Case Report: Combination of Pressure Guidewire and Optical Coherence Tomography-Guided Drug-Coated Balloon Revascularization for Renal Artery Fibromuscular Dysplasia

Fibromuscular dysplasia (FMD) is the second common cause of renovascular hypertension. With the advent of endovascular therapy, angiography has become a diagnostic gold standard for FMD. Optical coherence tomography (OCT) by reflecting in vivo histology may improve diagnostic and classification accuracy. Renal fractional flow reserve (rFFR), measured by pressure guidewire, may distinguish the patients who may benefit from revascularization by identifying physiologically significant stenoses. However, the role of usage of both OCT and rFFR is not well-studied. We herein report a 17-year-old male with renovascular hypertension due to FMD. Angioplasty of drug-coated balloon (DCB) guided by OCT and FFR favorably achieved blood pressure (BP) control. In conclusion, the utility of both OCT and FFR may be useful for the appropriate selection of patients with renal FMD.

Carotid Web Phenotype Is Uncommonly Associated With Classic Fibromuscular Dysplasia: A Retrospective Observational Study

Background and Purpose: Carotid web (CaW) is an intimal form of fibromuscular dysplasia (FMD) involving the carotid bulb which has been increasingly recognized as a potential cause of recurrent ischemic strokes. It is overlooked as a separate entity and often dismissed if no coexistent signs of classic FMD changes are observed. We aim to evaluate the frequency of classic FMD in high-yield vascular territories in patients with symptomatic CaW. Methods: This was a retrospective analysis of a symptomatic CaW database of 2 comprehensive stroke centers (spanning September 2014–October 2020). The diagnosis of a CaW during a stroke workup was defined as the presence of a shelf-like linear filling defect in the posterior aspect of the carotid bulb on computed tomography angiography in patients with acute ischemic stroke or transient ischemic attack of undetermined cause after a thorough evaluation. Neck computed tomography angiography and renal conventional angiography images were independently evaluated by two readers blinded to the laterality and clinical details to inspect the presence of underlying classic FMD. Results: Sixty-six patients with CaW were identified. Median age was 51 years (interquartile range, 42–57), and 74% were women. All patients had neck computed tomography angiography (allowing for bilateral vertebral and carotid evaluation), whereas 47 patients had additional digital subtraction angiography (which evaluated 47 carotids ipsilateral to the stroke and 10 contralateral carotids). Internal carotid artery classic FMD changes were noted in only 6 out of 66 (9%) in the ipsilateral carotids. No contralateral carotid or vertebral artery classic FMD changes were observed. Renal artery catheter-based angiography was obtained in 16 patients/32 arteries and only 1 patient/2 renal arteries demonstrated classic FMD changes. Conclusions: CaW phenotype is uncommonly associated with classic FMD changes. Coexistent classic FMD does not constitute a useful marker to corroborate or exclude CaW diagnosis.

Unifocal progressed to multifocal renal artery fibromuscular dysplasia

Catheter angiography revealed distal unifocal stenosis of the renal artery progressed to a middle typical string of beads appearance in a 24-year-old Chinese woman diagnosed with fibromuscular dysplasia

Overview on Renovascular Hypertension: Review Article

Renovascular hypertension (RVH) is a prevalent cause of secondary hypertension that frequently develops to resistant hypertension. It is characterised as systemic hypertension that develops as a result of a restricted blood supply to the kidneys. Patients cannot be recognized clinically from those with essential hypertension; therefore, diagnosis requires arteriography, however urography and isotope renography may hint to the diagnosis. Atherosclerotic renal artery stenosis (ARAS) and fibromuscular dysplasia are the two most prevalent causes of RVH. The ultimate objective of controlling RVH, like with other kinds of hypertension, is to minimize the morbidity and mortality associated with high blood pressure The widespread use of effective antihypertensive medication treatment, statins, and other strategies to control vascular disease has resulted in remarkable improvements. In this review we will be looking at etiology, pathogenesis and treatment or RVH.

Young Stroke Secondary To Fibromuscular Dysplasia Causing Carotid Artery Dissection

Background: Fibromuscular dysplasia (FMD) is a rare, medium-sized arteriopathy affecting less than 7% of the population. It affects predominantly young women of childbearing age. Stroke caused by fibromuscular dysplasia of the carotid artery is uncommon and reported to range between 0.6% by angiography and 1.1% by autopsy. Case Presentation: A 35-year-old mother of two children, previously well, was admitted to our hospital with right-sided spastic hemiparesis with upper motor neuron type facial nerve palsy. Non-contrast CT (NCCT) brain on admission revealed left-sided middle cerebral arterial territory infarction with possible hemorrhagic transformation with midline shift. She had progressive neurological features where NCCT brain in 24 hours showed an increase in the size of the hemorrhage. Magnetic resonance imaging (MRI) of the brain, including angiogram of neck vessels, revealed acute dissection of the left internal carotid artery with acute thrombosis, left frontoparietal hemorrhagic infarction, and the constellation of MRI showed features suggestive of left cervico-cranial fibromuscular dysplasia complicated by acute internal carotid artery dissection. She was started on low-dose aspirin, and 40 mg of atorvastatin and anticoagulants were not started because of the bleeding risk as she had a hemorrhagic transformation infarction. She was discharged and referred for inward physiotherapy and planned to repeat the MRI brain with the neck vessel angiogram in three weeks and arranged digital subtraction angiography in three months. Unfortunately, she was lost for follow-up.Conclusion: Detailed evaluation of the young patients with ischaemic stroke is helpful to diagnose rare diseases such as FMD involving carotid arteries leading to a better treatment choice between anticoagulation vs. antiplatelet therapy.

