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Congenital malformations of the mitral valve in adults are rare, and may be associated with other congenital heart diseases, such as atrial septal defects (ASD).Cyanosis in a patient with ASD without pulmonary vascular disease, removes the possibility of Eisenmenger syndrome and leads us to look for other possible causes. We report the case of a 23-year-old woman, who consults for dyspnea and cyanosis, diagnosed with right heart failure secondary to adult congenital heart disease without pulmonary vascular disease, so was decided to realized atrioseptoplasty with correction of anomalous venous drainage, mitral and tricuspid valve replacement. In six-month outpatient follow-up, the patient improved symptomatology with absence of cyanosis, decrease in the dilatation of the right chambers, and borderline right ventricle systolic function.