Fibromuscular Dysplasia Research Articles (Page 1)

Fibromuscular dysplasia (FMD) is found in 6 to 14% of patients with spontaneous cervical artery dissection (SCEAD) and may be associated with recurrent SCEAD. To evaluate the correlates of FMD in patients with SCEAD and to determine whether FMD is associated with recurrent SCEAD. This cohort study included patients from the Stroke Prevention in Cervical Artery Dissection (STOP-CAD) retrospective cohort study who presented from January 2015 to December 2022. This multicenter and international cohort included consecutive adult patients presenting at acute care hospitals and diagnosed with SCEAD in 63 sites in 16 countries. Data were analyzed from April to November 2024. Fibromuscular dysplasia was defined as either a history of FMD or presence of FMD on cervical or renal artery imaging. Clinical and radiological correlates were compared between patients with and without FMD using logistic regression models. Rates of recurrent SCEAD by 24 months were compared using a Cox proportional hazards model. This study included 3714 patients with SCEAD (median [IQR] age, 47 [38-56] years; 1637 [44.1%] females), of whom 196 (5.3%) had FMD. Patients with FMD were older (aOR per 10 years, 1.28; 95% CI, 1.14-1.43) and more often female (aOR, 2.00; 95% CI, 1.45-2.75). They more often had a history of dissection involving a noncervical artery (aOR, 8.10; 95% CI, 2.64-24.83), a history of SCEAD (aOR, 2.05; 95% CI, 1.07-3.93), a recent upper respiratory tract infection (aOR, 2.40; 95% CI, 1.52-3.78), a cerebral aneurysm (aOR, 2.22; 95% CI, 1.22-4.06), or a history of migraines (aOR, 2.44; 95% CI, 1.75-3.40). On imaging, they were less likely to have a single vertebral artery dissection (aOR, 0.37; 95% CI, 0.25-0.55) or an occlusive dissection (aOR, 0.55; 95% CI, 0.38-0.78). Eighty-one patients experienced a recurrent SCEAD, of which 46 (56.8%) occurred in the first 3 months of follow-up. The 24-month risk of recurrent SCEAD was 7.7% (95% CI, 3.1%-12.2%) and 2.8% (95% CI, 2.1%-3.5%) in patients with and without FMD, respectively (aHR, 2.75; 95% CI, 1.46-5.18; P = .002). In this cohort study of patients with SCEAD, FMD was associated with distinct correlates and a higher rate of recurrent SCEAD. These findings may help physicians in identifying and counseling patients with FMD and SCEAD.

Background: Spontaneous Coronary Artery Dissection (SCAD) is a cause of acute coronary syndrome, particularly among women, and is associated with psychological distress, including depression, anxiety, and post-traumatic stress (PTSD) symptoms. Fibromuscular dysplasia (FMD), a vascular condition that alters the arteries, is often found in patients with SCAD, adding complexity and uncertainty with a second serious diagnosis. This study examined the differences in mental health symptoms between SCAD survivors with and without FMD to better understand their unique psychosocial needs and inform interventions to enhance patient outcomes. Methods: Patients diagnosed with SCAD within the past 3 years were recruited from 6 Canadian cardiac hospitals. Participants completed questionnaires assessing socio-demographics, comorbidities, and validated psychological measures, including Patient Health Questionnaire-9 (PHQ-9) for depression, Generalized Anxiety Disorder-7 (GAD-7) for generalized anxiety, Cardiac Anxiety Questionnaire (CAQ) for cardiac-related anxiety, and PTSD Checklist (PCL-5) for PTSD symptoms. Differences in mental health outcomes between SCAD survivors with and without FMD were analyzed using t-tests for continuous variables and chi-square tests for categorical variables. Results: The study included 326 participants (92.9% female; mean age 53.3 years), with 67 participants (20.6%) having a confirmed diagnosis of FMD at the time of questionnaire completion (39.3% FMD at study completion). Post-SCAD, patients with FMD reported significantly higher scores than patients without FMD on the GAD-7 (6.4±5.9 vs. 4.7±4.9, p = .028), CAQ (29.9±13.1 vs. 26.0±12.3, p = .023), and PCL-5 (17.2±15.4 vs. 13.3± 14.0, p = .048). The proportion of SCAD survivors with FMD scoring in the clinically diagnostic range was significantly higher than those in patients without FMD: 32.8% vs. 20.8% for depression, 31.3% vs. 17.4% for anxiety, 64.2% vs. 49.2% for cardiac anxiety, and 25.4% vs. 11.6% for PTSD (all p < .05). Conclusions: Our findings suggest that SCAD survivors with comorbid FMD experience significantly higher distress —depression, generalized anxiety, cardiac-related anxiety, and PTSD symptoms—compared to those without FMD. Longitudinal research is needed to explore the role of these mental health concerns in long-term mental health and cardiac outcomes. Enhanced psychological interventions for those with FMD may be required.

