Abstract

Ectopia cordis is a rare disease, with an incidence of 5.5-7.9 cases per million births. Until 2010, 267 cases have been described, most of them (95%) with other associated heart disease. We study the heart diseases associated with 6 cases of ectopia cordis. Depending on the affected area, patients with ectopia cordis are classified into 4 groups: cervical, thoracic, thoracoabdominal and abdominal. The 2 patients described died before 3 days of life, four of them at birth. Three belonged to the thoracic ectopia group and another three to the thoraco abdominal group. All had interventricular communication, associated with double exit of the right ventricle in 3 (50%) and tetralogy of pulmonary fallot-atresia in another 3 (50%). In 2 hearts with double outlet there was mitral valve involvement, parachute valve stenosis and atrial mitral valve. This type of anomalies has not been described previously.For this ectopia cordis can be complete, due to the absence of skin and parietal pericardium, or partial if it exists under the pericardium sternum or on the skin sternum. Most cases are diagnosed in the second trimester, but can be visualized from 10 weeks gestation by means of Doppler ultrasound. With established prenatal diagnosis, cesarean birth is recommended, followed by corrective or palliative surgery.Also vaginal delivery is associated with an increased risk of cardiac compression, damage to herniated viceras or rupture of cardiac chambers. At the time of birth, a fetal echocardiogram is necessary since 80% of cases are associated with intracardiac abnormalities such as tetralogy of fallot. Thus surgical treatment is the only therapeutic option associated with most cardiac protection. Multiple surgical techniques have been described, therefore, this disease can be addressed in one or two surgical times. And the efficacy and possibility of surgery depend on the class of ectopia cordis and the congenital cadiacos defects present or absence of extracardiac malformation

Keywords

cardiac abnormality, parietal pericardium, abdominal group, pulmonary stenosis, skin sternum