Approximately 90 percent of children with Alagille syndrome have a heart defect at birth. The severity of the heart defect can range from a simple murmur causing no problems to a major structural heart defect. Most Alagille-related heart defects involve the pulmonary arteries, which carry blood from the right side of the heart to the lungs. The most common defect is peripheral pulmonary stenosis. Other potential structural cardiac defects include:
The more severe congenital heart defects may require treatment, including surgical intervention. If your child has Alagille-associated heart problems, his or her doctor will talk with you about treatment options.
Reviewed by: Binita M. Kamath, MD
Date: January 2009