Introduction: Spontaneous coronary artery dissection (SCAD) is a significant cause of acute coronary syndrome in young women. Fibromuscular dysplasia (FMD) is frequently associated with SCAD, but its impact on clinical outcomes remains unclear. Hypothesis: We hypothesized that SCAD patients with concurrent FMD would have different clinical outcomes compared to those without FMD, potentially reflecting distinct pathophysiological mechanisms. Methods: Using the TriNetX Global Collaborative Network across 129 healthcare organizations, we performed a retrospective propensity-matched cohort study. SCAD patients (ICD-10: I25.42) with FMD (ICD-10: I77.3) were compared to those without FMD. After 1:1 propensity matching based on demographics and comorbidities, 857 patients per group were analyzed. Primary outcome was all-cause mortality; secondary outcomes included heart failure, cardiogenic shock, arrhythmias, and acute kidney injury over five years. Analysis included risk ratios (RR), odds ratios, and hazard ratios (HR) with 95% confidence intervals. Results: SCAD patients with FMD had significantly lower all-cause mortality (1.3% vs 6.5%; RR 0.199, 95% CI 0.105-0.377; p<0.001), heart failure (4.7% vs 13.4%; RR 0.349, 95% CI 0.239-0.512; p<0.001), and acute kidney injury (1.2% vs 4.7%; RR 0.257, 95% CI 0.128-0.514; p<0.001). No significant differences were observed for cardiogenic shock, ventricular tachycardia, or atrial fibrillation. Conclusions: FMD is associated with markedly improved outcomes in SCAD patients, suggesting it represents a distinct, more favorable pathophysiological subtype. These findings support comprehensive FMD screening in SCAD patients for enhanced risk stratification and personalized management strategies.

Introduction: Spontaneous coronary artery dissection (SCAD) is a rare, underrecognized cause of acute coronary syndrome, responsible for up to 4% of ACS cases, with higher incidence in young females. It can result in myocardial infarction, arrhythmia, or sudden cardiac death. Fibromuscular dysplasia (FMD) is a common nonatherosclerotic arteriopathy found in SCAD patients. Pregnancy-associated SCAD (PASCAD) affects 1.81 per 100,000 pregnancies, according to national inpatient data. Case Presentation: A 38-year-old woman, G3P3, presented 16 days postpartum with chest pain radiating to both arms and transient left monocular vision loss. Troponins rose from 466 to 638 ng/L over 90 minutes. EKG showed ischemic changes. Cardiac catheterization revealed SCAD type 1, 2, and 3 on RPDA and RPLB, OM1, and mid to distal LAD respectively. Echocardiogram showed lateral wall hypokinesis with preserved ejection fraction. CT angiography identified 60% stenosis and subtle beading of the left internal carotid artery, <50% stenosis of the right carotid, and segmental narrowing of the superior mesenteric artery suggestive of FMD. ESR and CRP were elevated but rheumatologic workup was negative. Brain MRI and vascular duplex studies were unremarkable. The patient was treated with nitroglycerin and discharged on aspirin, clopidogrel, metoprolol, rosuvastatin, and amlodipine. Eleven days later, she presented with similar chest pain but no new EKG changes. She declined repeat angiography and opted to continue conservative management with cardiac rehabilitation. Discussion: PASCAD is a rare but important cause of ACS in postpartum women, likely driven by hormonal, hemodynamic, and vascular changes that increase arterial fragility. Coronary angiography is the diagnostic gold standard in symptomatic patients. Conservative medical therapy is preferred over PCI, given risks of stent misplacement and dissection extension. SCAD often resolves spontaneously, though recurrence occurs in ~11% within two years. Associations with pregnancy and FMD are well recognized and FMD is found in up to 86% of SCAD cases. SCAD and FMD share overlapping genetic mechanisms, underscoring the need for vascular screening in postpartum patients. Conclusion: This case highlights the importance of considering SCAD in postpartum women and the role of FMD as a predisposing arteriopathy. Prompt recognition and tailored management are essential to reduce morbidity and avoid invasive procedures when possible.

