Coronary artery fistulas are rare vascular anomalies that may cause chronic left-to-right shunting, cardiac chamber remodeling, and heart failure. We report a 72-year-old woman with a left main coronary artery-to-right atrium fistula draining through a giant aneurysmal atrial septal artery and accompanied by severe secondary mitral regurgitation. Echocardiography and cardiac computed tomography showed a 19 mm fistulous tract, a 31 × 30 mm aneurysmal vascular structure with a 5 mm outlet to the right atrium, and severe mitral regurgitation with an effective regurgitant orifice area of 0.56 cm2 and a regurgitant volume of 89 mL. Owing to high surgical risk and complex septal anatomy, a staged transcatheter strategy was adopted. The fistula was first closed using an AMPLATZER VASCULAR PLUG II and coil embolization, with satisfactory occlusion and mild residual flow. Two months later, mitral regurgitation worsened despite medical therapy. A three-dimensional printed model was used to plan a safe transseptal puncture trajectory, and transcatheter edge-to-edge repair was performed with two MitraClip XTR devices. Mitral regurgitation was markedly reduced, with sustained symptomatic, echocardiographic, and biomarker improvement at 12 months. This case suggests that sequential transcatheter fistula closure and mitral valve repair, supported by multimodality imaging and three-dimensional printing, may be feasible in carefully selected high-risk patients with complex coronary fistula anatomy and severe mitral regurgitation.
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