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The field of coronary artery pathology is overwhelmingly dominated by one disease—atherosclerosis. It is therefore not surprising that insight into the onset of symptomatic coronary artery diseases mainly stems from patients with plaques. Coronary atherosclerosis may lead to relatively benign symptoms, such as stable angina, due to bare stenosis or restenosis of lesions. Life threatening symptoms (unstable angina, myocardial infarction or sudden cardiac death (SCD)) usually arise from plaque disruption and superimposed thrombosis, whereas additional arterial spasm or microembolisation of atherothrombotic materials may worsen the situation. But many non-atherosclerotic coronary diseases may become symptomatic due to similar flow limiting complications, which is clearly of importance for differential diagnosis (table 1).
Autopsy studies on large series of SCD victims—as have been carried out in the Veneto region of Italy, for example—have mapped the age dependent differences in coronary artery disease patterns. In children there is a predominance of congenital lesions and vasculitis (particularly Kawasaki disease),1 although atherosclerotic coronary death may also occur at young ages. Such examples of juvenile onset of atherosclerosis, albeit rare, illustrate the complex genetic backgrounds of the disease.2 In the adult population, myocardial ischaemia is only seldom caused by non-atherosclerotic pathology. Even in those cases, atherosclerosis is usually superimposed on (and sometimes masquerading as) the initial disease, since any significant injury or geometric change of coronary arteries (be it congenital, inflammatory or degenerative) accelerates development of atherosclerosis.
NON-ATHEROSCLEROTIC CORONARY ARTERY PATHOLOGY
Acquired pathology of coronary ostia
Every autopsy on the heart of a patient with suspected ischaemic heart disease should begin with inspection of the coronary ostia.3 Apart from atherosclerotic ostial narrowing, the origin of one or more coronary arteries can be compromised iatrogenically (in the case of aortic valve surgery or because of coronary catheterisation related trauma), or by coronary vasculitis or aortitis with extension to coronary ostia (Takyashu disease), by …