Truncal Valve Disease | Diagnosis & Treatments
Treatment for truncal valve disease starts with fetal care
A child who has truncal valve disease will need a lifetime of care. The Congenital Heart Valve Program at Boston Children’s specializes in the care and treatment of truncal valve disease. That care often starts at birth. Using two- and three-dimensional cardiac echocardiography, CT scans, and cardiac magnetic resonance imaging (MRI), we work closely with the Fetal Cardiology Program to detect and diagnose truncal valve disease and any CHD in the fetal stage. We can potentially intervene before birth with specialized in utero treatment. We are at the forefront of fetal cardiac care and have performed more than 185 interventions.
How we approach treatment for truncal valve disease after birth
Getting that head start on care also allows us to treat a child immediately after birth. We work closely with Boston Children’s Neonatal Cardiac Surgery team to ensure treatment planning starts as soon as possible. A few weeks after a child is born and is ready for surgery, we separate the truncus arteriosus into two proper arteries, the pulmonary and aorta. We also repair the VSD.
Most truncal valves are repaired without having to replace it. That’s because when we separate the truncal valve, we use surgical techniques for an aortic valve repair, to ensure that what becomes a new aortic valve properly functions. Those techniques are:
- Simple leaflet repair: This procedure focuses on restoring an existing damaged leaflet of the aortic valve by thinning or implanting it again.
- Complex leaflet repair/bicuspidization: This involves more intricate modifications of the valve leaflets and their relation to the aortic root and neighboring aorta. The bicuspidization technique creates two symmetric leaflets.
- Valve-sparing aortic root repair: This procedure preserves the native aortic valve and replaces the aortic root, which is the section of the aorta connected to the heart. For some patients, the diseased aortic valve cannot be repaired and must instead be replaced with a mechanical or biological valve.
- Ozaki procedure: Also known as neocuspidization, the Ozaki procedure is a complex aortic valve treatment that reconstructs degenerated leaflets. We create customized leaflets from a patient’s own tissue (pericardium). During the same surgery, we create a functioning pulmonary vessel by implanting a homograft (human tissue valves) or artificial conduits (tubes). We also close the VSD with a patch to separate the two lower ventricles.
Because truncal valve disease is associated with abnormalities and malformations of the coronary arteries, we also work closely with the Coronary Artery Program to treat those coronary arteries.
We also perform truncal valve repairs for older patients who didn’t have treatment earlier in life and now experience valve stenosis and regurgitation.
Learn how 3D modeling has transformed the planning of surgery by showing details of a patient’s heart.
