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Page 4 of 14 Barioli et al. Vessel Plus 2024;8:13 https://dx.doi.org/10.20517/2574-1209.2023.68

vasculitis. However, the most frequent clinical manifestation is ischemic heart disease, including exertional
angina, acute coronary syndromes, and sudden death. These clinical manifestations may be determined by
the presence of concomitant obstructive CAD. Nevertheless, exertional angina and positive exercise stress
tests have been observed even in the absence of significant atherosclerosis and have been associated with
flow disturbances within the aneurysm, formation of endoluminal thrombi with subsequent embolization,
[19]
and microvascular dysfunction . Less frequent symptoms are those resulting from compression of
contiguous cardiac or extracardiac structures or from aneurysm rupture (into a low-pressure cavity or
[22]
pericardium) [14,20,21] . However, spontaneous rupture is a rare occurrence .
Assessment
Coronary angiography is the method of choice used for the diagnosis and characterization of aneurysms.
Indeed, it can assess the size, location, extent, and morphology of CAAs and CAE, as well as associated
obstructive coronary artery disease, if present [Figure 2A]. Proper visualization of aneurysms with
angiography is often hampered by the delayed progression of the injected contrast medium, the presence of
[3]
segmental backflow, and contrast stasis in the dilated segment . Intravascular ultrasonography (IVUS) has
proven to be a valuable adjunctive tool for determining aneurysm size, vessel wall characteristics, presence
of stenosis or thrombotic apposition . In addition, it facilitates stent sizing if percutaneous coronary
[23]
intervention (PCI) is planned and allows intra-procedural stent optimization. Optical coherence
tomography has a limited role in assessing CAAs, but in small aneurysms, it can provide important
information such as the differentiation between true and pseudoaneurysms and the state of the coronary
arteries in adjacent segments .
[24]

Coronary computed tomography (CT) is a noninvasive diagnostic tool that allows accurate assessment of
aneurysm size, wall characteristics, anatomical relationships, and degree of thrombosis or calcifications [25-27]
[Figure 2B]. Beyond the initial diagnostic characterization, coronary CT is useful for monitoring the
progression of aneurysms over time; however, its use in patient follow-up raises questions about the
possible risks associated with ionizing radiation.

MANAGEMENT
Appropriate management for CAA and CAE is still largely unsettled, and should be individualized
according to clinical presentation, aneurysm size and characteristics, disease progression over time, and
association with atherosclerotic disease . Treatment modalities include medical therapy and
[9]
revascularization with either PCI or surgery [Table 1]. A recent retrospective cohort study showed a
relatively high MACCE rate across all treatment groups, with a non-statistically significant trend favoring
surgery .
[28]
Medical management
Medical management of CAA, especially in the absence of concomitant atherosclerotic coronary stenosis or
thrombotic complications, is still being debated. The management of CAA in the setting of Kawasaki
disease falls beyond the purposes of the present paper; however, intravenous administration of
immunoglobulins at the early stages of the disease has been proven to positively impact prognosis [29,30] . In
adult subjects with CAA, as for non-CAA patients, search for coronary artery disease risk factors should be
pursued to modify or remove them, wherever possible.

Some Authors reported on the possible interplay of the renin-angiotensin-aldosterone system (RAAS) and
inflammation on aneurysm development, advocating both RAAS modulation and statin therapy as potential
strategies to prevent disease progression [31-34] . However, no formal recommendations can be provided, given
the lack of prospective and high-quality evidence.