Introduction: Spontaneous coronary artery dissection (SCAD) is an important cause of acute coronary syndrome, especially in young and middle-aged women. Little is known about characteristics of SCAD across age groups. While SCAD can occur across a broad age spectrum, age-specific differences in SCAD manifestations are underexplored. Methods: Sub-analysis of the iSCAD Registry with stratification of patients into 3 age groups at time of index SCAD: < 40 years (y), 40-65 y and 65+ y. Data are reported for patients with complete investigator case report form and patient baseline questionnaire. Differences of characteristics between groups were calculated across strata using Kruskal-Wallis test or chi-square test, with statistical significance defined as p < 0.05. Results: Among 960 eligible participants, mean age was 49.8 y and 93.4% were female (no difference by group). Overall, 88.7% of participants were White. While racial distribution was similar across age groups (p=0.20), the <40 y group had a numerically higher proportion of Black patients (11.2% < 40 y vs. 6.3% 40-65 y vs. 2.4% 65+y). Patients ≥40 years were more likely to have hypertension and hyperlipidemia while patients <40y were more likely to have an inherited connective tissue disorder and pregnancy-related SCAD. Extra-coronary vascular abnormalities were prevalent in all 3 but fibromuscular dysplasia (FMD) was less common in <40 y group. Emotional and physical stressors were not different across groups. Primary clinical presentation of SCAD was most commonly non-ST elevation MI across all groups (p=NS). Younger patients (<40 y) had predominant LAD involvement (69.3% vs. 59.6% 40-65 y vs. 38.2% 65+ y, p< 0.01). Compared to older groups, patients <40 y were less frequently managed with medical therapy only and more frequently received revascularization and mechanical circulatory support. Conclusions: In this large multicenter SCAD cohort, age-stratified analyses revealed distinct clinical and angiographic profiles. Patients <40 y had more pregnancy-related SCAD and connective tissue disorders, while older patients had more traditional risk factors and FMD. LAD involvement and revascularization were more common in younger patients. These findings underscore the importance of age-specific approaches to SCAD care.

Description of Case: A 34-year-old female G3P3 presented at 6 weeks postpartum with acute chest pain, dizziness, and bradycardia. Troponin in ED was 79, then 873 at 2-hours and 3,613 at 6-hours. Echocardiogram revealed an ejection fraction (EF) of 55% with mild diastolic dysfunction and apical hypokinesis. LHC revealed spontaneous coronary artery dissection (SCAD) of the mid-distal LAD (type III) with TIMI-3 flow, treated conservatively. She started aspirin 81 mg daily and discharged after 3 days of observation. 12 hours later, she had recurrence of crushing chest pain and presented again to the ED (troponin elevated to 9,599; EKG found anterior STEMI). Repeat LHC found severe multivessel SCAD with dissection flap extending throughout left main artery, occlusive SCAD distal to D2 in the LAD, and occlusion of the left circumflex artery at the ostium. Impella CP was placed due to cardiogenic shock. Injection of contrast in the right femoral artery suggested newly diagnosed fibromuscular dysplasia (FMD). She underwent emergent 3-vessel CABG and Impella 5.5 placement. Postoperative EF was estimated at 15%-20% and she started on milrinone. Impella was removed on postoperative day (POD) #10, and milrinone weaned off on POD #13. She discharged home on low dose losartan, spironolactone, metoprolol, and digoxin. EF improved to 38% at discharge. CT angio of the head/neck/abdomen/pelvis 1 month later confirmed diagnosis of isolated abdominopelvic FMD. 2 months post discharge, our patient was walking 6 blocks thrice daily and participating in cardiac rehab (NYHA I/no angina). Discussion: This case extends our rapidly evolving understanding of cardiogenic shock due to SCAD, which often has a morbid course in the peripartum period and when the left main artery is involved. Impella support for cardiogenic shock became available in 2008 but is rarely used in SCAD management (<100 cases reported). While SCAD recognition has improved, optimal management of cardiogenic shock secondary to SCAD is undefined and may require revascularization and/or mechanical circulatory support, inotropes, or advanced heart failure therapy consideration. Our patient’s ability to wean off Impella support and inotropes prior to discharge raises the question if Impella support alone could temporize cardiogenic shock due to SCAD in lieu of Impella support combined with operative management. Further research is needed to define best practices for management of cardiogenic shock due to SCAD.

