Alagille Syndrome
Conference Roundup

Early cardiac referral in ALGS key to good transplant outcomes

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Some studies estimate that up to 90% of patients with Alagille syndrome (ALGS) have congenital heart disease, which underscores the importance of early cardiac referral in these patients, according to a presentation at the 10th International Symposium & Scientific Meeting on Alagille Syndrome.

“A comprehensive review of cardiac and liver issues and careful planning and coordination are important in caring for these patients,” said co-presenter Noelle Ebel, MD.

“Early cardiac referral,” she said, “is key for good survival and transplant outcomes.”

During the presentation, Dr Ebel and Jeffrey A. Feinstein, MD, MPH highlighted several tests that are utilized in the work-up and treatment decision-making for individuals with ALGS.

This non-invasive diagnostic test is often part of the standard follow-up for children with ALGS, however, it is difficult to see the portions of the pulmonary arteries affected by ALGS on these images.

If more information is needed, additional tests will have to be ordered, according to the presenters.

Lung perfusion scan
This non-invasive test is done standardly both pre- and post-operatively in children with ALGS. Using an IV, a tiny amount of radioactive material is used to show which areas of the lung the blood is currently going to.

Although images from these tests provide exquisite detail without invasive measures, they are often not sufficient to make all the diagnoses needed. In addition, CT scans emit radiation, and MRI’s may require anesthesia for younger children unable to stay still.

Cardiac catheterization
This invasive diagnostic test that requires anesthesia, measures and provides pictures that can help “plan for treatment once sufficient evidence of a problem is identified,” the presenters said.

Ebel N and Feinstein J., 2022, Approach, Decision Making and Treatments for Heart and Liver interrelated issues. Presented at: International Symposium & Scientific Meeting on Alagille Syndrome.

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