Search

Background Fibromuscular dysplasia (FMD) is a non-atherosclerotic arterial disease characterized by the presence of string-of-beads or focal stenosis. Other FMD-related lesions include arterial dissections, aneurysms, and tortuosity. However, limited data are available on the involvement of coronary arteries in pts diagnosed with “extra-coronary” FMD. Purpose To examine FMD-related coronary artery involvement, coronary artery lesions and coronary tortuosity in patients with a diagnosis of “extra-coronary” FMD. Methods In a case control study we enrolled 103 (age 45.7±13.2, 82 women) consecutive patients with a diagnosis of FMD, in whom atherosclerotic coronary artery disease was excluded on the basis of coronary CTA, and 96 (age 47.3±12.2, 75 women) sex- and age-matched controls without coronary atherosclerosis based on CTA imaging (Siemens Force 2x192 scanner). Detailed analysis of coronary arteries was assessed, with detailed analysis of arterial tortuosity, defined as curves >30 OR >45 OR >90 degrees. Tortuosity Index (TI) was defined as number of curves x artery length (centerline) / vector artery start-end. Each arterial tortuosity evaluation was performed per coronary segment AND per artery. Size (area, length) of coronary arteries was also measured. Results Intravessel symmetry sign and cork-screw sign were more common in arteries of FMD patients than in controls (5.4% vs 1.3%, p30 degrees) (0.777; 95% CI:0.713 to 0.833) or TI (0.794; 95% CI: 0.731 to 0.848), both in distal LAD. For the distal LAD indices, the best sensitivity and specificity values were for ≥4 curves (sens. 74%, spec. 72%) or for a TI >5.6 (sens. 55%, spec. 94%), respectively. These predictive values were confirmed after correction for potential confounders. Interestingly, neither proximal LAD or proximal LCx number of curves or TI were related to FMD. No difference was found in terms of areas of coronary arteries between FMD patients and controls, with the only exception of LM (20.8±6.6 vs 17.9±5.8, p=0.002). Mean coronary artery lenght was significantly different between controls and patients with FMD (respectively LM 8.4±4.2 vs 9.8±4.5, p=0.022; LAD 117.8±28.6 vs 109.1±30.2, p=0.042; LCx 85.2±43.9 vs 62.9±31.4, p Conclusions Distal and not proximal coronary arterial tortuosity is related to FMD. Coronary tortuosity defined as 4 distal LAD curves or a TI >5.6 are highly specific for FMD. Funding Acknowledgement Type of funding source: None

Fibromuscular dysplasia (FMD) is an idiopathic, segmental, non-atherosclerotic and non-inflammatory disease, which leads to stenosis of small- and medium-sized arteries. With cerebrovascular FMD, renal artery FMD is the most frequent presentation of the disease. The prevalence of renal FMD remains elusive but an estimate of 3–5% seems reasonable. Renal artery FMD is usually diagnosed incidentally, on the occasion of a work-up for hypertension, or seldom following renal artery dissection. The typical patient with renal artery FMD is a ~50-year-old woman with hypertension, but FMD can also be diagnosed in men and at all ages of life. In children and adolescents, it is often associated with severe hypertension and cardiovascular damage. In most cases, CT-/MR-angiography may be considered as the test of choice for both screening and diagnosis of renal FMD, in association with duplex ultrasound evaluation, which provides additional hemodynamic information. In view of the high (>50%) prevalence of multivessel FMD, once a lifetime exploration of all vascular beds from brain to pelvis is recommended. Detection of cerebrovascular FMD is of particular importance, due to its high prevalence and potential dramatic consequences of complications. Smoking cessation is strongly advised. Antiplatelet treatment deserves to be considered on a case-by-case basis. In patients with hypertension, strict blood pressure control is required. Revascularization—usually by percutaneous balloon angioplasty without stenting—is justified, especially in young patients, in patients with recent onset or severe hypertension, as well as in rare cases of deterioration of renal function, in the presence of arguments in favour of a hemodynamically significant renal artery stenosis. FMD may be familial in up to 10% of cases; therefore, screening for FMD should be considered in relatives with suggestive symptoms. Finally, as patients with FMD may progress or develop complications, a lifelong yearly follow-up, tailored for each patient, is justified. The development of the US and European/International FMD registries and associated studies will undoubtedly shed new light on FMD and allow substantial progress in the understanding and management of the disease in the next decade.

Objective:Visceral artery fibromuscular dysplasia (VA FMD) is being rarely reported, with clinical picture ranging from asymptomatic to life-threatening. The aim of the study is to evaluate the prevalence and clinical characteristics of VA FMD.Design and method:Out of 330 patients enrolled into ongo

Objective:We previously reported results of a European survey suggesting that standard recommendations for blood pressure (BP) measurement are poorly followed in dialysis centres. The objectives of the current sub-study were (i) to compare adherence to office BP measurement guidelines in dialysis ce

Objective:In a retrospective study, the prevalence of preeclampsia in patients with renal FMD was estimated to 52% (Vance et al., 2015). The aim of our study was to assess the prevalence and nature of pregnancy-related complications in patients with FMD.Design and method:A call for participation was

Objective:Management of resistant hypertension (RHTN) is challenging and often implies the use of complex polypharmacy and interventional therapies. The main objectives of this study were (i) to describe the characteristics of patients with RHTN referred to two expert centres; (ii) to identify disti

Objective:Previous reports have shown a high prevalence of concomitant renal and cerebrovascular Fibromuscular Dysplasia (FMD). However, systematic whole body CT scan was performed in only few studies. Furthermore, distinctive features of patients with or without cerebrovascular FMD have not been re