Introduction: Spontaneous coronary artery dissection (SCAD) is an infrequent but increasingly recognized cause of myocardial infarction that disproportionately affects women. SCAD is influenced by hormone fluctuations, arteriopathies, and physical&emotional stressors. We present a unique case of recurrent multivessel SCAD after severe emesis following GLP-1 receptor agonist (GLP-1RA) use. Case: A 58-year-old woman with past medical history of hypertension and depression presented to an emergency room (ER) with acute chest pain after intense running and was diagnosed with single vessel SCAD of the right coronary artery on coronary angiography. She was treated with dual antiplatelet therapy (DAPT) for 30 days and beta blockers without recurrent angina. One year later, she began taking a GLP-1RA (semaglutide 0.25 mg SQ once weekly) for weight loss, and developed extreme nausea and vomiting hours after her first dose. While retching, she experienced chest pain reminiscent of her prior SCAD episode and presented to the ER. Serial ECGs showed dynamic inferolateral ST depressions. Her initial high-sensitivity troponin I was 224 pg/mL and peaked at 15,492 pg/mL. Transthoracic echocardiogram showed normal function and no wall motion abnormalities. Coronary angiography revealed triple-vessel Type 2 SCAD involving the first obtuse marginal branch, distal left anterior descending artery, and posterior descending artery; each with TIMI 3 flow and not requiring percutaneous coronary intervention. Her chest pain was controlled with oral nitrates and beta blockers. She was prescribed DAPT with aspirin and clopidogrel for 6 months and discharged home with recommendation for outpatient fibromuscular dysplasia screening and cardiac rehabilitation. She was cautioned to discuss with her outpatient providers prior to reinitiation of GLP-1RAs. Discussion: This case illustrates a unique recurrence of SCAD occurring after semaglutide-induced vomiting, supporting a potential link between intense retching and SCAD in predisposed individuals. Although GLP-1RAs are not known to directly cause SCAD, their gastrointestinal side effects may act as precipitating physical stressors. With the anticipated exponential increase in GLP-1RA use, this case underscores the importance of exercising caution when initiating therapies with emetogenic potential in patients with a history of SCAD. Further studies are needed to understand this potential risk and guide safe therapeutic practices.

Computational assessment of fibromuscular dysplasia-related renal artery stenosis.

Spontaneous coronary artery dissection (SCAD) occurs in 1-4% of acute coronary syndromes (ACS). In SCAD, an intramural hematoma compresses the true lumen of the coronary artery, leading to ischemia and, even acute myocardial infarction.Approximately, 90% percent of SCAD patients are premenopausal women without classical risk factors for atherosclerosis. The gold standard for diagnosis is invasive coronary angiography and optical coherence tomography or intravascular ultrasound can be useful tools to confirm the diagnosis. Coronary intervention with stent placement is generally not recommended unless there is complete occlusion of the coronary artery with ongoing ischemia. In the acute phase, antiplatelet therapy and beta-blockers are advised, which are usually continued for life. Despite medical treatment, 10-20% of SCAD patients experience arecurrence within 4years. Nearly two-thirds of SCAD patients have fibromuscular dysplasia (FMD) based on CT angiography. Current treatment recommendations are based on expert opinion. Therapy and follow-up are advised to include at least one antiplatelet agent, abeta-blocker, screening for FMD, cardiac rehabilitation and among patients with left ventricular systolic dysfunction ACE inhibitor or aldosterone receptor blocker. Randomized controlled trials have been initiated to evaluate the treatment effects of beta-blocker and antiplatelet therapy in SCAD patients.

