What is Congenital
Heart Disease?
Congenital Heart Disease, also known as CHD, is one of the most common birth defects in the United States. CHD is a defect or abnormality of the heart that is usually present from birth. It results when the heart or blood vessels near the heart do not develop normally before birth.
Approximately 40,000 babies are born each year with CHD. About 1 in 4 babies born with a heart defect has a critical CHD (also known as critical congenital heart disease). Babies with a critical CHD usually need surgery or other procedures in the first year of life.
What is the Pulmonary Valve?
The pulmonary valve (sometimes referred to as the pulmonic valve) is a one-way valve that lies between the right ventricle and the pulmonary artery and has three cusps. It controls blood flow from the right lower chamber (right ventricle) to the pulmonary artery and sends blood to the lungs to pick up oxygen. People with a congenital heart defect may need to have their pulmonic valve replaced due to disease.
What is Right Ventricular Outflow Tract (RVOT)?
The right ventricular outflow tract, also known as RVOT, is a tunnel-like structure in the heart that holds the pulmonary valve. When working properly, blood will pass from the heart through the RVOT and pulmonary valve to the pulmonary artery and out to the lungs.
For people whose RVOT begins to fail, they may need to have their native RVOT replaced with a conduit that may or may not include a pulmonary valve.
Types of Congenital Heart Defects That Can Affect the RVOT or Pulmonary Valve
In children, adolescents and adults, congenital heart defects may manifest in a variety of different ways. Some are serious and may need several surgeries and treatments over the course of a person’s lifetime. The list shown on this page is not all-inclusive, yet highlights some of the CHDs common to the right ventricular outflow tract and/or pulmonary heart valve:
A rare condition in which four defects occur together at birth. The defects include a hole between the left and right ventricles of the heart, a narrowed path between the heart and lungs, a thicker pumping chamber, and an aorta connected closer to the right side of the heart than normal.
Pulmonary valve stenosis is a condition in which a deformity on or near your pulmonary valve narrows the pulmonary valve opening and slows the blood flow.
Septal defects are due to an abnormal connection between the chambers of the heart ventricles. It is shown as an opening or hole in the heart that forms between the heart’s chambers (ventricles). This allows oxygen-rich and oxygen-poor blood to mix.
The Ross Procedure is a surgical option for children and young adults with diseased aortic valves or aortic valve failure. In this procedure, the diseased aortic valve (which is either leaking or stenotic) is removed, and the patient’s own pulmonary valve is substituted in its place.
The pulmonary valve that lets blood out of the heart to go to your lungs doesn’t form correctly. Instead of opening and closing to allow blood to travel from the heart to the lungs, a solid sheet of tissue forms. So blood can’t travel by its normal route to pick up oxygen from the lungs.
A serious but rare condition in which the two main arteries leaving the heart are reversed.
This rare defect is when a person has only one large artery instead of the normal two separate arteries to carry blood to the lungs and body. With only one artery, there is no specific path to the lungs for oxygen before returning to the heart to deliver oxygen to the body.
The aorta is incorrectly connected to the right ventricle (RV, the chamber of the heart that pumps oxygen-poor blood to the lungs) instead of to the left ventricle (LV, the chamber that normally pumps oxygen-rich blood to the body). Both the pulmonary artery (which carries oxygen-poor blood to the lungs) and aorta (which carries oxygen-rich blood from the heart to the body) come from the same pumping chamber. No arteries are connected to the left ventricle (the chamber that normally pumps blood to the body).
Typically children with these heart defects will need to have surgery to repair their heart usually within the first year of life.
Initial Treatment for RVOT or Pulmonary Valve Replacement
If someone has a heart defect involving their RVOT or pulmonary valve, it is usually addressed with open-heart surgery to help restore blood flow to the lungs. To repair the RVOT, a conduit may be surgically implanted to connect the right ventricle to the main pulmonary artery. If the pulmonary valve needs to be replaced, the failing valve is usually removed and a new surgical tissue valve is inserted.
Over time both of these options are often not the final solution as conduits and/or tissue valves will need to be replaced later in life.
What Does it Mean When Your RVOT Conduit or Surgical Tissue Valve is Failing?
A person with CHD may need multiple surgeries over the course of their lifetime. Placement of an artificial RVOT conduit or pulmonary surgical tissue valve initially helps to restore a person’s heart function; however these are temporary fixes.
Nearly all RVOT conduits or pulmonary surgical tissue valves will fail at some point and may require intervention, usually because the RVOT conduit or surgical tissue valve has narrowed (stenosis) and/or leaks (regurgitation).
Narrowing (stenosis)
Pulmonary stenosis is when either the RVOT conduit becomes obstructed or the valved conduit or surgical tissue valve become calcified and the valve opening becomes narrowed. When this happens, it can restrict blood flow from the lower right ventricle to the pulmonary arteries, which delivers blood to the lungs.
Leaking (regurgitation)
Regurgitation, also called leaking, happens when either the RVOT conduit or the surgical tissue valve allows blood to flow backwards from the lungs into the heart. This is due to the valve leaflets not opening and closing properly, and as a result can cause the heart to work harder to pump the same amount of blood.
Symptoms that Your Conduit or Pulmonic Surgical Tissue Valve May be Failing
Talk to your doctor if you experience any of these symptoms. Regular checkups can help you and your doctor understand your current medical condition.
Understanding Your Treatment Options
Transcatheter Pulmonic Valve Therapy
During this minimally invasive procedure, a catheter holding a new heart valve is inserted through a small tube and guided to the intended location in your heart. Once the valve has reached the correct location, it is expanded with the help of a balloon. The valve should immediately begin to work and help control blood flow.
Surgical Repair or Replacement
For people whose RVOT conduit or pulmonary surgical tissue valve has failed, open-heart surgery may be the best option. During open-heart surgery, the doctor will remove the failing RVOT conduit or surgical tissue valve and replace it with a new surgical tissue valve and/or conduit.
Balloon Angioplasty/Valvuloplasty
A thin tube (catheter) with a balloon at the tip is put into the body through a vein. The balloon is guided to the intended location and temporarily inflated. This opens the narrowed valve and/or RVOT conduit and allows blood to flow better.
Your doctor will weigh the risks and benefits of each procedure to determine which treatment option is best for you.
Transcatheter pulmonic valve (TPV) therapy is a less invasive procedure than open-heart surgery. Learn more about how it can help someone who has a RVOT conduit or pulmonary surgical tissue valve that is now failing.
To see the latest information on clinical trials enrolling for pulmonary therapies, click here.