Quadricuspid Aortic Valve

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Tudor Vagaonescu, M.D.
Patricia Ambrosio, R.D.C.S.
Paul Haluska, M.D., Ph.D.

Transthoracic short axis view showing the diastolic "X" configuration of the four aortic leaflets.
The right coronary ostium is also visible.

Partially open quadricuspid aortic valve with a distinct rectangular orifice.


Quadricuspid aortic valve not associated with truncal abnormalities is rare with a reported incidence of 0.008% to 0.033%. (European Heart Journal 1988;9:1269-1270) In a surgical/pathological series of 225 patients with pure aortic insufficiency the incidence was 1%. (Mayo Clin Proc 1984;59:853-841)


On pathological descriptions the aortic valve has four cusps: a posterior cusp, a left coronary cusp, a right coronary cusp, and an anterior supernumerary cusp. Raphes join the supernumerary cusp with the left and right coronary cusps. The raphes have been described as shallow and chordlike. The supernumerary cusp can have multiple fenestrations. (Am J Cardiovascular Pathology 1990;3(2):185) The cusps can vary in size, thickness, and pliability. (Am J Cardiovascular Pathology 1990;3(1):91-94)

Associated cardiac abnormalities


The semilunar valves are derived from mesenchymal swellings in the aortic and pulmonary trunk after the truncus arteriosus has been partitioned. It is in the early stages of truncal separation that four subendothelial buds appear instead of three. The presence of a corpus arnatii on all four cusps indicates that the valve resulted from abnormal embryogenesis. (Am J Cardiol 1991;67:323-324)

Excess in the number of the semilunar valves.
From Peacock's 1858 book on Malformations of the Human Heart.

Fig. 1. Four valves at the orifice of the pulmonary artery, the excess being apparently produced by the division of one of the valves at 'a'. The two segments so produced are imperfect and are freely blended together. From a female 75 years of age. The preparation is numbered B 13, in the Museum of the Victoria Park Hospital.

Fig. 2. Four valves at the orifice of the pulmonary artery; the excess consisting in three imperfectly divided segments and one complete segment. The larger fold at 'a', 'a', is attached to the side of the vessel by firm bands. From a man, 45 years of age, who was crushed to death.

Fig. 3. Four valves at the aortic orifice, from a preparation in St. Thomas's Hospital Museum, numbered beta 105. The excess is apparently due to the division of one fold at 'a' into two. The septum between these two segments is very imperfect, being, as seen in fig. 4, perforated by apertures, or displaying portions in which the fibrous tissue is wanting. It is doubtful whether the small body marked b, fig. 3, is an adhesion between the curtains or a supernumerary valve.

Fig. 5. Five valves at the orifice of the pulmonary artery, from a preparation marked B 12 in Museum of the Victoria Park Hospital, removed from a child aged four and a half years. The excess is apparently due to the division of two curtains at 'a' and 6. The supernumerary segments and those adjacent to them are imperfect.

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