In this case report, sudden cardiac death caused by intussusception of a coronary artery is discussed. A 47-year-old man was found dead in the nursing home where he lived, following an episode of polyphagia and two of vomiting. Upon cadaveric dissection, an overdistention of the large intestine was noted. Re-evaluation of the formalin-fixed whole heart revealed occlusion of the circumflex branch of the left coronary artery, which was not macroscopically attributable to vascular thrombosis or an atheromatous plaque. Histological investigations revealed ischaemic-type histological changes of the left ventricular wall in a hyperacute phase of evolution and, in the occluded coronary branch, extensive intraluminal invagination of the intima and media, as occurs in vascular intussusception. Further stains revealed the presence of fibromuscular dysplasia of the wall of the affected vessel. The subject's death was ascribable to an acute cardiovascular failure secondary to acute ischaemic myocardial injury induced by intussusception of a coronary artery affected by dysplastic degeneration. These findings fully account for death by a mechanism sustained both by a mechanical deficit of the cardiac pump and by the possible onset of arrhythmias. Arterial intussusception is a rare complication of spontaneous coronary artery dissection. It is assumed that a combination of predisposing factors, which weaken the arterial wall, and trigger events, such as Valsalva-like activities, underlie the onset of the latter condition. This case highlights the importance of considering rare causes of sudden cardiac death. Greater awareness of these conditions can contribute to a more accurate identification of causes of death, with significant implications in both forensic and clinical settings.

Introduction: Spontaneous isolated celiac artery dissection (SICAD) without aortic involvement is a rare but potentially life-threatening condition that needs emergent care. Case presentation: A 57-year-old African American male with a history of hypertension, smoking, and substance use disorder presented with sudden-onset severe abdominal pain after 30 minutes of cocaine use. Imaging confirmed isolated celiac artery dissection following recent cocaine use. The patient was managed conservatively for spontaneous isolated celiac artery dissection. Clinical discussion: Spontaneous isolated celiac artery dissection (SICAD), characterized by sudden-onset abdominal pain localized to the periumbilical and epigastric regions, is linked to various risk factors such as hypertension, atherosclerosis, smoking, fibromuscular dysplasia, and autoimmune disorders. Studies underscore male predominance, the distinctive nature of abdominal pain, and the efficacy of conservative management. Additionally, cocaine use has been associated with multiple vascular pathologies, including dissection. Conclusion: Although the occurrence of spontaneous isolated celiac artery dissection (SICAD) after cocaine use has not been reported, the timing of cocaine use and symptom onset is highly suggestive of the association. The use of more frequent imaging would likely uncover more cases. It is important to keep this condition on the differential when evaluating abdominal pain in patients with cocaine abuse.

Fibromuscular dysplasia (FMD) is a vascular disorder affecting medium-sized arteries, including the extracranial cervical arteries, and can lead to aneurysmal subarachnoid hemorrhage (aSAH). We aimed to determine the prevalence of cervical FMD in aSAH patients and assess whether cervical FMD in these patients is associated with more severe aneurysmal disease and internal carotid artery (ICA) elongation. We retrospectively reviewed computed tomography angiography (CTA) scans acquired on admission in a consecutive series of aSAH patients (2019-2024). The prevalence of FMD in the extracranial segments of the ICA and vertebral arteries (VAs) was determined. In addition, differences in aneurysm size, number of aneurysms, rebleeding rates, and ICA elongation were assessed between patients with and without FMD using logistic regression, adjusting for potential confounders. Cervical FMD was identified in 40 of 485 aSAH patients (prevalence 8.3%, 95% confidence interval (CI) = 6.0-11.1%). aSAH patients with FMD were older, more frequently women, and more likely to have hypertension than those without FMD. We found no statistically significant differences in aneurysm size, number of aneurysms, rebleeding rates, or ICA elongation. We report a high prevalence (8.3%) of cervical FMD among aSAH patients, but no clear differences in aneurysm severity and ICA elongation compared to those without. Given the high prevalence, we recommend routine screening for cervical FMD in aSAH patients on CTA (or another angiography modality). If FMD is suspected, full-body CTA (or other angiographic modality) should be considered for further vascular assessment.

Clinical spectrum and therapeutic strategies of fibromuscular dysplasia and segmental arterial mediolysis: A cohort study.

