Coronary Artery Aneurysms, Arteriovenous Malformations, and Spontaneous Dissections-A Review of the Evidence

Coronary artery aneurysms (CAAs), coronary arteriovenous malformations (CAVMs), and spontaneous coronary artery dissections (SCADs) are rare clinical entities, and much is unknown about their natural history, prognosis, and management.

A systematic search of MEDLINE, Embase, and Cochrane Library databases was performed in March 2023 to identify published papers related to CAAs, CAVMs, and SCADs.

CAAs are found in 0.3% to 12% of patients undergoing angiography and are often associated with coronary atherosclerosis. They are usually asymptomatic but can be complicated by thrombosis in up to 4.8% of patients and rarely by rupture (0.2%). CAAs can be managed medically, percutaneously with stents or coil embolization, and surgically. The most common surgical procedure is ligation of the aneurysm, followed by coronary artery bypass grafting. The incidence of CAVMs is 0.1% to 0.2% in patients undergoing angiography, and they are most likely associated with congenital abnormal development of the coronary vessels. The diagnosis of CAVMs is usually incidental. Surgical or percutaneous intervention is indicated for patients with large CAVMs, which carry a potential risk of myocardial infarction. SCADs represent 1% to 4% of all acute coronary syndromes and typically affect young women. SCADs are strongly correlated with pregnancy, suggesting the role of sex hormones in their pathogenesis. Conservative management of SCAD is preferred for stable patients without signs of ischemia as spontaneous resolution is frequently reported. Unstable patients should undergo revascularization either percutaneously or with coronary artery bypass grafting.

Further evidence regarding the management of these rare diseases is needed and can ideally be derived from multicenter collaborations.