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Fibromuscular Dysplasia and Other Arteriopathies Associated with Spontaneous Coronary Artery Dissection

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Fibromuscular Dysplasia and Other Arteriopathies Associated with Spontaneous Coronary Artery Dissection 4/17/2019 Fibromuscular Dysplasia and other Arteriopathies associated with Spontaneous Coronary Artery Dissection DEBORAH HORNACEK, MD RPVI CLEVELAND CLINIC ROBERT AND SUZANNE TOMSICH DEPARTMENT OF CARDIOVASCU LAR MEDICINE SECTION OF VASCULAR MEDICINE Disclosures •No disclosures. 1 4/17/2019 Overview •Brief introduction to Spontaneous Coronary Artery Dissection (SCAD) •How I approach the diagnostic evaluation of a patient with a SCAD event •Identifying Fibromuscular Dysplasia (FMD) and other arterial disorders associated with SCAD • FMD • Select collagen vascular disorders Spontaneous Coronary Artery Dissection (SCAD) •Refers to spontaneous tear of the coronary artery that is NOT iatrogenic or traumatic, generally in the absence of atherosclerotic disease •Increasing recognition of SCAD as a cause of MI • Predominantly female patients • Young • “Healthy” – paucity of traditional cardiovascular risk factors • Peripartum/postpartum MI •Increasing detection of SCAD is the result of… • Improved intracoronary imaging: OCT + IVUS • Increased clinical suspicion + better pattern recognition 2 4/17/2019 Why is it important to identify SCAD? •Atherosclerosis •SCAD • Immediate management of MI • Immediate management of MI • How do you treat the dissection? • Medication management of post-MI patient • Medication management of post-MI patient • Differs from traditional atherosclerotic disease • Whole body disease / panvascular • Whole body disease / panvascular • Disorder of young patients • Lifestyle: exercise / activity limitations • Impact on family planning • Genetic basis: need for family screening? • One time event (hopefully) but impact across the continuum of life Post-Acute Evaluation History Physical Exam Testing • Physical activity prior to or during onset of • Cardiopulmonary exam, Peripheral pulse exam, Neuro • Carotid artery ultrasound, renal artery symptoms; stressful or emotional triggers during exam, Abdominal exam ultrasound onset of symptoms • Face exam • Spacing of eyes (hypertelorism), anisocoria -> • Head to pelvis angiography (MRA or • Prior episodes of symptoms; other episodes Horner’s syndrome, blue sclera, palpebral fissure CTA head/neck, CTA chest/abd/pelvis) suggesting vascular events (ie TIA) abnormalities (large or protruding eyes); abnormalities of uvula (wide, long/narrow, bifid); • Referral to Genetic Counselor and • Details of other medical history or symptoms: dental crowding; abnormalities of hard palate or jaw; genetic arteriopathy panel headaches/migraines, tinnitus, vision impairment narrow nasal bridge; malar hypoplasia; micrognathia, / abnormalities of lens, bowel perforations, short chin or narrow chin; small ear pinnae (“elfish” pneumothorax, difficulty healing, easy features) bruising/bleeding, polycystic kidney/liver disease, • Skin autoimmune disorder, episodic BP elevations, • Abnormal scars (not keloids—“cigarette paper” substance abuse, herbal medications, Rx meds atrophic scars, impaired healing), striae, velvety associated with dissection (triptans, stimulants) texture, translucent/increased visible vascularity, laxity/elasticity • Family history: known SCAD, other dissections, • Skeletal/joint exam aneurysms, MI/stroke, sudden cardiac death, • Large or small joint laxity; scoliosis; dislocations autoimmune disorders • Pectus deformity (excavatum or carinatum) • Height, long digits, short neck (Turner syndrome) 3 4/17/2019 Arteriopathies •Non-inflammatory, non-atherogenic conditions which affect the fragility of vessels • These conditions are not vasculitis; vasculitis is rarely implicated in dissection •Fibromuscular Dysplasia • Multifocal vs unifocal •Heritable Connective Tissue Disorders (HCTD) or Collagen Vascular Disorders • Syndromes affecting connective tissue of many organ systems: cardiovascular, skin, skeletal, eyes, lungs • Most typically recognized: • Marfan syndrome • Ehlers-Danlos syndrome • Loeys-Dietz syndrome Fibromuscular Dysplasia (FMD) •Non-inflammatory, non-atherosclerotic disorder of medium-sized arteries • Extracranial cerebrovascular circulation (carotid and vertebral arteries) involved in ~75% of cases. • Renal arteries involved in 75-80% of cases; 65% of patients with renal FMD also have carotid/vertebral involvement (and vice versa) • Additionally: visceral arteries, external iliac arteries, coronaries, brachial, radial, ulnar, superficial femoral arteries can all be involved. Association with aneurysms (intracranial, splanchnic). •Disease presentation is dependent on arterial segment involved • Important cause of TIA, stroke. Can present as dissection, aneurysm, stenosis, occlusion, beading, tortuosity. • Accounts