Patients with fibromuscular dysplasia have a high occurrence of aneurysm and/or dissection prior to or at the time of diagnosis, according to a new study published in the Journal of the American College of Cardiology.

Fibromuscular dysplasia is a noninflammatory arterial disease that predominantly affects women and includes arterial manifestations such as beading, stenosis, aneurysm, dissection, or tortuosity.

Daniella Kadian-Dodov, MD, of the Zena and Michael A. Wiener Cardiovascular Institute at Icahn School of Medicine at Mount Sinai in New York, and colleagues compared the frequency, location, and outcomes of patients with fibromuscular dysplasia with aneurysm and/or dissection to those patients without those manifestations.


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They collected data from the US Registry for Fibromuscular Dysplasia which includes 12 clinical centers. A total of 921 patients had complete clinical history as well as diagnostic and therapeutic procedure results. The majority of patients were female (93.5%) and white (91%) with a mean age of 48.1 ± 14.0 years at the onset of symptoms and 52.2 ± 13.4 years at diagnosis. Aneurysm was present in 21.7% of patients and dissection was present in 25.7%, with approximately 6% who had both.

While men were a minority of the patient population, they were more likely to experience aneurysm (32 of 60 patients; 53.3%) and/or dissection (25 of 60 patients; 41.7%) compared to 175 women (20.3%) who had aneurysm and 205 (23.8%) who had a dissection.

In terms of location, the most common sites for aneurysm were extracranial, renal, and intracranial arteries. Extracranial carotid, vertebral, renal, and coronary arteries were the most common sites for dissection. The most common location for aortic aneurysm was in the thoracic aorta, with 9 out of 20 in the ascending segment, and all patients had a tricuspid aortic valve. Among the patients who had extracranial carotid or vertebral artery dissection, 66 experienced dissection of more than 1 cervical artery and all dissections were located to the descending thoracic and abdominal segments.

Those patients who experienced dissection were younger (48.4 ± 53.5 years; P<.0001) and had more neurological symptoms and other end-organ ischemic events than those patients without dissection. Therapeutic intervention was required for one-third of patients who experienced an aneurysm (63 out of 200 patients). Of those 63 patients, 36 underwent endovascular therapy (most commonly coil embolization) and 26 underwent surgery. For patients with dissections, percutaneous transluminal angioplasty and percutaneous transluminal angioplasty plus stent implantation were the most frequently performed procedures.

“These findings support the recommendation that all patients with FMD [fibromuscular dysplasia] obtain cross-sectional imaging once (CTA or MRA) from head to pelvis,” the authors wrote. “Long-term outcome data are lacking, but no patient in this cohort died due to FMD or associated vascular events.”

Researchers encouraged future studies to investigate genetic and environmental factors that may contribute to arterial aneurysms and dissections in patients with fibromuscular dysplasia.

Reference

Kadian-Dodov D, Gornik HL, Gu X, et al. Dissection and aneurysm in patients with fibromuscular dysplasia. J Am Coll Cardiol. 2016;68(2):176-185. doi: 10.1016/j.jacc.2016.04.044.