Multimodal imaging in young male with bicuspid aortic valve, right-sided aorto-atrial fistula and single coronary artery

A 35-year-old male, without significant cardiovascular history, presented with recurrent palpitations. Initial echocardiographic evaluation demonstrated eccentric left ventricular hypertrophy, mild systolic dysfunction, suspicion of a ventricular septal defect, bicuspid aortic valve, and right ventricular dilation. Transesophageal echocardiography revealed an aneurysmal dilation of the right coronary sinus with an aorto-atrial/ventricular fistula, further confirmed by contrast-enhanced computed tomography angiography. Coronary angiography identified a single anomalous coronary artery with left dominance and absence of the right coronary artery. Surgical repair successfully closed the fistula, with mild post-operative aortic regurgitation. Follow-up at one year indicated normalization of cardiac dimensions and function, with stable moderate aortic valve regurgitation. Genetic sequencing found no identifiable mutations. Regular monitoring was recommended due to the potential risk of complications related to the bicuspid aortic valve and coronary anomaly.