Isolated fibromuscular dysplasia of the coronary arteries with spontaneous dissection and myocardial infarction
Fibromuscular dysplasia (FMD) is a segmental, nonatherosclerotic vascular disease of unknown etiology. Both the renal and extrarenal arterial FMD may have focally deficient or absent muscular media, and spontaneous dissection is a serious complication of this arterial wall abnormality. Although FMD may be found in virtually any small and medium-sized arteries, to our knowledge, FMD of the coronary arteries previously has not been documented convincingly in the literature. This article describes one such unusual occurrence of FMD in the coronary arteries with spontaneous dissection and myocardial infarction.
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The presence of thrombus in spontaneous coronary artery dissection: A systematic review of autopsy findings
2022, American Heart Journal Plus: Cardiology Research and PracticeSpontaneous coronary artery dissection (SCAD) is an important cause of acute coronary syndrome in young women. There is no consensus on optimal treatment, though a conservative approach including antiplatelet agents is commonly used. We hypothesized that most cases of SCAD would not demonstrate true lumen thrombus in the dissected artery, suggesting that anti-platelet agents might not have a role in the treatment of SCAD.
We conducted a systematic review of the published literature through March 2022 to identify pathology images from individuals who died of SCAD. The images were independently reviewed by a pathologist to assess for the presence of thrombus and inflammatory cells.
We identified 40 cases from 34 publications with available pathology images and found only one case of true lumen thrombus. Additionally, we found that 53% of cases involved eosinophilic inflammation.
The role of antiplatelet agents in the treatment of SCAD should be re-evaluated. Further studies are needed to better understand the significance and treatment implications of eosinophilic inflammation.
Coronary Angiographic Manifestations and Outcomes in Spontaneous Coronary Artery Dissection Patients With and Without Fibromuscular Dysplasia
2021, Canadian Journal of CardiologyFibromuscular dysplasia (FMD) is widely recognized as an important predisposing condition for spontaneous coronary artery dissection (SCAD). However, it remains unclear in SCAD patients with coexistent extracoronary FMD whether SCAD can be attributed to coronary FMD.
We retrospectively analyzed consecutive patients enrolled in our Vancouver SCAD registries between September 2009 and October 2019 who were screened for extracoronary FMD. We reviewed coronary angiograms for manifestations of coronary FMD that were previously described (ie, irregular stenosis, smooth stenosis, dilatation/ectasia, and severe tortuosity). Outcome of interest was major adverse cardiovascular event (MACE).
We included 346 SCAD patients, of these, 250 (72.3%) had extracoronary FMD. Patients with FMD were older (54.6 ± 9.5 vs 51.7 ± 9.8 years) and more likely to have prior history of myocardial infarction (7.2% vs 1.0%, P = 0.047) and stroke (4.4% vs 0%, P = 0.081) compared with non-FMD patients. On coronary angiography, severe tortuosity was more prevalent in patients with extracoronary FMD (58.4% vs 36.5%, P < 0.001). Rates of irregular stenosis, smooth stenosis, and dilatation/ectasia were numerically higher in patients with extracoronary FMD, but differences were not significantly different. The rate of MACE at median follow-up of 807 (interquartile range, 392-1096) days was not different between groups (19.6% vs 15.6%; non-FMD as a reference: hazard ratio 1.44; 95% confidence interval, 0.76-2.71, P = 0.261).
SCAD patients with extracoronary FMD were more likely to have coronary FMD manifestations on angiogram, especially severely tortuous vessels, compared with those without extracoronary FMD, with similar clinical outcomes. This may suggest that SCAD can occur at sites of pre-existent subclinical coronary FMD.
La dysplasie fibromusculaire (DFM) est largement reconnue comme un facteur de risque important de la dissection spontanée de l’artère coronaire (DSAC). Cependant, chez les patients présentant une DSAC atteints de DFM extracoronaire coexistante, il n’est pas clair si la DSAC peut être attribuée à la DFM coronaire.
Nous avons analysé rétrospectivement des patients consécutifs inscrits à nos registres de DSAC de Vancouver entre septembre 2009 et octobre 2019 qui ont été soumis à un dépistage de la DFM extracoronaire. Nous avons examiné les coronarographies à la recherche des manifestations de la DFM coronaire décrites précédemment (c.-à-d. sténose irrégulière, sténose lisse, dilatation/ectasie et tortuosité grave). Le résultat d’intérêt était l’événement cardiovasculaire indésirable majeur (ECIM).
Nous avons inclus 346 patients présentant une DSAC, parmi lesquels 250 (72,3 %) étaient atteints de DFM extracoronaire. Les patients atteints de DFM étaient plus âgés (54,6 ± 9,5 vs 51,7 ± 9,8 ans) et plus susceptibles d’avoir des antécédents d’infarctus du myocarde (7,2 % vs 1,0 %, p = 0,047) et d’accident vasculaire cérébral (4,4 % vs 0 %, p = 0,081) que les autres patients. Sur la coronarographie, la tortuosité grave était plus fréquente chez les patients atteints de DFM extracoronaire (58,4 % vs 36,5 %, p < 0,001). Les taux de sténose irrégulière, de sténose lisse et de dilatation/ectasie étaient numériquement plus élevés chez les patients atteints de DFM extracoronaire, mais ces différences n’étaient pas significatives. Le taux d’ECIM après une durée médiane de suivi de 807 jours (intervalle interquartile : 392-1096) n’était pas différent entre les groupes (19,6 % vs 15,6 %; patients non atteints de DFM comme référence – risque relatif : 1,44; intervalle de confiance à 95 % : 0,76-2,71, p = 0,261).
