[1] Ectopia cordis results from a failure of proper maturation of midline mesoderm and ventral body wall (chest) formation during embryonic development.
[2] The exact etiology remains unknown, but abnormalities in the lateral body wall folds are believed to be involved.
[5] Due to the rarity and rapid postpartum mortality of ectopia cordis, limited treatment options have been developed.
In general, the prognosis for ectopia cordis is poor—most cases result in death shortly after birth due to infection, hypoxemia, or cardiac failure.
[2] It is typically classified according to location of the ectopic heart, which includes: Thoracic and thoraco-abdominal ectopia cordis constitute the vast majority of known cases.