Unique imaging findings in renal artery fibromuscular dysplasia.
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ABSTRACT:
SUBMITTER: Kang Y
PROVIDER: S-EPMC9257791 | biostudies-literature | 2022 Jul
REPOSITORIES: biostudies-literature
Publications
Unique imaging findings in renal artery fibromuscular dysplasia.
Kang Yuanyuan Y Xu Jianzhong J Wang Jiguang J
European heart journal. Case reports 20220616 7
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Project description:Fibromuscular dysplasia (FMD) involving the coronary arteries is an uncommon but important condition that can present as acute coronary syndrome, left ventricular dysfunction, or potentially sudden cardiac death. Although the classic angiographic "string of beads" that may be observed in renal artery FMD does not occur in coronary arteries, potential manifestations include spontaneous coronary artery dissection, distal tapering or long, smooth narrowing that may represent dissection, intramural hematoma, spasm, or tortuosity. Importantly, FMD must be identified in at least one other noncoronary arterial territory to attribute any coronary findings to FMD. Although there is limited evidence to guide treatment, many lesions heal spontaneously; thus, a conservative approach is generally preferred. The etiology is poorly understood, but there are ongoing efforts to better characterize FMD and define its genetic and molecular basis. This report reviews the clinical course of FMD involving the coronary arteries and provides guidance for diagnosis and treatment strategies.
| S-EPMC4370367 | biostudies-other
Project description:BackgroundWe present a patient with history of sinus venous thrombosis and hypertension during the last year. Her blood pressure was not controlled despite drugs, diet, and exercise. She denied symptoms. She does not smoke nor drink alcohol. Her body mass index was 20 kg/m2, NYHA Class I/IV.Case summaryA 40-year-old Latin-American female patient, concerned because despite taking verapamil 160 mg/day, losartan 100 mg/day, and hydrochlorothiazide 25 mg/day her diastolic blood pressure was over 100 mmHg. Routine serum and urine lab tests and a transthoracic echocardiogram were done and were normal. The treatment was increased over the next consultations but without control of her blood pressure. She developed resistant hypertension, and she was taking four antihypertensive drugs and two diuretics. A first renal artery Doppler was normal. Because of a high clinical suspicion a renal angio-CT was performed showing bilateral fibromuscular dysplasia. The patient underwent a renal artery angioplasty with balloon with excellent results and better control of her blood pressure after the procedure. Over the next month, her doctors were able to decrease her treatment to two drugs at intermediate doses. Currently, she is doing well and asymptomatic.DiscussionRenal artery fibromuscular dysplasia (FMD) could be a challenging disease to be diagnosed. Patients with this condition may suffer from symptomatic and resistant hypertension. Many patients do not have abnormalities on their physical exam or in the routine lab tests. Treatment includes renal artery angioplasty if patient is symptomatic and blood pressure is resistant.
| S-EPMC6439373 | biostudies-literature
Project description:A 53-year-old woman underwent a cardiac catheterization for evaluation of acute coronary syndrome. The coronary angiogram revealed evidence of spontaneous coronary artery dissection in multiple coronary arteries including the left anterior descending artery, posterior descending artery, and posterior left ventricular artery. Further diagnostic imaging revealed associated bilateral vertebral artery and renal artery fibromuscular dysplasia (FMD). Follow-up coronary angiogram 6 weeks later revealed a "string of beads" appearance of the posterior descending artery. This case highlights the importance of extra-coronary imaging for FMD and demonstrates angiogram findings suggestive of coronary FMD.
| S-EPMC8134944 | biostudies-literature
Project description:A 21-year-old female with a history of right nephrectomy due to trauma presented with several years of multidrug-resistant hypertension. Her workup included negative findings from autoimmune and vasculitides panels and urine catecholamine testing. Computed tomography showed an acute hairpin turn of her left renal artery. Intraoperatively, the artery demonstrated kinking with exhalation. She underwent excision of the diseased portion of the renal artery and an end-to-end anastomosis. Final pathologic examination demonstrated fibromuscular dysplasia. This is a unique case of mechanical artery kinking combined with fibromuscular dysplasia contributing to renovascular hypertension, for which open surgery was beneficial at improving the patient's hypertension.
