Background: Congenital quadricuspid aortic valve is rarely seen during aortic valve replacement (AVR). The diagnosis and treatment of the disease were reported in 11 cases.
Methods: Eleven patients (nine men and two women, mean age 33.4 years) with quadricuspid aortic valve were retrospectively evaluated. Medical records, echocardiograms and surgical treatment were reviewed.
Results: In accordance with the Hurwitz and Roberts classification, the patients were classified as type A (n = 2), type B (n = 7), type F (n = 1) and type G (n = 1). Three patients were associated with other heart diseases, including infective endocarditis and mitral prolaps, left superior vena cava, aortic aneurysm. All had aortic regurgitation (AR) except two with aortic stenosis (AS), detected by color-flow Doppler echocardiography. The congenital quadricuspid aortic valve deformity in seven patients was diagnosed by echocardiography. All patients underwent successful aortic valve replacement.
Conclusion: Quadricuspid aortic valve is a rare cause of aortic insufficiency, while echocardiography plays an important role in diagnosing the disease. Aortic valve replacement is the major therapy for the disease.