Pathology
Ectopia cordis results from a failure of proper maturation of midline mesoderm and ventral body wall formation during embryonic development. The exact etiology remains unknown, but abnormalities in the lateral body wall folds are believed to be involved. Normally, the lateral body walls are responsible for fusion at the midline to form the ventral wall. Corruption of this process may underlie ectopia cordis.
Defective ventral body wall formation yields a heart unprotected by the pericardium, sternum, or skin. Other organs may also have formed outside the skin, as well. Many cases of ectopia cordis have associated congenital heart defects, in which the heart has failed to properly form.
Defects more commonly associated with ectopia cordis include:
- Intracardiac defects
- Atrial septal defect
- Ventricular septal defect
- Tetralogy of Fallot
- Tricuspid atresia
- Double outlet right ventricle
- Non-cardiac malformations
- Pentalogy of Cantrell
- Omphalocele
- Anterior diaphragmatic hernia
- Cleft palate
Read more about this topic: Ectopia Cordis
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