Morphology and Function of the Bicuspid Aortic Valve With and Without Coarctation of the Aorta in the Young

This study sought to determine the morphology and function of bicuspid aortic valves (BAVs) with and without coarctation of the aorta (CoA) in a young population. The transthoracic echocardiograms of 117 patients with BAVs and 62 patients with CoA were retrospectively reviewed and compared with normal transthoracic echocardiographic results. In each patient, the area subtended by the aortic cusps and valve and the diameters of the aortic root at different levels were measured, and additionally in each BAV, the type of cusp fusion and the presence and degree of aortic stenosis and/or regurgitation were evaluated. The median age of patients with BAVs was 4 years (range 0 days to 34 years), and the median age of patients with CoA was 1.9 years (range 0 days to 16.5 years). BAVs with right and left coronary cusp fusion were significantly associated with CoA (p <0.0001) and cardiac anomalies (p <0.0001), whereas BAVs with noncoronary and right coronary cusp fusion were affected by valvar dysfunction (p <0.001). Compared with normal tricuspid aortic valves, BAVs had aortic root dilation, even in patients with no hemodynamic disturbance, particularly at the level of the ascending aorta (p <0.0001); the difference was still significant comparing BAVs and CoA with tricuspid aortic valves and CoA (p <0.0001). In conclusion, different morphologies of BAVs are associated with different cardiac abnormalities, valvar function, and aortic root dilation. Although detectable early in life, valvar dysfunction and aortic root dilation progress with age.