Unicuspid aortic valve by cardiac computed tomography: the best view is from the mountaintop—a case report

Abstract Background Unicuspid aortic valve (UAV) is a rare valvular heart disease and a challenging diagnosis. Advanced imaging techniques, particularly cardiac computed tomography (CT), appear to be invaluable tools to correctly identify this disease pre-operatively, as this may have an impact on the optimal surgical treatment. Case summary We describe the case of a young patient admitted with heart failure, due to a severely stenotic UAV. Cardiac CT allowed adjusting the imaging plane to the best view in two orthogonal planes to identify the top of the ‘dome’ and to accurately measure the smallest valve opening by planimetry. Surgical inspection confirmed a rare case of acommissural UAV. Discussion Cardiac CT angiography is crucial to understand the complexity of UAV disease and to differentiate the acommissural from the unicommissural type. Accurate positioning of the imaging plane through the smallest valve opening in systole reduces the risk of missing the diagnosis of this rare disease.