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Review
. 2016 Nov-Dec;31(6):454-460.
doi: 10.5935/1678-9741.20160090.

Quadricuspid Aortic Valve: A Comprehensive Review

Affiliations
Review

Quadricuspid Aortic Valve: A Comprehensive Review

Shi-Min Yuan. Braz J Cardiovasc Surg. 2016 Nov-Dec.

Abstract

Quadricuspid aortic valve (QAV) is a rare congenital heart disease. The functional status of QAV is predominantly a pure aortic regurgitation. Clinical manifestations of patients with a QAV depend on the functional status of the QAV and the associated disorders. Significant valvular regurgitation and (or) stenosis is often present with subsequent operation performed at the fifth to sixth decade of life. The functional status of QAV is predominantly regurgitant; whereas pure stenotic QAV can be as few as in only 0.7% of the patients. QAV is usually an isolated anomaly, but other congenital heart defects can be present in 18-32% of the patients. About one-fifth of them require a surgical operation. Tricuspidalization is a preferred technique for QAV repair. As not all the patients with a QAV necessarily warrant a surgical operation, decision-making in patient selection and surgical procedure of choice are crucial. Antibiotic prophylaxis against infective endocarditis is necessary in the QAV patients with unequal-sized cusps.

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Conflict of interest statement

No conflict of interest.

Figures

Fig. 1
Fig. 1
Hurwitz & Roberts[] classification of quadricuspid aortic valve with Vali et al.[] supplement.
Fig. 2
Fig. 2
Nakamura et al.[] simplified classification of quadricuspid aortic valve. L=left coronary cusp; N=non-coronary cusp; R=right coronary cusp; S=supernumerary cusp
Fig. 3
Fig. 3
Echocardiography of a type D/type III quadricuspid aortic valve with mild aortic regurgitation.
Fig. 4
Fig. 4
Management and prognosis of patients with a quadricuspid aortic valve[]. AR=aortic regurgitation; AS=aortic stenosis; AV=aortic valve; AVR=aortic valve replacement; IE=infective endocarditis; QAV=quadricuspid aortic valve

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