Specialized fibromuscular dysplasia care

By Kamal Gupta, MD, vascular medicine specialist, interventional cardiologist

gupta-kamal-KS430554Fibromuscular dysplasia (FMD) is a relatively uncommon disease that predominately affects women. By some estimates, it could affect up to 1% of women, though it often goes undiagnosed.

The condition may never cause symptoms. But in some cases, it can lead to stroke or poorly controlled hypertension. While it often requires no treatment, all patients, even those who are asymptomatic, can benefit from evaluation, information and reassurance.

That's the role of the FMD clinic at The University of Kansas Health System. As one of only 11 FMD centers nationwide, we provide a valuable resource to primary care physicians and their patients with diagnosed or suspected FMD.

FMD at a glance

FMD is a noninflammatory, nonatherosclerotic arterial disease that can result in arterial stenosis, aneurysm or dissection. It occurs almost exclusively in women, most ages 20 to 60.

The most common FMD is medial fibroplasia, presenting as a beaded appearance of the affected arteries and found most commonly in the renal and carotid arteries. About 10% of cases present as focal, severe stenosis or longer, tubular lesions.

FMD concentrated in the renal arteries can cause severe and difficult-to-control hypertension, while carotid or vertebral FMD can cause headaches, pulsatile tinnitus and dizziness. It can lead to stroke or bleeding in the brain. FMD can affect the coronary arteries and result in spontaneous dissection of these arteries and heart attacks. It is believed that 85% or more of patients with spontaneous coronary dissection have FMD.

A specialized, multidisciplinary approach

FMD is often identified via CT or MRI scans following a stroke or during an investigation of uncontrolled hypertension. Because it often does not require treatment, it can receive little attention in the primary care setting.

Our FMD clinic works with a multispecialty team of neurologists, neuroradiologists, neurosurgeons, nephrologists, vascular specialists, vascular surgeons, cardiologists and geneticists. We also work closely with the complex hypertension clinic, run by our cardiovascular medicine and nephrology teams.

Upon referral, we collect and review previous records and images. If testing is necessary, we try to schedule it in the patient's home community. If treatment is necessary, we provide intensive blood pressure management and, in some cases, stenting or vascular surgery. Often, our primary role is to provide education and reassurance. We counsel patients in considering risk levels and understanding genetic connections. Consultation reduces patients' anxiety, helps them understand and live with their disease and improves quality of life.

We collaborate with primary care physicians treating FMD patients. FMD can skew the results of a typical carotid artery stenosis evaluation, for example, making it look overly severe. FMD has unique implications for vascular ballooning and stenting procedures, which we can help manage.

Dr. Kamal Gupta, FACC, FSCAI, RPVI, is the director of the vascular medicine program at The University of Kansas Health System. He is board-certified in vascular medicine, cardiology and interventional cardiology.