ガイドライン解説 AHA/ACCF secondary prevention and risk reduction therapy for patients with coronary and other atherosclerotic vascular disease : 2011 update

Spontaneous coronary artery dissection (SCAD) has emerged as an important cause of acute coronary syndrome, myocardial infarction, and sudden death, particularly among young women and individuals with few conventional atherosclerotic risk factors. Patient-initiated research has spurred increased awareness of SCAD, and improved diagnostic capabilities and findings from large case series have led to changes in approaches to initial and long-term management and increasing evidence that SCAD not only is more common than previously believed but also must be evaluated and treated differently from atherosclerotic myocardial infarction. High rates of recurrent SCAD; its association with female sex, pregnancy, and physical and emotional stress triggers; and concurrent systemic arteriopathies, particularly fibromuscular dysplasia, highlight the differences in clinical characteristics of SCAD compared with atherosclerotic disease. Recent insights into the causes of, clinical course of, treatment options for, outcomes of, and associated conditions of SCAD and the many persistent knowledge gaps are presented.

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Spontaneous Coronary Artery Dissection: Current State of the Science: A Scientific Statement From the American Heart Association.

This article is a comprehensive document on the diagnosis and management of fibromuscular dysplasia (FMD), which was commissioned by the working group ‘Hypertension and the Kidney’ of the European ...

https://journals.sagepub.com/doi/pdf/10.1177/1358863X18821816

First International Consensus on the diagnosis and management of fibromuscular dysplasia.

Spontaneous Coronary Artery Dissection: JACC State-of-the-Art Review.

Summary of Updated Recommendations for Primary Prevention of Cardiovascular Disease in Women: JACC State-of-the-Art Review

Objectives This study sought to evaluate the diagnostic yield of a dedicated computed tomography angiography (CTA) protocol of the chest, abdomen, and pelvis in patients with fibromuscular dysplasia (FMD). Background FMD is an uncommon vascular disease that may result in stenosis, dissection, or aneurysm of nearly all arterial distributions, typically affecting medium-sized arteries. Findings from the United States Registry for Fibromuscular Dysplasia have suggested the potential need to perform screening imaging of the aorta and medium branch vessels. Methods A total of 113 consecutive patients enrolled in our institutional FMD registry who received a tailored CTA protocol at our institution between March 2013 and June 2015 were included in this study. Arterial phase contrast-enhanced images were obtained on a dual-source scanner using high pitch and electrocardiogram trigger. Images were analyzed by 2 readers. Results Abnormalities including beading, aneurysm, dissection, and stenosis/occlusion were noted in aortic, renal, mesenteric, and iliac distributions. The most commonly affected vessels were the renal arteries (n = 76 [67%]), followed by the lower extremity/iliac arteries (n = 37 [32%]). Aortic abnormalities were less frequently encountered (n = 3 [3%]), including 1 case with mild dilation (4.2 cm) of the ascending aorta and 2 cases of dissection involving the descending aorta, 1 with mild dilation (4.4 cm). Incremental findings beyond those known at patient intake were commonly noted, including new areas of arterial beading (n = 55 [49%]), new aneurysms (n = 21 [19%]), and new dissections (n = 3 [3%]). Reformatted images were crucial, affecting final assessment in 56% of cases evaluated by reader 1 and 36% evaluated by reader 2. Conclusions Screening chest, abdomen, and pelvis CTA in patients with FMD showed substantial and incremental diagnostic yield. Reformatted images should routinely be included in imaging analysis. Abnormalities in the aorta were not common, so screening of the thoracic aorta may not be indicated.

Screening CT Angiography of the Aorta, Visceral Branch Vessels, and Pelvic Arteries in Fibromuscular Dysplasia

Several key findings in recent years have reshaped our understanding of fibromuscular dysplasia (FMD), an uncommon nonatherosclerotic disease of medium-sized arteries that affects mainly women. While the true prevalence of this disease remains unknown, studies suggest that more people may be affected than previously reported. Better understanding of the clinical manifestations and natural history of FMD and advances in diagnostic imaging have altered the clinical approach to managing patients with this uncommon vascular disease. Although there are a multitude of unanswered questions regarding FMD, this review highlights recent insights and how this information has modified clinical care for those affected.

https://www.ccjm.org/content/ccjom/83/11_suppl_2/S45.full.pdf

Fibromuscular dysplasia: Advances in understanding and management.

