Ectopia cordis: a rare congenital anomaly

Clin Anat. 2014 Nov;27(8):1193-9. doi: 10.1002/ca.22402. Epub 2014 Apr 19.


Ectopia cordis (EC) is a rare congenital anomaly associated with the heart positioned outside of the thoracic cavity either partially or completely. The ectopic heart can be found along a spectrum of anatomical locations, including the cervical, thoracic and abdominal regions and in most cases, it protrudes outside the chest through a split sternum. Although the first case of EC was identified during the early 1600s only 91 cases have been reported since then in the literature. This review will discuss the history and prevalence of EC, its etiology, morphology, presentation and symptoms, complications, diagnosis, treatment and management and prognosis.

Keywords: abdominal; cervical; congenital cardiac anomaly; ectopia cordis; thoracoabdominal.

Publication types

  • Historical Article
  • Review

MeSH terms

  • Ectopia Cordis / embryology*
  • Ectopia Cordis / history
  • Ectopia Cordis / surgery
  • History, 17th Century
  • History, 18th Century
  • History, 19th Century
  • History, 20th Century
  • History, 21st Century
  • Humans
  • Rare Diseases*