Ectopia Cordis is a rare disease with incidence of 5.5 to 7.9 cases per million births. Until 2001, 267 cases have been reported, most of them (95%) associated with other heart diseases. We studied heart disease of 6 cases of Cordis ectopia. Depending on the affected area, patients with Cordis ectopia classified into 4 types: cervical, chest, thoracoabdominal, and abdominal. The 6 patients described died before 3 days of life, four of them at birth. Three belong to the group of ectopia chest and three to the chest and abdomen. All had a ventricular septal defect in the third ventricle (50%) and tetralogy of Fallot, pulmonary atresia in the other 3 (50%). In 2 hearts with double outlet mitral valve involvement, valve stenosis and parachute mitral valve atresia. This type of anomaly has not been described previously.
Patients with serious heart disease of ectopia Cordis, most of them is troncoconal anomalies. Two of our patients have a defect that was not previously described. Although several attempt was made such as surgery, the survival of patients with this heart disease is very low, and most of them die within the first week of life.
