Unlabelled: While bicuspid aortic valve (BAV) is a common congenital cardiac anomaly, quadricuspid aortic valve (QAV) is rare. The usual three-leaflet aortic valve is characterized by engineering advantages with superior long-term performance, and thus, degenerative changes and significant functional deterioration appear at advanced age.

Aim: Evaluation of long-term performance, similarities, and differences between QAV and BAV.

Methods: Screening of 19 000 consecutive echocardiographic studies was performed.

Results: BAV was reported in 131 subjects with a prevalence of 0.7%, while QAV was seen in 11 with a prevalence of 0.06%, P < .00001. Age of BAV patients was younger, 45 ± 20 years vs 62 ± 17 years in QAV, P < .05, with higher proportion of females in those with QAV, 40% vs 30%. Chamber diameters were similar in both groups. Higher atrial contraction-A-wave mitral peak velocities and longer E-wave deceleration times were found in subjects with QAV, P < .05 for both. Dilated ascending aorta was found in 25% of patients with BAV and in 18% of those with QAV, P = .2. Moderate and severe aortic valve stenosis were found in 21% of patients with BAV and in 27% of those with QAV, P = ns. More than moderate aortic regurgitation was found in 15.5% of BAV patients and in 9% of QAV, P = ns. Aortic valve infective endocarditis was found in 1.5% of BAV patients and in 9% of those with QAV.

Conclusions: BAV is a common congenital anomaly, while QAV is rare. Similar prevalence of significant valve disease and aortopathy was found in both anomalies, though at younger age in BAV patients.

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http://dx.doi.org/10.1111/echo.14471DOI Listing

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