Coronary Artery Tortuosity and Spontaneous Coronary Artery Dissection: Association With Echocardiography and Global Longitudinal Strain, Fibromuscular Dysplasia, and Outcomes.

BACKGROUND: The etiology and significance of coronary artery tortuosity (TCA) among patients with spontaneous coronary artery dissection (SCAD) are unknown. The aim of this prospective imaging cohort study was to report echocardiographic findings and evaluate whether TCA correlates with cardiac anatomy and function among patients with SCAD. Comorbidities including fibromuscular dysplasia (FMD) and outcomes were also assessed. METHODS: TCA was determined on coronary angiography performed during the diagnosis of SCAD, and cardiac structure and function were evaluated using prospective comprehensive echocardiography. RESULTS: Among 116 patients with SCAD, the mean age at echocardiography was 50.8 ± 8.8 years, a median of 10.9 months after SCAD. Sixty-two patients (53.4%) had FMD, 41 (35.3%) had histories of hypertension, and 17 (14.8%) were hypertensive during echocardiography. Most patients (n = 78 [69%]) had normal left ventricular geometry with normal median ejection fraction (61%; interquartile range, 56% to 64%) and normal global longitudinal strain (-22.2%; interquartile range, -24.0% to -19.9%). Fifteen patients (13.4%) had diastolic dysfunction that was associated with hypertension at the time of echocardiography. Patients with TCA (n = 96 [82.8%]) were older (mean age, 52.1 ± 8.0 vs 44.7 ± 9.9 years; P < .001) with a higher prevalence of FMD (59.4% vs 25%, P = .007) but a similar prevalence of hypertension (35% vs 35%, P > .99) compared with patients without TCA. Across the age range (31.5 to 66.9 years), each decade of age was associated with an approximately 0.89-unit increase in coronary tortuosity score (P < .0001). Echocardiographic parameters were not significantly different between the two groups. Median follow-up duration was 4.4 years (95% CI, 3.8 to 5.2 years). The Kaplan-Meier 3-year SCAD recurrence rate was 9.4% (95% CI, 3.7% to 14.8%). There were no deaths. CONCLUSIONS: The majority of patients with SCAD had normal or near normal echocardiographic results, including global longitudinal strain, with no differences according to TCA. However, patients with SCAD with TCA were older, with a higher prevalence of FMD.

Not So Benign Chest Pain in the Young: A Story of Spontaneous Coronary Artery Dissection and Fibromuscular Dysplasia.

Spontaneous coronary artery dissection (SCAD) is a rare clinical entity, often presenting similar to atherosclerotic acute coronary syndrome (ACS), although it is a non-atherosclerotic, non-traumatic, and non-iatrogenic coronary artery pathology. We report the case of a 36-year-old woman who presented with substernal, pressure-like chest pain without aggravating, alleviating, or associated symptoms. Initial evaluation revealed elevated troponin levels which peaked at 8.71 ng/mL. Electrocardiogram showed borderline J point elevation in the lateral leads. A transthoracic echocardiography revealed mild left ventricular dysfunction with an estimated ejection fraction of 45% to 50%, an akinetic apex with hyperdynamic basal segments, suggestive of stress cardiomyopathy. However, a left anterior descending artery (LAD) infarction could not be excluded. Emergent coronary angiography revealed SCAD of the LAD. No coronary interventions were performed. The patient was managed medically with dual antiplatelet therapy and beta-blockers. Given the high suspicion for fibromuscular dysplasia (FMD), computed tomographic angiography (CTA) of the head to pelvis was performed. The CTA neck showed bilateral focal areas of mild stenosis and dilation of the distal cervical internal carotid artery, and CTA abdomen revealed multiple splenic artery aneurysms, diagnostic of FMD. Unlike atherosclerotic ACS, our patient was managed medically without percutaneous intervention. This case illustrates the rare occurrence of myocardial injury due to SCAD which results in spontaneous intramural hematoma formation rather than atherosclerotic plaque rupture typically seen in ACS. FMD remains the most commonly associated condition with SCAD.

Cerebrovascular Fibromuscular Dysplasia - A Practical Review.

