A Rare Congenital Cardiac Anomaly Presenting with Amaurosis Fugax: Quadricuspid Aortic Valve

Quadricuspid aortic valve (QAV) is a rare cause of aortic regurgitation. Most cases are detected incidentally during echocardiography, angiography, autopsy or surgery. It may also be associated with other congenital anomalies of the heart. A 27-year-old male was admitted to our department to evaluate the etiology of amaurosis fugax. On physical examination, his blood pressure was 125/85 mmHg, and an evident diastolic murmur of grade 2/6 was heard at the third left intercostal space. Results of electrocardiography and chest radiography were normal. Transthoracic and transoesophageal echocardiography revealed a quadricuspid aortic valve, a mild aortic regurgitation. The importance of recognizing this condition lies in the fact that more than 50% of people with QAV require valve replacement in the fifth or sixth decade of life, because of worsening aortic regurgitation.