- adjacent to the thorax: ~60%
- abdominal: 15-30%
- thoracoabdominal: 7-18%
- cervical: ~3%
The incidence is 5.5 to 7.9 per 1,000,000 live births 10.
It results from the failure of migration of lateral mesoderm into the midline. Some forms may be the outcome of amniotic band syndrome 11.
Ectopia cordis may occur as an isolated malformation or it may be associated with a larger category of ventral body wall defects that affect the thorax, abdomen, or both.
A well-known association is pentalogy of Cantrell 1 which comprises:
- ectopia cordis
- omphalocele (typically supraumbilical)
- congenital diaphragmatic hernia
- sternal cleft
- congenital heart disease
When in isolation, the heart is seen in the amniotic cavity with a thoracic wall defect. If in association with pentalogy of Cantrell, it may seen within an omphalocele 2.
Imaging clues on frontal chest radiographs include:
- abnormal cardiac position and configuration
- air lucency may surround the ectopic heart
- sternal defect is often present
- wide separation of the sternal ends of the clavicles
- widening of the superior mediastinum
The lateral view may confirm the extrathoracic location of the heart.
Cross-sectional modalities allow for the best visualization of ectopia cordis, and is required for any surgical planning.
Treatment and prognosis
Although surgical correction may be attempted 9, the prognosis is generally poor and depends on the severity of intracardiac malformations and the presence of associated abnormalities. Most infants are stillborn or die within the first hours or days of life.
- 1. Kumar B, Sharma C, Sinha DD et-al. Ectopia cordis associated with Cantrell's pentalogy. Ann Thorac Med. 2008;3 (4): 152-3. doi:10.4103/1817-1737.43084 - Free text at pubmed - Pubmed citation
- 2. Doubilet PM, Benson CB. Atlas of ultrasound in obstetrics and gynecology, a multimedia reference. Lippincott Williams & Wilkins. (2003) ISBN:0781736331. Read it at Google Books - Find it at Amazon
- 3. Bianchi DW, Crombleholme TM, D'Alton ME. Fetology, diagnosis & management of the fetal patient. McGraw-Hill Professional. (2000) ISBN:0838525709. Read it at Google Books - Find it at Amazon
- 4. Entezami M, Albig M, Knoll U et-al. Ultrasound Diagnosis of Fetal Anomalies. Thieme. (2003) ISBN:1588902129. Read it at Google Books - Find it at Amazon
- 5. Balakumar K, Misha K. Ectopia cordis with endocardial cushion defect: Prenatal ultrasonographic diagnosis with autopsy correlation. Ann Pediatr Cardiol. 2010;3 (2): 166-8. doi:10.4103/0974-2069.74048 - Free text at pubmed - Pubmed citation
- 6. Wicks JD, Levine MD, Mettler FA. Intrauterine sonography of thoracic ectopia cordis. AJR Am J Roentgenol. 1981;137 (3): 619-21. AJR Am J Roentgenol (citation) - Pubmed citation
- 7. Achiron R, Shimmel M, Farber B et-al. Prenatal sonographic diagnosis and perinatal management of ectopia cordis. Ultrasound Obstet Gynecol. 1991;1 (6): 431-4. doi:10.1046/j.1469-0705.1991.01060431.x - Pubmed citation
- 8. Spindola-Franco H, Fish BG. Radiology of the Heart: Cardiac Imaging in Infants, Children, and Adults. Springer. ISBN:1461382076. Read it at Google Books - Find it at Amazon
- 9. Shad J, Budhwani K, Biswas R. Thoracic ectopia cordis. (2012) BMJ case reports. doi:10.1136/bcr.11.2011.5241 - Pubmed
- 10. Hornberger LK, Colan SD, Lock JE, Wessel DL, Mayer JE Jr. Outcome of patients with ectopia cordis and significant intracardiac defects. Circulation. 1996 Nov 1;94(9 Suppl):II32-7.Pubmed citation
- 11. Engum SA> Embryology, sternal clefts, ectopia cordis, and Cantrell's pentalogy. Semin Pediatr Surg. 2008 Aug;17(3):154-60.doi:10.1053/j.sempedsurg.2008.03.004 - Pubmed