Heart & vascular pathology

Ischemic disease

Nonatherosclerotic coronary artery disease

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Last staff update: 27 December 2021

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PubMed Search: Nonatherosclerotic [title] coronary artery disease

R. Amita, M.D.
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Cite this page: Amita R. Nonatherosclerotic coronary artery disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/heartnonatheroscleroticCAD.html. Accessed May 19th, 2024.
  • 4 - 7% with acute myocardial infarction have nonatherosclerotic coronary artery disease
  • Various disorders, other than atherosclerotic CAD, can reduce or interrupt coronary blood flow

  • Congenital coronary artery anomalies: ostial lesions, passage of a major artery between the walls of the pulmonary trunk and aorta, origin of a major artery from the pulmonary trunk, myocardial "bridges"
  • Coronary aneurysm: congenital or due to angioplasty, arteritis (including syphilis), atherectomy, atherosclerosis, dissection (spontaneous or secondary), laser procedures, mucocutaneous lymph node syndrome, mycotic emboli, trauma
  • Coronary arteritis: due to ankylosing spondylitis, Burger disease (thromboangiitis obliterans), giant cell arteritis, infective endocarditis, leprosy, mucocutaneous lymph node syndrome, polyarteritis nodosa, rheumatoid arthritis, rheumatic fever, Salmonella, syphilis, systemic lupus erythematosus, Takayasu disease, tuberculosis, typhus, granulomatosis with polyangiitis (Wegener's)
  • Coronary artery dissection: primary, due to coronary angiography, cardiac surgery, chest trauma, postpartum women (spontaneous); or secondary due to extension from aortic root dissection
  • Coronary artery thrombosis without underlying atherosclerotic plaque (thrombosis in situ): due to leukemia, polycythemia vera, primary thrombocytosis, sickle cell anemia, thrombocytopenia purpura
  • Coronary emboli: natural causes, iatrogenic causes, and "paradoxical" causes
  • Coronary fistula
  • Coronary ostia: high take off position
  • Dynamic changes in wall of otherwise normal artery (spasm)
  • Encroachment of lumen by disease of arterial wall or adjacent tissues (external narrowing): sinus of valsalva aneurysms and epicardial tumor metastases
  • Fixed luminal obstructions (internal narrowing / ostial narrowing):
    • Aneurysm, saccular, of aorta
    • Aortic dissection extending into coronary ostium (right ostium more common than left)
    • Aortic valve surgery with or without coronary artery canulation
    • Embolus, causing occlusion
    • Fibromuscular hyperplasia associated with methysergide therapy
    • Nonatheromatous, calcific protrusion from sinotubular junction into right or left ostium
    • Obliteration of ostium due to adhesion of free edge of aortic cusp to aortic wall above coronary ostium
    • Occlusive fibroelastois
    • Ostial valve like ridges
    • Supravalvular aortic stenosis with severe intimal thickening
    • Syphilis
    • Takayasu disease
  • Intimal fibrous proliferation: due to cardiac transplantation, fibromuscular hyperplasia of renal arteries, mediastinal irradiation, methysergide, ostial cannulation during cardiac surgery or following aortic valve repIacement, percutaneous balloon angioplasty
  • Myocardial oxygen demand supply disproportion: due to carbon monoxide poisoning, prolonged shock
  • Substance abuse (cocaine): coronary artery vasoconstriction due to intranasal cocaine
  • Systemic metabolic disorders: Fabry disease, homocystinuria, Hunter and Hurler diseases, primary oxalosis, Sandhoff disease
  • Trauma: coronary angiography (embolus, inadvertent ligation, intimal dissection, laceration), nonpenetrating blunt chest wall injury (steering wheel injury), penetration trauma (laceration from stab wound or bullet)
  • Intramural coronary artery disease (small vessel disease): due to amyloid heart disease, cardiac transplantation, collagen vascular disorders (scleroderma, SLE), diabetes mellitus, hypertrophic cardiomyopathy, metabolism abnormalities, neuromuscular disorders (Friedreich ataxia, progressive muscular dystrophy), polyarteritis nodosa, rheumatoid arthritis
Pathophysiology of specific conditions
  • Origin of both right and left coronary arteries from the same sinus of valsalva:
    • Normally, the coronary ostia are round to oval in shape, but in this anomaly, the coronary artery has an acute takeoff angle that makes the ostium slit like in shape
    • With increased cardiac output (e.g., exercise), the aorta dilates, and upon aortic wall stretching this slit like ostium becomes severely narrowed
  • High take off of coronary arteries:
    • Normally, the coronary ostia are located within the sinuses of Valsalva, which permits maximal opportunity for coronary artery diastolic filling
    • Location of the ostia in the tubular portion of the aorta (i.e., high takeoff position) may be associated with decreased coronary perfusion
  • Angina pectoris or acute MI in patient < 20 years old is suggestive of a congenital coronary artery anomaly or a congenital coronary artery aneurysm (Br Heart J 1992;68:601)
  • Asymptomatic older patients with these anomalies are usually discovered by abnormal electrocardiogram, precordial murmur or sudden death
  • Coronary arterial emboli are clinically suspected due to severe chest pain (acute MI) during cardiac catheterization or cardiac surgery or if these conditions are present: active infective endocarditis, atrial fibrillation, cardiac tumor, dilated cardiomyopathy, left ventricular aneurysm, native left sided valve stenosis, prosthetic left sided valve
Case reports
  • 31 year old woman with acute myocardial infarction due to polyarteritis nodosa (Korean Circ J 2010;40:197)
  • 33 year old man and 37 year old woman with non atherosclerotic coronary artery aneurysms (Heart 1997;78:613)
Clinical images

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Coronary artery aneurysm

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Small aneurysm

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Small aneurysm in thyrocervical trunk

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Multiple aneurysmal changes

Gross description
Myocardial bridges ("tunneled" epicardial coronary artery):
  • Coronary arteries which normally course over the epicardial surface of the heart may dip into the myocardium to travel for varying lengths and then reappear on the heart's surface
  • The muscle overlying the intramyocardial segment of the epicardial coronary artery is termed a "myocardial bridge"; the artery coursing within the myocardium is called a "tunneled" artery
  • Congenital coronary artery aneurysms are found most commonly in the right coronary artery
  • Abnormal flow patterns within the aneurysm may lead to thrombus formation with subsequent vessel occlusion, distal thromboembolization and myocardial infarction
Micro description - coronary arteritis
  • Baroldi considers these findings as signs of coronary arteritis: (Baroldi G: Diseases of the coronary arteries, Cardiovascular Pathology, 3rd Edition, 2001)
    • Focal artery necrosis with or without calclfication
    • Acute coronary artery thrombosis or recanalized thrombus unassociated with underlying atherosclerotic plaque
    • Rupture of the vessel wall unassociated with trauma or an interventional procedure
    • Coronary artery wall thickening with secondary luminal narrowing
    • Wall thinning with aneurysm formation
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