Fibromuscular dysplasia (FMD) is a rare idiopathic, segmental, noninflammatory and nonatherosclerotic arteriopathy of medium-sized arteries. It is classically considered to be a disease of young and middle adulthood, with females more commonly affected than males. FMD is a systemic disease. Although historically considered to be rare, cerebrovascular FMD (C-FMD) has now been recognized to be as common as the renovascular counterpart. Extracranial carotid and vertebral arteries are the most commonly involved vascular territories in C-FMD with the clinical presentation determined by vessels affected. Common symptoms include headaches and pulsatile tinnitus, with transient ischemic attacks, ischemic stroke and subarachnoid or intracerebral hemorrhage constituting the more severe clinical manifestations. Cervical artery dissection involving carotids more often than vertebral arteries and intracranial aneurysms account for the cerebrovascular pathologies detected in C-FMD. Our understanding regarding C-FMD has been augmented in the recent past on account of dedicated C-FMD data from North American, European and other international FMD cohorts. In this review article, we provide an updated and comprehensive overview on epidemiology, clinical presentation, etiology, diagnosis and management of C-FMD.

Derivation and Validation of Spontaneous Coronary Artery Dissection Prediction Score in Patients With Myocardial Infarction.

Spontaneous coronary artery dissection (SCAD) is an important cause of acute myocardial infarction (AMI); however, the prevalence of SCAD and its predictors in AMI are unknown. We sought to derive and validate a simple score that can help predict SCAD in patients with AMI. We analyzed the Nationwide Readmissions Database and created a risk score for SCAD in patients with an index hospitalization for AMI. We used a multivariate logistic regression analysis to determine the independent predictors of SCAD, and each was assigned points proportional to its regression coefficient. Among 1,155,164 patients with AMI, 8,630 (0.75%) had SCAD. Based on the derivation cohort, the independent predictors of SCAD were fibromuscular dysplasia (odds ratio [OR] 67.0, 95% confidence interval [CI] 42.0 to 107.9, p <0.01), Marfan or Ehlers-Danlos syndrome (OR 4.7, 95% CI 1.7 to 12.5, p <0.01), polycystic ovarian syndrome (OR 5.4, 95% CI 3.0 to 9.8, p <0.01), female gender (OR 1.99, 95% CI 1.9 to 2.1, p <0.01), and aortic aneurysm (OR 1.41, 95% CI 1.1 to 1.7, p <0.01). The SCAD risk score included fibromuscular dysplasia (5 points), Marfan or Ehlers-Danlos syndrome (2 points), polycystic ovarian syndrome (2 points), female gender (1 point), and aortic aneurysm (1 point). The C-statistics of the score were 0.58 (derivation cohort) and 0.61 (validation cohort). In conclusion, the SCAD score is an easy bedside clinical tool that can help clinicians identify patients with AMI who are at risk for SCAD.

Prevalence and clinical characteristics of renovascular hypertension associated with fibromuscular dysplasia in China.

OBJECTIVES: The aim of this study was to investigate the clinical characteristics of renal artery fibromuscular dysplasia (FMD) in patients in China and identify the cure rate of hypertension after angioplasty. METHODS: Consecutive hypertensive patients with renal artery stenosis caused by FMD who underwent catheter-based angiography, and were followed at two Chinese referral centres, were retrospectively analysed. All patients underwent a detailed investigation, including demographic characteristics, clinical characteristics, biochemical sampling, Doppler ultrasonography of carotid arteries, magnetic resonance angiography (MRA) of the intracranial artery, and CTA or MRA of the abdominal artery and catheter-based renal angiography. Patients were routinely followed up at 1 month, 6 months and every year after the procedure. RESULTS: Among 245 study participants, with a mean diagnosed age of 26.9 ±â€Š9.9 years, 137 (55.9%) were women, and 38 (15.5%) were children. All patients were diagnosed with hypertension at a mean age of 23.4 ±â€Š8.4 years. There were 73.5% focal and 15.2% multivessel cases. Aneurysms, arterial dissections and total occlusions were found in 21.6, 4.1 and 12.2% of patients, respectively. Patients with multifocal FMD were older (26.0 vs. 23.7 years, P  = 0.021) and more often female (70.8 vs. 50.6%, P  = 0.004). Among children with renal FMD, 55.2% were men, and 86.8% were focal. After a median follow-up of 7.0 years, multifocal FMD had a higher cure rate of hypertension than focal FMD after revascularization (71.7 vs. 55.8%, P  = 0.032). CONCLUSION: In a cohort of mostly young Chinese patients, the prevalence of hypertension associated with renal FMD is similar in both sexes. Focal FMDs were more frequent than the multifocal ones and, after angioplasty, were associated with a worse blood pressure outcome.

