Aim: To present accidental detection of quadricuspid aortic valve (QAV), a rare congenital heart anomaly with an incidence of 0.01–0.04%.

Case presentation: 49-years-old patient came for an examination due to tachycardia and palpitations during physical exertion. Transthoracic echocardiography verified the regular dimensions of the left and right heart cavities, with preserved systolic function of the left ventricle, and mild mitral and tricuspid regurgitation. The existence of QAV was suspected, while moderate aortic regurgitation was verified (pressure half-time 390 ms, vena contracta 5 mm, jet covered 40% left ventricular outflow tract, regurgitant volume 35 mL, regurgitant fraction 36%, effective regurgitant orifice area 0.16 cm²). Transesophageal echocardiography confirmed the diagnosis of QAV (Figure 1).

Conclusion: In QAV, diagnosis, as well as monitoring of aortic regurgitation, are of essential importance because of timely surgical treatment (1, 2).