Retinal Artery Embolism Considering Bicuspid Aortic Valve

The aortic valve has usually three cusps. The aortic valve regulates blood flow, and it opens to let blood flow from the left ventricle to the aorta (systole), and it closes to prevent blood from flowing in the wrong direction (diastole).

Bicuspid aortic valve (BAV) is a common congenital disease in which the aortic valve has only two valves. Due to this disorder and non-parallel opening and closing, the valve will not function properly. However, the function is usually proper that patients have normal clinical conditions for several years without noticing any visible signs or symptoms. This disease is presented as a sporadic or hereditary form, and it has been observed in 9% of the first-degree relatives of the affected. BAV is frequently discovered during the echocardiography performed for another indication or after developing complications. Affected patients are at risk of progressive aortic stenosis, aortic regurgitation, aortopathy with aortic aneurysm or dissection and endocarditis. There are also associated congenital anomalies including coarctation of aorta. Due to the progressive and asymptomatic nature of the disease, annual echocardiography to check valve function and CT scan or magnetic resonance imaging (MRI) to check aortic aneurysm is recommended.

Calcium deposits gradually on the valve in patients with different degrees of aortic valve stenosis due to damage to the endothelium and degenerative valvular tissue. One of the uncommon but serious complications of BAV is systemic embolism owing to these deposits to various organs, including cerebral vessels and retinal artery. Embolism may occur even earlier and from a valve that is not yet ready for surgery according to existing signs and symptoms. In these cases, clinical decision-making for early valve replacement can be challenging.

This study investigated a case of a young athlete male presenting with sudden onset unilateral visual loss. The echocardiography results showed degenerative changes and calcification of the aortic valve considering BAV without severe stenosis that was identified incidentally.