The Role of Calcification in the Pathogenesis of Inflammatory Reaction in the Arterial Wall (Exemplified by the Vessels of the Neck and Head in Adults)

Calcification provoked by inflammation accompanies many comorbid conditions (including atherosclerosis and fibromuscular dysplasia). What is more, they mutually aggravate each other. The aim of this study was to reveal the patterns of calcification of the arteries of the neck and head in patients with clinical manifestations of vascular disorders of varying severity in inflammatory and non-inflammatory diseases. Materials and methods. A total of 155 patients were examined (mean age 49.1 ± 16.3 years) using computed tomography. The subjects were divided into three groups according to the severity of functional changes in the arterial bed: 1) no vascular obstruction (n = 43); 2) arterial obstruction ≤ 50 % according to NASCET criteria (n = 55); 3) arterial obstruction > 50 % (n = 57). A visual analysis of atherosclerotic lesions of the vessels of the neck and head revealed that the calcification was a dystrophic type of intimal lesion in almost half of the cases (44 %). A visual analysis of stenosis caused by fibromuscular dysplasia showed that calcium deposition is not typical of this process. The absence of calcium phosphate deposition in this case was associated with genetic connective tissue abnormalities. Results. We created a flowchart representing the pathogenesis of artery wall calcification in chronic inflammation. In addition, we revealed previously not described in the literature links between the processes of osteoporosis, progression of biomineralization of atherosclerotic plaques, and decalcification of plaques, which allowed us to indirectly assess their degree of maturation. The results of the study indicate that artery calcification could be considered as a possible marker of the progression of atherosclerosis. For citation: Mal’kov O.A., Govorukhina A.A., Burykin Yu.G., Afineevskaya A.Yu. The Role of Calcification in the Pathogenesis of Inflammatory Reaction in the Arterial Wall (Exemplified by the Vessels of the Neck and Head in Adults). Journal of Medical and Biological Research, 2021, vol. 9, no. 4, pp. 435–443. DOI: 10.37482/2687-1491-Z081

Segmental Arterial Mediolysis: A Multiguised Vasospastic Arteriopathy with Collateral Mesangial Cell Hyperplasia and Cardiac Toxicity Generated by Norepinephrine and Hyperdense Adrenoceptors Alone or by Crosstalk with Other Pressor Agents

Segmental arterial mediolysis (SAM), an uncommon vasospastic arteriopathy occurring in the muscular arteries innervated by the peripheral sympathetic nervous system, usually presents with catastrophic abdominal and retroperitoneal hemorrhages in elderly patients. SAM is initiated by the coupling of norepinephrine to plastically derived hyperdense foci of alpha-1 adrenergic receptors on the sarcolemma of arterial muscle. This ligand is created by stimuli signaled by iatrogenic sympathomimetic agonists, some beta-2 agonists, or an excessive release of adrenal catecholamines. Coupling of this ligand with cytoplasmic heterotrimeric Gq protein excessively signals a cascade of biochemical events generating two principal lesions of injurious-phase SAM—the shearing of the outer media from the adventitia and an overload of cytoplasmic calcium ions toxic to mitochondria causing mediolysis and/or apoptosis. The massive hemorrhages are caused by ruptured gap aneurysms created by the transmedial loss of the medial muscle. A norepinephrine-directed reparative response rapidly develops either resolving angiographic injurious lesions or creating a body of vascular disorders, the new guises of SAM with ischemic clinical profiles. These present in the epicardial, vertebral, intestinal, and retroperitoneal arteries, often in younger females as fibromuscular dysplasia, dissecting hematomas, and persistent aneurysms. Norepinephrine can crosstalk with other pressor agents to create SAM lesions—serotonin with idiopathic pulmonary hypertension and persistent pulmonary hypertension in the newborn, histamine in spontaneous coronary artery dissections with eosinophilia, and endothelin-1 in a field effect generated by SAM that creates venous fibromuscular dysplasia. Norepinephrine also participates in the collateral development of mesangial hyperplasia with focal segmental glomerulosclerosis and myocardial mediolysis and apoptosis in subjects with markedly elevated heart rates. Conclusion. Norepinephrine coupling with plastically elevated alpha-1 adrenoceptor or other pressor agents generates SAM, a histologically recognizable vasospastic arteriopathy, that with repair is transformed into several different standardized arterial diseases that alter SAM’s clinical profile from a hemorrhagic to an ischemic disorder.