Surgical treatment of congenital quadricuspid aortic valve

Objective: To improve the understanding of congenital quadricuspid aortic valve (QAV), explore its echocardiographic diagnostic value and summarize the methods and outcomes of surgical treatments.

Methods: The clinical data of 11 QAV patients from January 2000 to December 2008 were retrospectively analyzed. There were 9 males and 2 females with a mean operative age of (32±16) years (range: 4-55).

Results: In 766 patients undergoing aortic valve surgery, 11 were of congenital quadricuspid aortic valve (1.4%); cardiac pathology: infective endocarditis (n=1), left superior vena cava (n=1), aortic aneurysm (n=1), mitral prolapse (n=1) and tricuspid insufficiency (n=1). The patients of congenital QAV deformity was diagnosed by echocardiography (n=7), misdiagnosed as single valve (n=1), misdiagnosed as bicuspid valve (n=1) and misdiagnosed as rheumatic heart disease (n=2). Type B (n=7), Type A (n=2), Type F (n=1) and Type G (n=1). Eleven patients underwent the procedure of aortic valve replacement. And the concomitant procedures were aortic root broadening (n=1), ascending aortoplasty (n=1), mitral valvuloplasty (n=1) and tricuspid valvuloplasty (n=1).

Conclusions: Quadricuspid aortic valve is rare in clinical practice. And echocardiography plays an important diagnostic role. Surgical replacement of aortic valve is the first-line therapy for these patients.