Les patients présentant une DSAC atteints de DFM extracoronaire étaient plus susceptibles de présenter des manifestations de DFM coronaire à l’angiographie, en particulier des vaisseaux gravement tortueux, que ceux qui ne présentaient pas de DFM extracoronaire, avec des résultats cliniques similaires. Cela laisse supposer que la DSAC peut survenir sur des sites de DFM coronaire subclinique préexistante.
Spontaneous Coronary Artery Dissection and Fibromuscular Dysplasia: Chicken or Egg? Which Comes First?
2021, Canadian Journal of CardiologySpontaneous Coronary Artery Dissection: JACC State-of-the-Art Review
2020, Journal of the American College of CardiologyOver the past decade, spontaneous coronary artery dissection (SCAD) has emerged as an important cause of myocardial infarction, particularly among younger women. The pace of knowledge acquisition has been rapid, but ongoing challenges include accurately diagnosing SCAD and improving outcomes. Many SCAD patients experience substantial post-SCAD symptoms, recurrent SCAD, and psychosocial distress. Considerable uncertainty remains about optimal management of associated conditions, risk stratification and prevention of complications, recommendations for physical activity, reproductive planning, and the role of genetic evaluations. This review provides a clinical update on the diagnosis and management of patients with SCAD, including pregnancy-associated SCAD and pregnancy after SCAD, and highlight high-priority knowledge gaps that must be addressed.
Coronary artery myointimal dysplasia in patients with pheochromocytoma—possible causal relationship: pathophysiology and clinical implication with reference to Takotsubo cardiomyopathy and spontaneous coronary dissection
2018, Cardiovascular PathologyMyocardial damage in catecholamine cardiomyopathy, characterized by patchy myocyte necrosis commonly with contraction band appearances, interstitial fibrosis, and varying degrees of inflammatory infiltrates, has been well documented. However, coronary vascular pathology has not been recognized. Autopsy of a 43-year-old housewife who died of acute apical anteroseptal myocardial infarction revealed the incidental finding of a left adrenal pheochromocytoma. The epicardial and intramyocardial median- and small-sized coronary arteries exhibited myointimal dysplasia in the form of fibroblastic proliferation in the intima and media, resulting in thickened dysplastic vessels with marked luminal narrowing, occasionally leading to near-total occlusion. The distal left anterior descending artery showed features of recanalization after prior embolic occlusion. The density and severity of vascular involvement revealed a decreasing gradient from apical to basal region, mainly affecting the left ventricle, but the proximal coronary arteries were minimally affected. Myointimal dysplasia was not seen in control cases of hypertensive heart, and despite its presence in hearts with hypertrophic cardiomyopathy, it lacked the distinctive pattern of distribution and the epicardial vessels are uninvolved.
Myointimal dysplasia probably results from reactive fibroplasia in response to the vasoconstrictive effect of circulating or local neurosecretory catecholamine and appears crucial in the pathogenesis of various types of catecholamine cardiomyopathy, including Takotsubo or stress cardiomyopathy. Together with the direct catecholamine cardiotoxicity, they result in diffuse microscopic ischemic necrosis and fibrosis. Depending on the type of catecholamine overproduction and action, together with the characteristic anatomic distribution and density of the various types of adrenergic receptors in the ventricles, different regions of the heart are variously affected so that different patterns of ventricular dysfunction are produced, with the subsequent angiographic appearances ranging from apical through midventricular to basal ballooning. Additional complications from the myointimal dysplasia include spontaneous coronary dissection, acute myocardial infarction, and superimposed thrombus formation in the dysplastic vessels and dyskinetic ventricle, with the risk of further damage from embolic events.
Spontaneous Coronary Artery Dissection: Cardiac Manifestations of Vascular Disease
2018, Progress in Cardiovascular DiseasesSpontaneous coronary artery dissection (SCAD) is an increasingly recognized cause of acute coronary syndrome (ACS) in young women who otherwise do not have traditional risk factors for coronary artery disease. Though previously considered to be a rare occurrence and primarily associated with pregnancy, contemporary series have demonstrated that SCAD may account for 35% of ACS in women under the age of 50 years, and peripartum SCAD accounts for the minority of cases. Importantly, an association between SCAD and arterial abnormalities in non-coronary arterial distributions has been described. The most common of these arterial abnormalities is fibromuscular dysplasia. This manuscript will briefly review the epidemiology, diagnosis, and treatment of SCAD and provide an overview of how SCAD may be a cardiac manifestation of an underlying vascular disease in the majority of patients.
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from the Department of Pathology, Mayo Clinic and the Mayo Graduate School of Medicine, Rochester, Minnesota.