Background: Integrated transthoracic and transesophageal echocardiography enables identification and characterization of a quadricuspid aortic valve anomaly.
Case presentation: A totally asymptomatic 40-year-old white man was referred to our Division of Cardiology after accidental finding of a heart murmur. Transesophageal echocardiography detected a quadricuspid aortic valve characterized by four cusps of equal size and severe aortic valvular regurgitation, without any further anomalies. He underwent a successful aortic valve repair.
Conclusions: Quadricuspid aortic valve anomaly is a rare congenital cardiac defect that can cause progressive valvular complications.
Keywords: Congenital cardiac defect; Quadricuspid aortic valve.