Fibromuscular Dysplasia and Abdominal Aortic Aneurysms Are Dimorphic Sex-Specific Diseases With Shared Complex Genetic Architecture.

BACKGROUND: The risk of arterial diseases may be elevated among family members of individuals having multifocal fibromuscular dysplasia (FMD). We sought to investigate the risk of arterial diseases in families of individuals with FMD. METHODS: Family histories for 73 probands with FMD were obtained, which included an analysis of 463 total first-degree relatives focusing on FMD and related arterial disorders. A polygenic risk score for FMD (PRSFMD) was constructed from prior genome-wide association findings of 584 FMD cases and 7139 controls and evaluated for association with an abdominal aortic aneurysm (AAA) in a cohort of 9693 AAA cases and 294 049 controls. A previously published PRSAAA was also assessed among the FMD cases and controls. RESULTS: Of all first degree relatives of probands, 9.3% were diagnosed with FMD, aneurysms, and dissections. Aneurysmal disease occurred in 60.5% of affected relatives and 5.6% of all relatives. Among 227 female first-degree relatives of probands, 4.8% (11) had FMD, representing a relative risk (RR)FMD of 1.5 ([95% CI, 0.75-2.8]; P=0.19) compared with the estimated population prevalence of 3.3%, though not of statistical significance. Of all fathers of FMD probands, 11% had AAAs resulting in a RRAAA of 2.3 ([95% CI, 1.12-4.6]; P=0.014) compared with population estimates. The PRSFMD was found to be associated with an AAA (odds ratio, 1.03 [95% CI, 1.01-1.05]; P=2.6×10-3), and the PRSAAA was found to be associated with FMD (odds ratio, 1.53 [95% CI, 1.2-1.9]; P=9.0×10-5) as well. CONCLUSIONS: FMD and AAAs seem to be sex-dimorphic manifestations of a heritable arterial disease with a partially shared complex genetic architecture. Excess risk of having an AAA according to a family history of FMD may justify screening in family members of individuals having FMD.

Characteristics and Prognosis of Patients With Fibromuscular Dysplasia in a Population of Spontaneous Coronary Artery Dissections (from the French Registry of Spontaneous Coronary Artery Dissections "DISCO").

Spontaneous coronary artery dissection (SCAD) and fibromuscular dysplasia (FMD) are pathologies that appear to be closely related. This study compares the characteristics of the FMD population to the non-FMD population in a SCAD cohort. It thus assesses the involvement of the FMD phenotype in a SCAD population. From the data of the French DISCO registry, we included patients with a diagnosis of SCAD and in whom a search for FMD was performed. We collected the following characteristics of this population: the clinical and angiographic presentation, the data concerning the management, and the events occurring during the follow-up. In the 373 SCADs confirmed in the DISCO registry, we obtained imaging data for 340 of them. FMD was found in 45% of cases. The mean age was higher in the FMD group, 53.2 ± 8.8 years, versus 50.1 ± 11 years in the non-FMD group. High blood pressure and postmenopausal status were significantly higher in the FMD group. Clinical presentation, angiographic data, and management were comparable. The major adverse cardiac event rate and recurrence rate were not different between the 2 groups after 1 year of follow-up. In conclusion, we confirmed a 45% prevalence of FMD in the SCAD population. The median age was higher in the FMD group, suggesting that FMD may develop over time. The rate of major adverse cardiac events and recurrence were similar in the FMD group versus the non-FMD group after 1 year of follow-up.

Coronary fibromuscular dysplasia with three-vessel involvement: a rare cause of ischaemic dilated cardiomyopathy in a very young male.

Coronary artery disease of non-atherosclerotic aetiology, while rare in incidence, can have a wide aetiology, such as fibromuscular dysplasia, which is a non-inflammatory arteriopathy of numerous histopathological types of fibromuscular tissue accumulation. This brief report describes the case of a 22-year-old male with a recently developed dilated cardiomyopathy and a history of aborted cardiac arrest at the age of 14 years. Coronary angiogram revealed severe three vessels disease, while optical coherence tomography established fibromuscular dysplasia as aetiology. Balloon and stent angioplasty was performed guided by fractional flow reserve with acceptable angiographic result.

