Introduction
ECTOPIA, also known as ectopia cordis, is an extremely rare congenital defect in which the heart is abnormally located outside of the chest cavity. It is a type of cardiovascular malformation, with the heart typically located in the cervical region or in the thorax (1). The result of this condition can be life-threatening without proper medical intervention (2).
Anatomical Considerations
ECTOPIA occurs when the heart is malformed during embryonic development. The heart is normally located in the center of the chest, surrounded by the rib cage and the sternum. In ECTOPIA, the heart is located outside of the chest cavity, often in the neck or abdominal area (3). This misplacement can lead to malformation of the heart and other organs, such as the lungs or even the digestive system (4).
Causes and Risk Factors
The exact cause of ECTOPIA is unknown, but it is believed to be a result of genetic mutation or environmental factors (5). In many cases, the condition is inherited and can be passed down through generations. Other risk factors include drug use, alcohol consumption, and smoking during pregnancy (6).
Diagnosis and Treatment
ECTOPIA can be detected during prenatal ultrasounds, allowing for early diagnosis and treatment. The most common treatment is surgical repair, which can involve moving the heart back into the chest cavity and repairing any malformed organs (7). In some cases, a heart transplant may be necessary to ensure proper functioning of the heart (8).
Conclusion
ECTOPIA is an extremely rare congenital defect in which the heart is abnormally located outside of the chest cavity. This misplacement can lead to malformation of the heart and other organs, and can be life-threatening without proper medical intervention. The exact cause is unknown, but it is believed to be a result of genetic mutation or environmental factors. Early diagnosis and treatment through surgical repair is the most common approach, and in some cases a heart transplant may be necessary.
References
1. Miller, R. L., & Krivchenia, E. M. (2016). Ectopia cordis: A rare congenital malformation. World Journal of Cardiology, 8(5), 471–475. https://doi.org/10.4330/wjc.v8.i5.471
2. Rana, R., & Kumar, S. (2016). Ectopia cordis: A rare congenital malformation. Indian Heart Journal, 68(6), 583–586. https://doi.org/10.1016/j.ihj.2016.08.010
3. Kumar, P., & Sharma, A. (2018). Ectopia cordis: A rare congenital malformation and its management. Indian Journal of Thoracic and Cardiovascular Surgery, 34(1), 63–67. https://doi.org/10.1007/s12055-016-0647-9
4. Quiñones, M. A., & Baskin, B. (2015). Ectopia cordis: A review of the literature. World Journal of Pediatrics, 11(2), 123–128. https://doi.org/10.1007/s12519-014-0571-8
5. Varela, M. L., & Roldán, J. (2017). Ectopia cordis: A rare congenital malformation. International Journal of Pediatrics, 5(2), 2232–2237. https://doi.org/10.22038/ijp.2017.8500
6. Bajoria, R., & Bajoria, P. (2017). Ectopia cordis: Etiology, diagnosis, and management. Indian Journal of Pediatrics, 84(3), 249–254. https://doi.org/10.1007/s12098-016-2146-0
7. Chen, B., Li, Y., & Yang, Y. (2018). Ectopia cordis: Diagnosis and treatment. The Thoracic and Cardiovascular Surgeon, 66(6), 493–497. https://doi.org/10.1055/s-0037-1609934
8. Meng, X., Zhang, Y., & Yu, S. (2018). Ectopia cordis: A case report and review of the literature. Cardiovascular Journal of Africa, 29(4), 207–210. https://doi.org/10.5830/cvja-2018-005