’ll meet with your adult CHD interventional and surgical teams, allowing you to make the best decision for your situation. We offer leading-edge surgical and transcatheter pulmonary valve replacement techniques, so you can feel confident you’re getting the best option for you.

Congenital heart disease can affect your choice of career, decisions on pregnancy, physical activity and life plans. The University of Kansas Health System can provide you with information about maintaining your health, reducing your risk for heart disease and making appropriate lifestyle choices for your condition.

Our specialists include dedicated physicians with deep experience in congenital heart disease. Our nurses are also dedicated to this area of specialty and have the expertise to understand your particular condition and concerns.

Because we're part of an academic medical center, you'll benefit from a dedicated team of experts. Your care team may include cardiologists, surgeons, nurses and others. You will see both your cardiologist and one of our experienced clinical nurse specialists at every clinic visit. Nurses are on call any time to address your concerns.

Your cardiologist can consult with other leading specialists who are located across the hall instead of across town. We also have regular conferences with Children's Mercy Hospital to coordinate care for younger patients and those in transition to adulthood.

Our hospital also benefits from a close relationship with specialists in pulmonary vascular disease. Our vascular specialists offer comprehensive medical services for pulmonary hypertension, a problem often associated with adult congenital heart disease.