Brain-to-Pelvis Imaging Substantially Impacts Management of Patients With Fibromuscular Dysplasia.

During the past decade, and in the wake of French, United States, and more recently European/International registries, our image of fibromuscular dysplasia (FMD) as a rare cause of renovascular hypertension in young women due to the iconic string-of-beads has evolved into a constellation of different clinical patterns. The latter include such diverse presentations as: focal stenosis in children or young men; multifocal FMD in older women coexisting with atherosclerotic lesions; carotid/vertebral, renal, or mesenteric arterial dissections leading to stroke, renal infarction, or mesenteric ischemia; cerebral aneurysms; and even arterial tortuosity. Furthermore, in various cohorts of potential kidney donors, silent renal artery FMD lesions were found in 2.3% to 6.6% of subjects, suggesting that symptomatic FMD is only the tip of the iceberg of a not so rare disease. Finally, it has been shown that FMD affects 2 or more arterial beds in 48% to 66% of patients according to cohort studied, definition of FMD, and screening modality. Such novelties have been incorporated in the First International Consensus on the diagnosis and management of FMD. According to the Consensus, while the diagnosis of FMD still requires presence of at least one string-of-beads or focal stenosis, in presence of such lesion(s), coexisting dissections, aneurysms, or tortuosity identified in other arterial beds are considered as additional manifestations of the disease (Figure). Furthermore, the Consensus recommends once a lifetime brain-to-pelvis CT-angiography (CTA) or contrast-enhanced MR angiography to detect additional FMD lesions, aneurysms, or dissections. While the International Consensus has been generally well received by clinical FMD experts, presentation of the consensus statement advocating brain-to-pelvis imaging in all patients with FMD to wider audiences has inevitably led to someone raising his/her hand asking: “how will this change patient management? ”. The timely report the Assessment of Renal and Cervical Artery Dysplasia-Poland study (ARCADIA-POL) by Warchol-Celinska et al in the current issue of Hypertension provides us important elements to answer that question based on data obtained in a near ideal setting: a cohort of 232 consecutive patients with FMD from all over Poland explored in a single institution using state-of the-art methods—including the recommended whole body CTA. The key findings are the following: (1) new FMD lesions were identified in 34% of patients; (2) new vascular complications (ie, dissections or aneurysms) in 25% and, most importantly, (3) 25% of all patients qualified for interventional treatment due to newly diagnosed FMD lesions or vascular complications. While the proportion of newly identified arterial abnormalities is not unexpected and consistent with or even lower than reported in previous studies, the prevalence of patients requiring outright intervention—1 out of 4 patients is surprisingly high and is not in line with our experience in Brussels or Cleveland. Indeed, while FMD is often discovered on the occasion of a work-up for hypertension or severe vascular complications such as cervical, renal, or coronary dissection, it is our experience that additional lesions detected by systematic CTA are often milder and seldom require immediate action. This seems to be even more the case when the initial lesion is discovered incidentally. Several elements may have influenced the results of the ARCADIA-POL report: (1) as indicated in the Limitations section, the cases referred to the Warsaw Institute of Cardiology for inclusion in ARCADIA-POL may be particularly symptomatic and/or severe (potential for referral bias); (2) as in most other European cohorts but in contrast with the US Registry for FMD, patients included in ARCADIA-POL mostly presented with renovascular hypertension, the most frequent FMDrelated symptom was hypertension (91%) and FMD lesions were most frequently found in renal arteries (88%). In addition, the prevalence of symptoms associated with cerebrovascular FMD, such as headache, dizziness, and pulsatile tinnitus was relatively low; (3) out of 59 lesions qualifying for endovascular intervention, 31 were newly diagnosed significant renal artery stenosis—which is not equivalent to newly diagnosed renal FMD; 9 of these lesions corresponded to restenosis of a previously revascularized lesion; (4) the other predominant intervention group consisted in aneurysms (n=25), mostly affecting intracranial (n=16) and renal (n=10) arterial beds. However, at least in the published version of the article, no detailed documentation of aneurysm size or individual rationale for intervention is available. It seems nevertheless that the size of aneurysm was not the primary criterion for intervention and that small or very small aneurysms were considered as eligible The opinions expressed in this article are not necessarily those of the American Heart Association. From the Division of Cardiology, Cliniques Universitaires Saint-Luc and Pole of Cardiovascular Research, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium (A.P.); Harrington Heart and Vascular Institute, University Hospitals Cleveland Medical Center (H.L.G.); and Department of Medicine, Case Western Reserve University, Cleveland, OH (H.L.G.). Correspondence to: Alexandre Persu, MD, PhD, FESC, Division of Cardiology, Cliniques Universitaires St-Luc, Unversité catholique de Louvain, 10 Ave Hippocrate, 1200, Brussels, Belgium. Email alexandre. persu@uclouvain.be Brain-to-Pelvis Imaging Substantially Impacts Management of Patients With Fibromuscular Dysplasia