What is a pulmonary valve?
The pulmonary valve manages the flow of oxygen-poor blood from the right ventricle to the pulmonary artery on its journey to the lungs, where blood is enriched with oxygen before moving throughout the body.
What is the pulmonary valve’s role in circulation?
Just like the aortic valve, the pulmonary valve has three leaflets (also known as flaps or cusps) that serve as a one-way gate for blood flowing out of a ventricle into a main artery — in this case, from the right ventricle into the pulmonary artery. When the heart beats, the right ventricle contracts and the pulmonary valve opens, letting blood flow out of the heart. The pulmonary valve’s leaflets then close to prevent blood from flowing back into the heart.
What is pulmonary valve disease?
Pulmonary valve disease narrows the pulmonary valve and affects the critical flow of blood to the lungs. It can cause fatigue, shortness of breath, and potentially heart failure. Many of our patients are born with primary congenital pulmonary valve disease. Other patients develop a form of the disease after birth.
Pulmonary valve disease can present in several ways:
- Pulmonary atresia, a congenital heart defect (CHD) that causes the pulmonary valve to not form properly
- Pulmonary valve stenosis, a narrowing in the opening of the pulmonary valve
- Pulmonary valve regurgitation, when the pulmonary valve doesn’t close properly, allowing blood to reenter the right ventricle
- Absent pulmonary valve, a CHD in which the valve has underdeveloped or missing leaflets and causes pulmonary valve stenosis and regurgitation
The disease and its presentations can also be associated with:
- Truncus arteriosus
- Aortic valve disease and complications from the Ross procedure
- Pulmonary atresia with intact ventricular septum (PA/IVS)
- Endocarditis involving the pulmonary valve
How does tetralogy of Fallot affect pulmonary function?
A rare CHD called tetralogy of Fallot (TOF) can also affect the pulmonary valve. TOF causes significant changes to the heart anatomy, one of which is called an overriding aorta. Here, the pulmonary artery can be incorrectly positioned over a CHD known as a ventral septal defect.
Sometimes, the pulmonary valve is completely obstructed by a more severe form of TOF called tetralogy of Fallot with pulmonary atresia. Another form is tetralogy of Fallot with absent pulmonary valve (TOF-APV); this often causes airway obstruction because of tracheobronchomalacia (TBM) and a compression from massive dilatation of the main pulmonary artery and its first- and second-order branches.
Overcoming tetralogy of Fallot
After surgery to repair tetralogy of Fallot with pulmonary valve stenosis, James is all smiles. Read all about this “happy warrior.”
Pulmonary Valve Disease | Diagnosis & Treatments
How we approach treatment for pulmonary valve disease
The Congenital Heart Valve Program at Boston Children’s specializes in the care and treatment of pulmonary valve disease. Our team carefully considers two approaches — pulmonary valve repair and pulmonary valve replacement — when treating pulmonary valve disease. The approach depends on the patient’s condition, severity of the disease, their heart anatomy, and overall health.
Innovative three-dimensional modeling — as well as two- and three-dimensional cardiac echocardiography, CT scans, and cardiac magnetic resonance imaging (MRI) — give us a clear understanding of the state of a child’s pulmonary valve and their heart anatomy. We can then determine the right approach to treatment.
Pulmonary valve repair or reconstruction
We always first focus on pursuing less-invasive repair approaches that help children avoid surgery altogether and give them a better chance at improved health, potentially reducing the number of operations they might need later in life. There are two primary ways we can repair or reconstruct the diseased pulmonary valve:
- Cardiac catheterization: We often use catheterization to treat the disease as soon as possible. For example, we will use catheter-based techniques to treat pulmonary valve atresia and stenosis during the fetal or newborn stages. For children who have tetralogy of Fallot, we first try to rehabilitate the pulmonary valve through intraoperative balloon dilation. These approaches try to minimize the need for future pulmonary valve replacements.
- Ozaki procedure: We have also successfully adapted the Ozaki procedure, usually reserved for aortic valve neocuspidation, to surgically treat pulmonary valve disease. We use a modified Ozaki technique to create a three-leaflet pulmonary valve.
Pulmonary valve replacement
Not all patients are candidates for pulmonary valve repair and instead need a replacement valve. There are several proven commercially available replacement pulmonary valves; our team has the expertise to determine which one is best for a patient.
A heart valve that grows along with a child could reduce invasive surgeries
Invented at Boston Children's, a pulmonary valve replacement can be adjusted as a child grows, eliminating the need for invasive procedures.
