Introduction: Ebstein’s anomaly is a heart malformation in which the main defect lies in the apical position of the septal leafl et of the tricuspid valve and right ventricle atrialization. 50% of patients may have associated an atrial septal defect (ASD). Therapeutic solution is, in most of the cases, surgery, with specific indications. Methods: We present the case of a 55 years old lady, diagnosed with Ebstein’s anomaly and ASD at the age of 28. In the natural history of the disease she presented rhythm disturbances and, more recently, heart failure phenomena. Results: Echocardiography and magnetic resonance imaging revealed massive biatrial dilatation, reduced overall kinetics (LV FE 36%, RV FE 21%). Cardiopulmonary test revealed severe decrease in functional capacity. When performing cardiac catheterization and atrial shunt temporary closure of a balloon, we noticed increased systemic saturation, increased cardiac output and reduced pulmonary hyperflow (Qp: Qs pre test 2.5:1 and post test 1.1:1). The defect was closed immediately with two devices Amplatzer Septal Occluder 30 and 19 mm (for the accessory defect). Conclusions: Due to the importance of the biventricular contractile dysfunction, the corrective or palliative surgery (Glenn cavopulmonary anastomosis) was contraindicated in this patient. Palliative therapeutic solution was evident following ASD test occlusion.