for 5-10% of cases of renovascular HTN in adult patients <60yo; however, many patients with renal involvement do not have renovascular HTN (do not have hemodynamically significant renal stenosis) • Accounts for 35-50% of renovascular HTN in children • Due to increasing imaging in the population –and practice of screening other arterial beds when FMD is identified in one vessel— asymptomatic disease is increasingly detected 4 4/17/2019 Fibromuscular Dysplasia •Approximately 90% (or more) cases are reported in women •Prevalence is likely higher than we once thought • Review of angiographic data in asymptomatic potential kidney donors: 4.4% prevalence • Cardiovascular Outcomes in Renal Atherosclerotic Lesions (CORAL) trial: known FMD was exclusion criterion, incident renal FMD dx in 5.7% of total study participants (8.8% of females who were enrolled) •Previously described as disease of young women, however, FMD is being identified in older patients with increasing frequency • United States FMD Registry: mean age at diagnosis 52yo • Recent data suggesting elderly patients >65yo may have milder disease course Olin JW et al. The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients. Circulation. 2012;125(25):3182. Gornik HL et al. First international consensus on the diagnosis and management of fibromuscular dysplasia. J Hypertens. 2019;37(2):229. Shivapour DM et al. Epidemiology of fibromuscular dysplasia: A review of the literature. Vasc Med. 2016;21(4):376. Fibromuscular Dysplasia •Etiology is unknown: genetic basis + hormonal/environmental factors • Suspected hormonal influences as majority of patients are women of childbearing age • Studies have suggested autosomal inheritance with variable penetrance •Recent data: association between FMD and a single nucleotide variant in the PHACTR1 gene (phosphatase and actin regulator 1 gene, rs9349379-A allele) • Also a risk locus for coronary artery disease (rs9349379-G), migraine headache and cervical artery dissection (rs9349379-A) • Recent data in 2019 now showing association of PHACTR1 with SCAD (rs9349379-A) Perdu et al. Inheritance of arterial lesions in renal fibromuscular dysplasia. J Hum Hypertens. 2007;21(5):393. Kiando SR et al. PHACTR1 Is a Genetic Susceptibility Locus for Fibromuscular Dysplasia Supporting Its Complex Genetic Pattern of Inheritance. PLoS Genet. 2016;12(10):e1006367. Debette S et al. Common variation in PHACTR1 is associated with susceptibility to cervical artery dissection. Nat Genet. 2015 Jan;47(1):78-83. Adlam D et al. Association of the PHACTR1/EDN1 Genetic Locus With Spontaneous Coronary Artery Dissection. J Am Coll Cardiol 2019;73:58–66. 5 4/17/2019 Fibromuscular Dysplasia •FMD types: classification is by angiographic appearance (simplified from former histologic classification scheme) • Multifocal FMD: classic “string of beads.” Most common type (>80% cases), correlates to histologic medial fibroplasia • Appearance is due to alternating fibromuscular webs and areas of dilatation. The internal elastic lamina is absent at areas of dilation. • Focal FMD: circumferential or tubular stenosis. Not frequently seen/recognized. Appearance is associated with histologic types: intimal fibroplasia, medial hyperplasia, periarterial hyperplasia. • Some question of whether this represents a different disease. Fibromuscular Dysplasia Case 1: Mrs. M 52yo white female referred for SCAD with STEMI 9/25/2018, with prior hx of STEMI 4/9/2017 o 4/9/2017: STEMI, unknown mechanism o 9/25/2018: recurrent STEMI o Woke at 4am from sleep with chest pain; presented to ER o Returned home from evening walk with her husband, o EKG: ST elevations in inferior/posterior leads and developed increasing chest and jaw pain; presented to ER anterior/lateral leads, with ST depression in leads 1 and o EKG: ST elevations in anterior leads aVL o Positive troponins o Positive troponins o LHC: normal coronaries (no ASO), identified dissection in o LHC: reported as normal coronaries, apical WMA, LVEF LAD, LVEF 35% 40% o Transthoracic echo the following day: LVEF 45-50% with o Transthoracic echo the following day: LVEF 55-60% anterior and apical WMA o Course complicated by AV fistula at left radial access site, surgically repaired o Medical management: o aspirin 81mg QD, clopidogrel 75mg QD, metoprolol o Original event attributed to “stress” succinate 75mg QD o Mother suffering from terminal IPF; taking care of o Cardiac rehab longtime HHC patient with ALS o 11/2018 appointment: currently asymptomatic 6 4/17/2019 Case 1: Mrs. M •Additional PMH: • Physical Exam NORMAL! • Hx of mild dyslipidemia, improved lipid profile with diet • BP [R] 113/66, [L] 115/57| HR 76 | Wt 55.7kg| Ht 5’ 7.5” | BMI 18.9 changes (Esselstyn plant based diet) • Gen: Cooperative, in no acute distress, alert. • Migraine headaches (does not use triptans) • Skin: Normal with no rashes or lesions. Warm and dry. • Anxiety
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