SummaryRelation of two cases of dextrocardia with situs inversus totalis, complicated by associated cardiovascular malformations. In both cases, the catheterization of the heart cavities showed the existence of a single auricle; one of our cases also presented two superior vena cava related by a venous brachio-cephalic branch and a stenosis of the pulmonary artery. Two similar cases were reported by Taussig and Cournand.