Acute Renal Infarction Caused by Renal Arteriovenous Fistula in a Patient with Fibromuscular Dysplasia: A Rare Case Report

Non-invasive evaluation of the hemodynamic significance of renal artery stenosis in fibromuscular dysplasia through quantitative image analysis.

Background: Cervical artery dissection (CAD) is a leading cause of acute ischemic stroke among young and middle-aged patients. Currently, the growing availability of high-resolution magnetic resonance imaging (MRI), particularly fat-saturated T1-weighted black-blood SPACE sequences, allows the non-invasive, rapid, and reliable diagnosis of multiple arterial dissections. Methods: We reported our experience from two tertiary stroke centers of patients diagnosed with spontaneous multiple cervical artery dissections, detected with high-resolution MRI, during a three-year period (2022–2025). Results: Among 95 consecutive patients with CAD, 11 patients (mean age: 48 ± 9 years, 6 (55%) females) were diagnosed with multiple symptomatic or asymptomatic CADs, whereas in 84 patients (mean age: 49 ± 11 years, 32 (38%) females) a single CAD was detected. In all patients, high-resolution MRI and MR-angiography were performed, whereas digital subtraction angiography (DSA) with simultaneous evaluation of renal arteries was conducted in nine patients. A history of trauma or chiropractic manipulations, intense physical exercise prior to symptom onset, recent influenza-like illness, and recent childbirth in a young female patient were reported as predisposing risk factors. Cervicocranial pain, cerebral infarctions leading to focal neurological signs, and Horner’s syndrome were among the most commonly documented symptoms. Characteristic findings in the high-resolution 3D T1 SPACE sequence were detected in all patients. Fibromuscular dysplasia and Eagle syndrome were detected in four patients and one patient, respectively. Eight patients were treated with antiplatelets, whereas three patients received anticoagulation with low-molecular-weight heparin. There was only one case of stroke recurrence during a mean follow-up period of 9 ± 4 months. Conclusions: This case series highlights the utility of specific high-resolution MRI sequences as a very promising method for detecting multiple CADs in young patients. The systematic use of these sequences could enhance the sensitivity of detecting multiple cervical CADs, affecting also the thorough investigation for underlying connective tissue vasculopathies, stratifying the risk for first-ever or recurrent ischemic stroke, and influencing acute reperfusion and secondary prevention therapeutic strategies.

Impact of access site on carotid artery stenting in patients with challenging aortic arch anatomy.

Subclavian artery web (SAW) is a rare intimal variant of fibromuscular dysplasia that has been recognized as a potential cause of posterior-circulation stroke. On imaging, SAW typically presents as a thin, shelf-like intraluminal projection that could be misinterpreted as dissection or atherosclerotic plaque. Given the rarity of this entity, the optimal management strategy remains uncertain. Analogous to carotid artery web, stent implantation could resolve underlying hemodynamic disturbances and reduce the risk of embolic events. Herein, we report a case of SAW diagnosed via digital subtraction angiography (DSA) combined with computed tomography angiography (CTA), followed by stent implantation to prevent embolization.

Abstract Spontaneous coronary artery dissection (SCAD) has been increasingly recognized as a potentially serious nonatherosclerotic condition that can mimic atherosclerotic acute coronary syndromes. This condition has been identified as presenting with the classical clinical and electrocardiographic features that are encountered with acute coronary syndromes. Its formal identification has been historically challenging and difficult. A high index of clinical suspicion before invasive coronary angiography depends on the presence of some inherited and acquired risk factors. Early suspicion plays a key role in the identification of SCAD lesions during coronary angiography. Multiple angiographic features may be present, thus resulting in different types of SCAD lesions. Acute SCAD management favors conservative approaches for stable cases, with revascularization for high-risk presentations. Pharmacotherapy typically includes antiplatelet agents and β-blockers, avoiding routine anticoagulation. Long-term care involves recurrence prevention, monitoring, counseling, and screening for extra coronary fibromuscular dysplasia. Significant areas of uncertainty remain, as this relates to the best long-term management and surveillance strategies. This review aims to summarize the current state of knowledge and evidence, while pointing out the remaining challenges and the need for additional research.

Impact of carotid web orientation on long-term thrombus growth risk: A numerical study using fluid-solid interaction.