| S-EPMC10547822 | biostudies-literature
Project description:Whether fibromuscular dysplasia (FMD) is a progressive disease, remains unclear. We reported a case of focal renal artery FMD that slowly progressed to a branching artery over a few years after the angioplasty without in-stent restenosis, which reconfirms that focal FMD is progressive and that such progression may be segmental. Stenting may be an option for young, risk factor-free patients with focal FMD.
| S-EPMC9713009 | biostudies-literature
Project description:Fibromuscular dysplasia (FMD), formerly called fibromuscular fibroplasia, is a group of nonatherosclerotic, noninflammatory arterial diseases that most commonly involve the renal and carotid arteries. The prevalence of symptomatic renal artery FMD is about 4/1000 and the prevalence of cervicocranial FMD is probably half that. Histological classification discriminates three main subtypes, intimal, medial and perimedial, which may be associated in a single patient. Angiographic classification includes the multifocal type, with multiple stenoses and the 'string-of-beads' appearance that is related to medial FMD, and tubular and focal types, which are not clearly related to specific histological lesions. Renovascular hypertension is the most common manifestation of renal artery FMD. Multifocal stenoses with the 'string-of-beads' appearance are observed at angiography in more than 80% of cases, mostly in women aged between 30 and 50 years; they generally involve the middle and distal two-thirds of the main renal artery and in some case also renal artery branches. Cervicocranial FMD can be complicated by dissection with headache, Horner's syndrome or stroke, or can be associated with intracerebral aneurysms with a risk of subarachnoid or intracerebral hemorrhage. The etiology of FMD is unknown, although various hormonal and mechanical factors have been suggested. Subclinical lesions are found at arterial sites distant from the stenotic arteries, and this suggests that FMD is a systemic arterial disease. It appears to be familial in 10% of cases. Noninvasive diagnostic tests include, in increasing order of accuracy, ultrasonography, magnetic resonance angiography and computed tomography angiography. The gold standard for diagnosing FMD is catheter angiography, but this invasive procedure is only used for patients in whom it is clinically pertinent to proceed with revascularization during the same procedure. Differential diagnosis include atherosclerotic stenoses and stenoses associated with vascular Ehlers-Danlos and Williams' syndromes, and type 1 neurofibromatosis. Management of cases with renovascular hypertension includes antihypertensive therapy, percutaneous angioplasty of severe stenoses, and reconstructive surgery in cases with complex FMD that extends to segmental arteries. The therapeutic options for securing ruptured intracerebral aneurysms are microvascular neurosurgical clipping and endovascular coiling. Stenosis progression in renal artery FMD is slow and rarely leads to ischemic renal failure.
| S-EPMC1899482 | biostudies-literature
Project description:Fibromuscular Dysplasia (FMD) is “an idiopathic, segmental, non-atherosclerotic and non-inflammatory disease of the musculature of arterial walls, leading to stenosis of small and medium-sized arteries” (Persu, et al; 2014). FMD can lead to hypertension, arterial dissections, subarachnoid haemorrhage, stroke or mesenteric ischemia. The pathophysiology of the disease remains elusive. While familial cases are rare (<5%) in contemporary FMD registries, there is evidence in favour of the existence of multiple genetic factors involved in this vascular disease. Recent collaborative efforts allowed the identification of a first genetic locus associated with FMD. This intronic variant located in the phosphatase and actin regulator 1 gene (PHACTR1) may influence the transcription activity of the endothelin-1 gene (EDN1) located nearby on chromosome 6. Interestingly, the PHACTR1 locus has also been involved in vascular hypertrophy in normal subjects, carotid dissection, migraine and coronary artery disease. National and international registries of FMD patients, with deep and harmonised phenotypic and genetic characterisation, are expected to be instrumental to improve our understanding of the genetic basis and pathophysiology of this intriguing vascular disease.
