Ectopia cordis totalis: Case in images

ABSTRACT

@#Ectopia cordis (EC) is a rare congenital malformation characterized by a complete or partial extrathoracic presentation of the heart,1 2 with an incidence of only 6 to 8 in a million live births globally.3 4 It occurs due to failure of maturation of the midline mesoderm and improper formation of the chest and abdomen during embryonic development.5 6 Depending on the heart’s ectopic location, EC is classified into four types—i.e., thoracic (65% of cases), thoracoabdominal (20%), abdominal (10%), and cervical (5%).1 2 4 7 Only two cases of EC, one thoracic and one thoracoabdominal, from the Philippines had been reported in literature.8 Cantrell’s pentalogy—findings of bifid sternum, absence of the diaphragm, defect of anterior diaphragmatic pericardium, defect of the anterior abdominal wall, and intracardiac defects—usually accompanies the thoracoabdominal type of EC.