Spontaneous cervical artery dissection and fibromuscular dysplasia: Epidemiologic and biologic evidence of a mutual relationship.

Cervical artery dissection (CeAD) is the most common cause of ischemic stroke in young and middle-aged adults. Over the last decade, a relation between CeAD and fibromuscular dysplasia (FMD), an idiopathic, segmental, non-atherosclerotic and non-inflammatory arterial disease, has been suggested based on a number of epidemiologic observations, while preliminary data support the idea that the two conditions may share common biologic mechanisms. In this article, we review the literature on the relation between CeAD and FMD, focus on the potential pathogenetic mechanisms common to the two conditions, summarize clinical features, management and outcome, and provide support to the hypothesis that the coexistence of the two diseases in one individual might be conceptualized as a distinct non-atherosclerotic non-inflammatory arteriopathy.

Spontaneous coronary artery dissection: an Italian single centre experience.

AIMS: Spontaneous coronary artery dissection (SCAD) is an emerging cause of acute myocardial infarction (AMI) in young women without a typical cardiovascular risk profile. Knowledge on SCAD is based on observational studies and is still scarce. The aim of this monocentric observational study was to evaluate the predisposing factors, clinical features and prognosis of SCAD patients. METHODS: Between 2013 and 2020, 58 patients with angiographic diagnosis of SCAD were identified in our centre with an overall prevalence of 0.9% among patients admitted for AMI (58 of 6414 patients). RESULTS: The mean age was 54 ±â€Š11 years and the majority were women (n = 50, 86%) with one or fewer cardiovascular risk factors (n = 35, 60%). The prevalence of Fibromuscolar Dysplasia (FMD) was 39% (7 of 18 screened patients). The rate of major adverse cardiovascular and cerebrovascular events (MACCEs) was used to assess the prognosis. Out-of-hospital cardiac arrest because of ventricular fibrillation was observed in four (7%) patients. The majority of patients (n = 51, 88%) were treated conservatively without revascularization. The in-hospital and 30-day clinical course was uneventful in most patients (n = 54, 93%) with two cardiac deaths. During a median follow-up of 12 months, there were no further deaths. The global rate of SCAD recurrence was significant (n = 8, 14%) but predictors have not been identified. CONCLUSION: Although overall survival seems good, SCAD is a potentially malignant, not rare disease, which can present with sudden cardiac death and not uncommon recurrence. Prognostic stratification and optimal management of SCAD patients remain to be defined.

Prevalence and Disease Spectrum of Extracoronary Arterial Abnormalities in Spontaneous Coronary Artery Dissection.

Importance: Spontaneous coronary artery dissection (SCAD) has been associated with fibromuscular dysplasia (FMD) and other extracoronary arterial abnormalities. However, the prevalence, severity, and clinical relevance of these abnormalities remain unclear. Objective: To assess the prevalence and spectrum of FMD and other extracoronary arterial abnormalities in patients with SCAD vs controls. Design, Setting, and Participants: This case series included 173 patients with angiographically confirmed SCAD enrolled between January 1, 2015, and December 31, 2019. Imaging of extracoronary arterial beds was performed by magnetic resonance angiography (MRA). Forty-one healthy individuals were recruited to serve as controls for blinded interpretation of MRA findings. Patients were recruited from the UK national SCAD registry, which enrolls throughout the UK by referral from the primary care physician or patient self-referral through an online portal. Participants attended the national SCAD referral center for assessment and MRA. Exposures: Both patients with SCAD and healthy controls underwent head-to-pelvis MRA (median time between SCAD event and MRA, 1 [IQR, 1-3] year). Main Outcome and Measures: The diagnosis of FMD, arterial dissections, and aneurysms was established according to the International FMD Consensus. Arterial tortuosity was assessed both qualitatively (presence or absence of an S curve) and quantitatively (number of curves ≥45%; tortuosity index). Results: Of the 173 patients with SCAD, 167 were women (96.5%); mean (SD) age at diagnosis was 44.5 (7.9) years. The prevalence of FMD was 31.8% (55 patients); 16 patients (29.1% of patients with FMD) had involvement of multiple vascular beds. Thirteen patients (7.5%) had extracoronary aneurysms and 3 patients (1.7%) had dissections. The prevalence and degree of arterial tortuosity were similar in patients and controls. In 43 patients imaged with both computed tomographic angiography and MRA, the identification of clinically significant remote arteriopathies was similar. Over a median 5-year follow-up, there were 2 noncardiovascular-associated deaths and 35 recurrent myocardial infarctions, but there were no primary extracoronary vascular events. Conclusions and Relevance: In this case series with blinded analysis of patients with SCAD, severe multivessel FMD, aneurysms, and dissections were infrequent. The findings of this study suggest that, although brain-to-pelvis imaging allows detection of remote arteriopathies that may require follow-up, extracoronary vascular events appear to be rare.

