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Congenital malformations of the aortic valve are not uncommon. Bicuspid aortic valve is the most common with an incidence of 2%. Quadricuspid valves are rare. Reported incidence varies between 1 in 1000 to 1 in 10 000. Previously this anomaly was detected accidentally during surgery or during necropsy. Now this condition is increasingly being detected with the advent of echocardiography.
A 5 month old male child was referred for evaluation of a cardiac murmur detected on routine physical examination. The child did not have any cardiac symptoms. Physical examination was unremarkable except for a short grade 2 systolic murmur along the left sternal border.
Transthoracic echocardiography revealed a quadricuspid aortic valve with four approximately equal sized cusps providing the classical X shaped diastolic short axis commissural pattern (panel A). Full excursion of the leaflets to the margins of the aortic annulus was noted in systole (panel B). The sinotubular junction and ascending aorta were normal. There was no significant stenosis or regurgitation of the aortic valve.
Echocardiography did not reveal any other notable abnormality in the child.