Heart Valve Replacement and Repair

Heart valve disease (HVD), or heart valve malformation, is a type of congenital or acquired heart defect that affects one or more of the heart’s valves. A normal heart has four heart valves that open to let blood flow forward and then close tightly to prevent blood from flowing back into the heart. As a baby develops, the valves of the heart can be too small, abnormally shaped, missing altogether or they may not close all the way, allowing blood to flow backward into the heart. If left untreated, heart valve disease, also known as a leaky heart valve, can result in other complications, including arrhythmias and heart failure.

Types of Heart Valve Disease

• Atrioventricular canal defects: a congenital heart defect in which there is a large hole in the center of the heart that affects all four chambers, which are normally separated, as well as heart valves.
• Double outlet right ventricle: a congenital heart defect in which both the pulmonary artery and aorta connect to the right ventricle, causing blood with too little oxygen to be pumped to the body.
• Ebstein’s anomaly: a congenital heart defect that affects the tricuspid valve on the right side of the heart. The valve does not close properly and, as a result, blood can leak back into the heart, causing the upper right chamber to become larger than normal. It often occurs with an atrial septal defect (ASD), which is a hole in the wall between the two upper chambers of the heart.
• Heterotaxy syndrome: a rare birth defect in which the heart or other organs that normally develop on the left or right side of the body are switched.
• Pulmonary atresia: a single ventricle congenital heart defect in which the pulmonary valve does not develop normally or is absent. This prevents blood from flowing from the heart to the lungs, resulting in other complications.
• Pulmonary stenosis: a condition in which there is narrowing at one or more points from the heart’s right ventricle to the lungs. It can include the narrowing of the valve, thickening of the muscle or narrowing of the pulmonary artery above the valve.
• Pulmonary valve regurgitation: a condition of the heart in which the pulmonary valve does not close completely, which allows blood to flow back into the right ventricle.
• Tricuspid atresia: a congenital heart defect in which the tricuspid valve is missing, so blood cannot flow normally from the right atrium to the right ventricle. This causes the right side of the heart to be underdeveloped.
• Tricuspid valve regurgitation: a condition of the heart in which the tricuspid valve does not close completely, allowing blood to flow backward into the right atrium.
• Truncal valve disease: a congenital heart defect in which there is one blood vessel that arises from the heart instead of the aorta and pulmonary artery.

Children’s is the only nationally ranked pediatric cardiology and heart surgery program in Georgia. Our skilled team of providers specialize not only in the treatment of heart valve disease—they also specialize in meeting the unique healthcare needs of infants, children and teens. This is why so many Georgia families turn to us when a child is diagnosed with a complex heart condition. Our multidisciplinary Heart Center team takes a personalized approach to treatment, utilizing advanced imaging, nonsurgical care and surgical procedures to help give your child the best possible chance at a healthy future.

Why Choose the Children’s Heart Center for Heart Valve Repair?

If your child has been diagnosed with heart valve disease, you can count on us to provide them with the comprehensive, multidisciplinary care they deserve.

• Heart valve treatment expertise: As one of the highest volume programs in the country, we perform hundreds of heart surgeries each year, including the Ross procedure. We have outcomes and survival rates that regularly exceed the national average despite performing some of the most complex procedures. 
• Accurate surgical planning: Your child's surgery will be designed based on their unique anatomy, diagnosis and needs. Our 3D structural imaging specialists create detailed models of your child's heart to guide surgical planning and help us tailor these complex treatments. 
• Raising the bar for treatment with research: Our team is actively researching the causes of heart valve disease and is dedicated to innovating advanced techniques and new approaches to care that will lead to better outcomes for patients. We are developing new treatments for heart valve disease, including new devices and valve sparing root procedures for connective tissue disorders. Additionally, we are developing transcatheter procedures to repair heart valves, which may allow some patients to be treated without the need for surgery.

Heart valve disease can be diagnosed either before or after a baby is born. It is commonly diagnosed during a pregnancy ultrasound, which shows images of a developing fetus. If doctors suspect that your growing baby has heart valve disease, they will refer you to a fetal cardiologist. This specialist will confirm a diagnosis using a test called a fetal echocardiogram, and they will then work with you to create a treatment plan.

Some children may be diagnosed with heart valve disease later in life. Your child’s pediatrician may hear a heart murmur, which is an unusual sound caused by blood flowing through the heart. In some cases, a heart murmur can be a sign of a heart valve condition.