| S-EPMC5983654 | biostudies-literature
Project description:BackgroundFibromuscular dysplasia (FMD) is a non-inflammatory vascular disease that in children unlike in adults shows no sex predilection. FMD is often underdiagnosed, and its pathophysiology is unclear. Delayed diagnosis may lead to refractory hypertension and decreases the chance of successful treatment. Doppler ultrasound (US), magnetic resonance angiography (MRA), computed tomography angiography (CTA), and catheter-based angiography (angiography) are currently used to help make a clinicoradiological diagnosis of FMD. The main aim of the study was to compare the efficacy of imaging modalities which can allow for earlier and improved detection. Furthermore, an anatomical mapping of the location of lesions can help determine the best treatment modalities.MethodsAll patients with non-syndromic non-inflammatory renovascular hypertension were recruited from the Nephrology Department at the Children's Hospital of Philadelphia (CHOP) and enrolled in the U.S. FMD Registry maintained at the University of Michigan. Clinical presentation and imaging findings on US, CT, and MRI of children diagnosed with FMD were evaluated.ResultsMean age at diagnosis was 7 ± 4.9 years (4 months-17 years). Family history of hypertension (HTN) (52%), FMD (8.7%), Caucasian (60%), headache (48%), and HTN (80%) were the most prevalent symptom and sign at presentation. Bruits were 100% specific for renal artery stenosis (RAS) diagnosis but were heard in the minority of patients (3 patients, 12%). FMD was mainly unifocal within a single site (68%) or multiple sites (28%) and involved the main or first order renal branch in about 68% of children. Isolated distal lesions beyond the second order branches were found in about 25% of children. US imaging was significantly less sensitive than angiography (28%, p = 0.003). MRA had a better sensitivity (62.5%, p = 0.3) than US. Overall, CTA had the best sensitivity (84.2%, p = 0.4) compared to angiography; however, only angiography showed distal vessel disease.ConclusionsLimitations of the study include the sample size and biases-only patients diagnosed with FMD were included in this study and most patients were referred to a pediatric nephrologist for unexplained hypertension. Angiography should be performed as part of the initial work-up of any child suspected of having renovascular FMD, regardless of the findings seen on US, MRA, or CTA.
| S-EPMC6082421 | biostudies-literature
Project description:BackgroundSpontaneous coronary artery dissection (SCAD) is an important cause of acute coronary syndrome and is associated with fibromuscular dysplasia (FMD). The diagnosis of stress cardiomyopathy in patients with SCAD and FMD is uncommon, though an important consideration given the shared risk profile. Complications of severe left ventricular (LV) dysfunction associated with stress cardiomyopathy, such as LV thrombus, complicate the management of SCAD where anticoagulation is controversial in the context of SCAD-associated intramural haematoma.Case summaryA 65-year-old female presented with non-ST elevation myocardial infarction with a recent diagnosis of hypertension but no other traditional cardiovascular risk factors. There was, however, a family history of early cardiac death from myocardial infarction affecting her mother. Echocardiography demonstrated severe biventricular dysfunction with circumferential akinesis of the mid to apical segments. Coronary angiography demonstrated type 2A SCAD involving the first diagonal artery. Cardiac magnetic resonance imaging (MRI) confirmed a diagnosis of stress cardiomyopathy with biventricular involvement, complicated by LV apical thrombus and a focal region of myocardial infarction. Vascular imaging confirmed the presence of FMD. Guideline-directed heart failure therapy in addition to clopidogrel and rivaroxaban was prescribed. Follow-up contrast echocardiography at six-weeks confirmed resolution of LV dysfunction and resolution of the LV thrombus with no adverse events.DiscussionThe dual diagnosis of SCAD and stress cardiomyopathy is uncommon. Cardiac MRI was useful for confirming the diagnosis of stress cardiomyopathy and the presence of LV thrombus, where anticoagulation may complicate the management of intramural haematoma in patients with concomitant SCAD and FMD.
| S-EPMC9149788 | biostudies-literature
Project description:The burden of cardiovascular disease in women is being increasingly appreciated. Nevertheless, both clinicians and the general public are largely unaware that cardiovascular disease is the leading cause of death worldwide in women in all countries and that outcomes after a heart attack are worse for women than men. Of note, certain types of cardiovascular disease have a predilection for women, including spontaneous coronary artery dissection (SCAD) and fibromuscular dysplasia (FMD). Although uncommon, SCAD is being increasingly recognised as the cause of an acute coronary syndrome (ACS) and can recur. It is a potentially fatal, under-diagnosed condition that affects relatively young women, who often have few traditional risk factors, and is the commonest cause of a myocardial infarction associated with pregnancy. In contrast, FMD often remains silent but when manifested can also cause major sequelae, including renal infarction, stroke, cervical artery dissection and gut infarction. Here we provide an update on the diagnosis, aetiology and management of these important disorders that overwhelmingly affect women.
| S-EPMC7710561 | biostudies-literature