Cardiac Center for Marfan Syndrome and Related Aortic Disorders
Primary Children's Hospital hosts a premier cardiac center for the treatment of patients with Marfan syndrome and related aortic syndromes.
Adults and children with known or suspected Marfan syndrome, Loeys-Dietz syndrome, Ehlers-Danlos syndrome, and other connective tissue disorders, are evaluated in this clinic. A multidisciplinary approach to patient care is used and patients are referred for genetic, orthopedic and ophthalmologic evaluation as needed. The clinic receives referrals from physicians and patients in Utah, Idaho, Wyoming and Nevada.
The PCMC Marfan clinic is one of only a few Marfan clinics in the country currently involved in a multi-centered trial of Losartan vs. Beta Blocker therapy in Marfan syndrome. This study is sponsored by both the National Heart & Lung & Blood Institute and the National Marfan Foundation.
Marfan syndrome is a heritable disorder of connective tissue that affects many organ systems, including the skeleton, heart and blood vessels, eyes and lungs. The syndrome may go undiagnosed leading to premature death from cardiac complications. Routine cardiac care has been instrumental in greatly improving the lifespan of patients with Marfan syndrome.
Loeys-Dietz syndrome, a newly recognized connective tissue disorder, shares many features with Marfan syndrome including a predisposition to develop aneurysms of the aorta. This syndrome also has unique features which help distinguish it from Marfan syndrome including widely spaced eyes (hypertelorism), a relatively large head, abnormalities of the bones in the upper part of the spine (cervical vertebrae) and a cleft palate or cleft uvula. Patients with Loeys-Dietz syndrome appear to be prone to develop aneurysms in vessels other than the aorta.
Director
Clinic Days
Mondays 8 am-5 pm