Other symptoms of heart valve disease may include:

• Chest pain
• Irregular heartbeat
• Dizziness or fainting (syncope)
• Swelling around the eyes, legs or abdomen
• Shortness of breath

If your child’s pediatrician suspects that your child has heart valve disease, they may refer your child to a pediatric cardiologist for an evaluation, which may include a review your child’s medical history and family history, as well as imaging tests, such as:

• CT scan: Also known as a computed tomography scan, a CT scan uses X-ray technology to create cross-sectional, detailed images of your child’s heart, heart valves and blood vessels.
• Cardiac MRI: Magnetic resonance imaging, or MRI, is an imaging test that uses a large magnet, radio waves and a computer to take high-quality pictures of your child’s heart, valves and blood vessels.
• Echocardiogram: This test uses sound waves to create a detailed picture of the heart, blood vessels and heart valves.
• 3D Echocardiogram: Using sound waves, this test creates multiple detailed pictures of the heart, blood vessels and heart valves.

Your child’s pediatric cardiologist will carefully discuss treatment options with you and select one that is best for your child.

Nonsurgical and Minimally Invasive Heart Valve Treatment

Heart valve disease treatment without surgery is possible for some patients.

• Watchful waiting: For patients with a mild case of heart valve disease that is not causing symptoms, your doctor may recommend monitoring the condition over time to look for any changes. Your child will have an echocardiogram each year to look at the heart and its valves to make sure the disease is not progressing.
• Transcatheter valve replacement: This is a minimally invasive procedure in which a heart valve is replaced using a catheter, a thin, flexible tube that is inserted into a large vein or artery. An artificial heart valve will be guided to the diseased heart valve and put in place without the need for surgery.
• Balloon aortic valvuloplasty: This is a minimally invasive heart valve repair procedure in which a balloon is guided by a catheter to a narrowed valve. The balloon will then be inflated, which widens the valve and keeps it from narrowing.

Cardiothoracic Surgery

If your child, teen or young adult needs heart valve surgery, our doctors will provide the latest in advanced procedures. Each surgery is carefully planned using a detailed, 3D model of your child’s heart.

• Ross procedure: an open heart surgery in which a child’s narrowed or leaky aortic valve is replaced with the child’s pulmonary valve. The pulmonary valve is then replaced with one from a donor. This allows blood to flow normally through the heart and to the lungs.
• Cone procedure: an open heart surgery to repair a tricuspid aortic valve in patients with Ebstein’s anomaly. During the procedure, the doctor separates the tricuspid valve leaflets from the heart muscle, reshapes them into a cone shape and reattaches the cone to your child’s heart valve. Many patients may need a second procedure as an adult to replace the cone with a prosthetic or donor valve.
• Hybrid surgery: a procedure that combines traditional surgery with interventional catheterization techniques in a single, tailored approach.
• Partial heart transplant: also known as a living root replacement, this is a surgical procedure that aims to fix heart valve problems by replacing your child’s diseased valves with ones from a donor heart instead of replacing the whole heart.
• Valve-sparing repair of tetralogy of Fallot: a procedure to surgically repair tetralogy of Fallot in which the surgeon widens the pulmonary valve instead of replacing it with a prosthetic valve.

Prognosis After Heart Valve Repair

Children who undergo heart valve replacement or repair are often able to live normal lives after heart surgery. Most patients will need to continue to see a pediatric cardiologist each year to monitor their heart health. At the Children’s Heart Center, we follow patients until 21 years of age before referring them to an adult cardiologist. Some patients may require additional surgeries into adulthood as the heart changes and grows over time.

Cardiothoracic Surgeons

• Paul Chai, MD, Chief, Cardiothoracic Surgery; Co-Chief, Children’s Heart Center
• Subhadra Shashidharan, MD, Associate Chief, Cardiothoracic Surgery
• Mohan John, MD
• Josh Rosenblum, MD

Cardiologists 

• William Mahle, MD, Chief, Cardiology; Co-Chief, Children’s Heart Center
• Glen Iannucci, MD
• Dennis Kim, MD
• James Kuo, MD
• Allen Ligon, MD
• Fred Rodriguez, MD
• Denver Sallee, MD
• Dongngan Truong, MD
• Hunter Wilson, MD

Arthur M. Blank Hospital
2220 North Druid Hills Road NE
Atlanta, GA 30329