Bicuspid aortic valve disease,Die bikuspide Aortenklappe

Bicuspid aortic valve disease (BAV) is one of the most frequent congenital heart defects, and can be associated with other cardiovascular lesions. The most common complication is an associated valvular dysfunction consisting of aortic stenosis, aortic regurgitation, or infective endocarditis. Furthermore, it can lead to dilation of the ascending aorta with the potential complication of aortic dissection. Histopathological alterations such as cystic media necrosis with loss of smooth muscle cells indicate that BAV may be a systemic disease. Cardiologic imaging modalities such as echocardiography and MRI or CT of the aorta are necessary for timely detection of cardiovascular complications, and for timing of prophylactic aortic surgery. Further clinical studies are substantial for better understanding of the disease and to define optimal timing for interventions. This article focuses on current clinical guidelines, summarizes the pathophysiological and genetic basis of BAV, and outlines therapeutic strategies.