Quadricuspid Aortic Valve by Cardiac Magnetic Resonance Imaging

Abstract

Quadricuspid aortic valve (QAV) is a rare congenital cardiac entity. The recognition of QAV has clinical significance as it causes aortic valve dysfunction, commonly aortic regurgitation, and is often associated with other congenital cardiac abnormalities. We showed the important role played by cardiac magnetic resonance imaging in detecting QAV and review the available literature to explain its incidence, diagnosis, classifications, embryology, correlation between morphology of the QAV and its function, associated conditions, and management.

Quadricuspid aortic valve: a rare but important abnormality

“…This classification is important in identifying the different types of juxtaposition of the valves. The majority of patients have one of the first three types—that is, four equal size cusps, as seen in our patient (type A; figure 1A and B and online supplementary video 1), three equal cusps with one smaller cusp (type B) and two equally large with two equally small cusps (type C) 1 4 5. Eighty-four per cent of patients with QAV have abnormal function of the aortic valve such as AR 3.…”

“…Over 50% of patients with QAV may require surgery due to significant AR, particularly among elderly patients due to progressive valvular deterioration. Early diagnosis and recognition of QAV will therefore have great clinical importance in the treatment and follow-up 1 3…”

“…QAV is characterised by the presence of four aortic cusps of varying sizes. Clear delineation of the anatomy of the QAV is difficult but of great clinical importance as these patients may present with progressive aortic regurgitation (AR) and other cardiovascular abnormalities 1 2. We present a case of QAV in an asymptomatic patient who presented with a murmur on routine examination.…”

“…Since first reported in 1862, quadricuspid aortic valve (QAV) remains rare with a reported incidence of 0.008–0.033% on autopsy; however, the actual incidence of QAV is underestimated 1 2. QAV is characterised by the presence of four aortic cusps of varying sizes.…”

Diagnostic accuracy study of routine echocardiography for bicuspid aortic valve: a retrospective study and meta-analysis

“…We excluded one individual with intraoperative confirmation of a unicuspid aortic valve, and seven individuals with aortic valves that exhibited two cusps but three sinuses with three interleaflet triangles indicating an acquired rather than congenital bicuspid structure of the aortic valve. We did not identify other congenital aortic valve malformations such as a quadricuspid aortic valve (9)(10)(11). Therefore, a total of 108 individuals with BAV confirmed during surgical inspection qualified for inclusion in this study.…”