Coronary artery Ectatic/Aneurysmatic disease: From pathophysiology to management

Coronary artery ectatic/aneurysmatic disease (CAE/CAA) is a rare but clinically significant condition characterized by abnormal dilatation of the coronary arteries, either diffusely (CAE) or focally (CAA). Its pathophysiology is multifactorial, involving atherosclerosis, genetic predisposition, systemic autoimmunity, and infectious triggers, with distinct etiologies in adults (e.g., atherosclerosis, connective tissue disorders) and children (e.g., Kawasaki disease, multisystem inflammatory syndrome in children). CAE/CAA is associated with increased risks of thrombosis, embolization, and acute coronary syndromes, even in the absence of obstructive coronary artery disease. Diagnostic evaluation relies on multimodality imaging, including transthoracic echocardiography (especially in children), computed tomography coronary angiography, cardiovascular magnetic resonance angiography, and invasive coronary angiography with intravascular ultrasound. Each modality offers unique advantages, but challenges such as radiation exposure, contrast use, expertise and availability persist. Therapeutic management remains empirical due to a lack of randomized trials. In adults, risk factor modification, statins, angiotensin converting enzyme inhibitors / angiotensin receptor blockers, calcium channel blockers are commonly used. Optimal strategies for antiplatelet/anticoagulant treatment are currently under debate. In children, anticoagulation and antiplatelet therapy are recommended for large aneurysms, particularly in Kawasaki disease. Future research should focus on elucidating pathophysiology, comparing imaging modalities, and establishing evidence-based treatment strategies to improve outcomes in this complex patient population.