Purpose: To analyze the relationship between the new TransAtlantic Inter-Society Consensus (TASC II) infrapopliteal classification and limb outcomes among patients with critical limb ischemia (CLI)...

https://journals.sagepub.com/doi/pdf/10.1177/1526602817704643

Midterm Outcomes After Infrapopliteal Interventions in Patients With Critical Limb Ischemia Based on the TASC II Classification of Below-the-Knee Arteries.

Fibromuscular dysplasia (FMD) is a non-atherosclerotic disease associated with hypertension, headache, dissection, stroke, and aneurysm. The etiology is unknown but hypothesized to involve genetic and environmental components. Previous studies suggest a possible overlap of FMD with other connective tissue diseases that present with dissections and aneurysms. The aim of this study was to investigate the prevalence of connective tissue physical features in FMD. A total of 142 FMD patients were consecutively enrolled at a single referral center (97.9% female, 92.1% of whom had multifocal FMD). Data are reported for 139 female patients. Moderately severe myopia (29.1%), high palate (33.1%), dental crowding (29.7%), and early-onset arthritis (15.6%) were prevalent features. Classic connective features such as hypertelorism, cleft palate, and hypermobility were uncommon. The frequency of systemic connective tissue features was compared between FMD patients with a high vascular risk profile (having had ⩾1 dissection and/or ⩾2 aneurysms) and those with a standard vascular risk profile. A history of spontaneous pneumothorax (5.9% high risk vs 0% standard risk) and atrophic scarring (17.6% high risk vs 6.8% standard risk) were significantly more prevalent in the high risk group, p<0.05. High palate was observed in 43.1% of the high risk group versus 27.3% in the standard risk group, p=0.055. In conclusion, in a cohort of women with FMD, there was a prevalence of moderately severe myopia, high palate, dental crowding, and early-onset osteoarthritis. However, a characteristic phenotype was not discovered. Several connective tissue features such as high palate and pneumothorax were more prominent among FMD patients with a high vascular risk profile.

/pdf/systemic-connective-tissue-features-in-women-with-46yoz0h8gd.pdf

Systemic connective tissue features in women with fibromuscular dysplasia

Ankle-brachial indices (ABIs) are important for the assessment of disease burden among patients with peripheral artery disease. Although low values have been associated with adverse clinical outcomes, the association between non-compressible ABI (ncABI) and clinical outcome has not been evaluated among patients with critical limb ischemia (CLI). The present study sought to compare the clinical characteristics, angiographic findings and clinical outcomes of those with compressible (cABI) and ncABI among patients with CLI. Consecutive patients undergoing endovascular evaluation for CLI between 2006 and 2013 were included in a single center cohort. Major adverse cardiovascular events (MACE) were then compared between the two groups. Among 284 patients with CLI, 68 (24%) had ncABIs. These patients were more likely to have coronary artery disease ( p=0.003), diabetes ( p<0.001), end-stage renal disease ( p<0.001) and tissue loss ( p=0.01) when compared to patients with cABI. Rates of infrapopliteal disease were similar between the two groups ( p=0.10), though patients with ncABI had lower rates of iliac ( p=0.004) or femoropopliteal stenosis ( p=0.003). Infrapopliteal vessels had smaller diameters ( p=0.01) with longer lesions ( p=0.05) among patients with ncABIs. After 3 years of follow-up, ncABIs were associated with increased rates of mortality (HR 1.75, 95% CI: 1.12-2.78), MACE (HR 2.04, 95% CI: 1.35-3.03) and major amputation (HR 1.96, 95% CI: 1.11-3.45) when compared to patients with cABIs. In conclusion, ncABIs are associated with higher rates of mortality and adverse events among those undergoing endovascular therapy for CLI.

https://journals.sagepub.com/doi/pdf/10.1177/1358863X16689831

Non-compressible ABIs are associated with an increased risk of major amputation and major adverse cardiovascular events in patients with critical limb ischemia.

Ellen Brinza

Papers

Screening CT Angiography of the Aorta, Visceral Branch Vessels, and Pelvic Arteries in Fibromuscular Dysplasia

Fibromuscular dysplasia: Advances in understanding and management.

Midterm Outcomes After Infrapopliteal Interventions in Patients With Critical Limb Ischemia Based on the TASC II Classification of Below-the-Knee Arteries.

Systemic connective tissue features in women with fibromuscular dysplasia

Non-compressible ABIs are associated with an increased risk of major amputation and major adverse cardiovascular events in patients with critical limb ischemia.