High prevalence of extrarenal artery involvement in children with fibromuscular dysplasia - a single-center experience.

BACKGROUND: Although the clinical presentation of fibromuscular dysplasia (FMD) and its generalized character has been previously described in adults, data on FMD in children are limited. METHOD: In this study, we aimed to assess visceral artery involvement in pediatric FMD patients with documented renal artery stenosis (RAS) and renovascular hypertension (RVH) in comparison with healthy individuals. We retrospectively analyzed the results of angiographic studies of 16 patients with a median age of 13.9 years (10 girls) in comparison with 16 age- and sex-matched healthy controls. RESULTS: Out of the 16 FMD patients, 10 (63%) had stenotic lesions identifiable only in renal arteries, whereas six (37%) had additional stenoses in other vascular beds - in the celiac trunk (four patients), superior mesenteric artery (four patients), inferior mesenteric artery (one patient), splenic artery (one patient), common hepatic artery (three patients), and abdominal aorta (one patient). The comparison of ostial diameters of vessels, in which no periostial narrowing, stents, or occlusions were found, revealed that patients with FMD had a significantly smaller diameter of the celiac trunk (P = 0.017), splenic arteries (P = 0.007), and common hepatic artery (P = 0.026) than their age- and sex-matched healthy peers. CONCLUSION: We found that 69% of children with RVH caused by FMD had clinically silent stenoses or tortuosity of visceral arteries. The results of our study suggest that pediatric FMD is a generalized arterial condition, and the patients may need screening for both renal as well as nonrenal manifestations of the disease.

Association of Fibromuscular Dysplasia and Pulsatile Tinnitus: A Report of the US Registry for Fibromuscular Dysplasia.

Background Fibromuscular dysplasia (FMD) is a nonatherosclerotic arterial disease that has a variable presentation including pulsatile tinnitus (PT). The frequency and characteristics of PT in FMD are not well understood. The objective of this study was to evaluate the frequency of PT in FMD and compare characteristics between patients with and without PT. Methods and Results Data were queried from the US Registry for FMD from 2009 to 2020. The primary outcomes were frequency of PT among the FMD population and prevalence of baseline characteristics, signs/symptoms, and vascular bed involvement in patients with and without PT. Of 2613 patients with FMD who were included in the analysis, 972 (37.2%) reported PT. Univariable analysis and multivariable logistic regression were performed to explore factors associated with PT. Compared with those without PT, patients with PT were more likely to have involvement of the extracranial carotid artery (90.0% versus 78.6%; odds ratio, 1.49; P=0.005) and to have higher prevalence of other neurovascular signs/symptoms including headache (82.5% versus 62.7%; odds ratio, 1.82; P<0.001), dizziness (44.9% versus 22.9%; odds ratio, 2.01; P<0.001), and cervical bruit (37.5% versus 15.8%; odds ratio, 2.73; P<0.001) compared with those without PT. Conclusions PT is common among patients with FMD. Patients with FMD who present with PT have higher rates of neurovascular signs/symptoms, cervical bruit, and involvement of the extracranial carotid arteries. The coexistence of the 2 conditions should be recognized, and providers who evaluate patients with PT should be aware of FMD as a potential cause.

Fibromuscular dysplasia: its various phenotypes in everyday practice in 2021.

Fibromuscular dysplasia (FMD) is a non-atherosclerotic vascular disease that may involve medium-sized muscular arteries throughout the body. The pathogenesis of FMD remains poorly understood, but a combination of genetic and environmental factors may be involved. The majority of FMD patients are women, but men may have a more progressive disease, especially when smoking. Besides the classical phenotype of string of beads or focal stenosis, arterial aneurysms, dissections, and tortuosity are frequent manifestations of the disease. However, the differential diagnosis of FMD is extensive and includes imaging artefacts as well as other arterial diseases. Diagnosis is based on CT-, MR-, or conventional catheter-based angiography during work-up of clinical manifestations, but clinically silent lesions may be found incidentally. Arterial hypertension and neurological symptoms are the most frequent clinical presentations, as renal and cerebrovascular arteries are the most commonly involved. However, involvement of most arteries throughout the body has been reported, resulting in a variety of clinical symptoms. The management of FMD depends on the vascular phenotype as well on the clinical picture. Ongoing FMD-related research will elaborate in depth the current progress in improved understandings of the disease's clinical manifestations, epidemiology, natural history and pathogenesis. This review is focused on the clinical management of adult FMD in daily practice.

FMD and SCAD: Sex-Biased Arterial Diseases With Clinical and Genetic Pleiotropy.

Multifocal fibromuscular dysplasia (FMD) and spontaneous coronary artery dissection are both sex-biased diseases disproportionately affecting women over men in a 9:1 ratio. Traditionally known in the context of renovascular hypertension, recent advances in knowledge about FMD have demonstrated that FMD is a systemic arteriopathy presenting as arterial stenosis, aneurysm, and dissection in virtually any arterial bed. FMD is also characterized by major cardiovascular presentations including hypertension, stroke, and myocardial infarction. Similar to FMD, spontaneous coronary artery dissection is associated with a high prevalence of extracoronary vascular abnormalities, including FMD, aneurysm, and extracoronary dissection, and recent studies have also found genetic associations between the two diseases. This review will summarize the relationship between FMD and spontaneous coronary artery dissection with a focus on common clinical associations, histopathologic mechanisms, genetic susceptibilities, and the biology of these diseases. The current status of disease models and critical future research directions will also be addressed.

Prevalence of Cervical Artery Abnormalities on CTA in Patients with Spontaneous Coronary Artery Dissection: Fibromuscular Dysplasia, Dissection, Aneurysm, and Tortuosity.

BACKGROUND AND PURPOSE: Little is known about associations between spontaneous coronary artery dissection and cervical artery abnormalities. This study sought to assess the prevalence of cervical artery abnormalities among patients with spontaneous coronary artery dissection. MATERIALS AND METHODS: A retrospective analysis was completed of patients who underwent CTA neck imaging as part of arterial assessment following the diagnosis of spontaneous coronary artery dissection. The internal carotid and vertebral arteries were evaluated for the presence of fibromuscular dysplasia, dissection and/or pseudoaneurysm, ectasia and/or aneurysmal dilation, atherosclerosis, and webs. Carotid tortuosity was categorized into kinks, loops, coils, and retrojugular and/or retropharyngeal carotid courses; vertebral tortuosity was classified by subjective analysis of severity. RESULTS: Two hundred fourteen patients were included in the final cohort, of whom 205 (95.8%) were women; the average age was 54.4 years. Fibromuscular dysplasia was the most frequently observed abnormality (83 patients; 38.8%), followed by dissections and/or pseudoaneurysms (n = 28; 13.1%), ectasia and/or aneurysmal dilation (n = 22; 10.3%), and carotid webs (n = 10; 4.7%). At least 1 type of carotid tortuosity was present in 99 patients (46.3%). The majority (n = 185; 86.4%) of patients had no carotid atherosclerosis; and 26 (12.2%) had mild; 3 (1.4%), moderate; and 0, severe carotid atherosclerosis. CONCLUSIONS: The most common abnormality in the cervical artery vasculature of patients with spontaneous coronary artery dissection is fibromuscular dysplasia. Cervical dissections were higher than previously reported but were not observed in most patients.

Fibromuscular dysplasia: A comprehensive review on evaluation and management and role for multidisciplinary comprehensive care and patient input model.

Fibromuscular dysplasia is a nonatherosclerotic, under-recognized disorder primarily seen in middle-aged women. It can lead to several complications, such as hypertension, headaches, dissections, aneurysms, myocardial infarctions, and cerebrovascular accidents, to name a few. This article provides a comprehensive review of current literature on epidemiology, etiology, diagnosis, treatment, and long-term surveillance and fibromuscular dysplasia management. In addition, it renders the role of education and prevention for patients living with this condition and family screening. Lastly, it emphasizes the importance of a comprehensive multidisciplinary care model and patient input, given the complexity of this disease and its systemic presence and protean manifestations.

Long-term outcomes of kidney donors with fibromuscular dysplasia.

BACKGROUND: Fibromuscular dysplasia (FMD) is a non-atherosclerotic systemic arterial disease that is not infrequently discovered during kidney donor evaluation. Current guidelines do not provide recommendations regarding the use of kidneys from donors with FMD and there is a paucity of data on the outcomes of these donors. METHODS: The Renal and Lung Living Donor Evaluation (RELIVE) study addressed long-term outcomes of 8922 kidney donors who donated between 1963 and 2007. We compared the development of hypertension, cardiovascular disease (CVD), proteinuria and reduced estimated glomerular filtration rate (eGFR) in 113 kidney donors with FMD discovered during donor evaluation versus 452 propensity score matched donors without FMD. Outcomes modeling with logistic and Cox regression analysis and Kaplan-Meier statistics were performed. RESULTS: Donors with FMD were older (51 versus 39 years), were more likely to be women (80% versus 56%) and had a higher systolic blood pressure at donation (124.7 versus 121.3 mmHg) (P < 0.05 for all). After a mean ± standard deviation follow-up of 15.5 ± 8.9 years, a similar proportion of donors with and without FMD were alive, and developed hypertension (22.2% versus 19.8%), proteinuria (20.6% versus 13.7%) and CVD (13.3% versus 13.5%). No donor with FMD developed an eGFR <30 mL/min/1.73 m2 or end-stage kidney disease. The multivariable risk of mortality, CVD and renal outcomes in donors with FMD was not elevated. CONCLUSIONS: Kidney donors with FMD appear to do well, do not appear to incur increased risks of hypertension, proteinuria, CVD or reduced eGFR, and perhaps carefully selected candidates with FMD can safely donate as long as involvement of other vascular beds is ruled out.

Clinical Features of Patients With Cervical Artery Dissection and Fibromuscular Dysplasia.

BACKGROUND AND PURPOSE: Observational studies have suggested a link between fibromuscular dysplasia and spontaneous cervical artery dissection (sCeAD). However, whether patients with coexistence of the two conditions have distinctive clinical characteristics has not been extensively investigated. METHODS: In a cohort of consecutive patients with first-ever sCeAD, enrolled in the setting of the multicenter IPSYS CeAD study (Italian Project on Stroke in Young Adults Cervical Artery Dissection) between January 2000 and June 2019, we compared demographic and clinical characteristics, risk factor profile, vascular pathology, and midterm outcome of patients with coexistent cerebrovascular fibromuscular dysplasia (cFMD; cFMD+) with those of patients without cFMD (cFMD-). RESULTS: A total of 1283 sCeAD patients (mean age, 47.8±11.4 years; women, 545 [42.5%]) qualified for the analysis, of whom 103 (8.0%) were diagnosed with cFMD+. In multivariable analysis, history of migraine (odds ratio, 1.78 [95% CI, 1.13-2.79]), the presence of intracranial aneurysms (odds ratio, 8.71 [95% CI, 4.06-18.68]), and the occurrence of minor traumas before the event (odds ratio, 0.48 [95% CI, 0.26-0.89]) were associated with cFMD. After a median follow-up of 34.0 months (25th to 75th percentile, 60.0), 39 (3.3%) patients had recurrent sCeAD events. cFMD+ and history of migraine predicted independently the risk of recurrent sCeAD (hazard ratio, 3.40 [95% CI, 1.58-7.31] and 2.07 [95% CI, 1.06-4.03], respectively) in multivariable Cox proportional hazards analysis. CONCLUSIONS: Risk factor profile of sCeAD patients with cFMD differs from that of patients without cFMD. cFMD and migraine are independent predictors of midterm risk of sCeAD recurrence.