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Year : 2017  |  Volume : 31  |  Issue : 1  |  Page : 50-51

Ectopia cordis : A case report

1 Department of Anatomy, Regional Institute of Medical Sciences, Imphal, Manipur, India
2 Department of Pediatrics, Regional Institute of Medical Sciences, Imphal, Manipur, India

Date of Web Publication17-Jan-2017

Correspondence Address:
Kinthanliu Guanmei
Department of Anatomy, Regional Institute of Medical Sciences, Lamphelpat, Imphal - 795 004, Manipur
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0972-4958.198465

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Ectopia cordis is a rare congenital heart anomaly where the heart is abnormally located outside the thoracic cavity. The defect lies in the failure of the midline closure of the body wall by lateral and head and tail folds. Because of its associated abnormalities, ectopia cordis is a challenging congenital anomaly. The prognosis of ectopia cordis depends on its classification and associated intracardiac anomalies.

Keywords: Ectopia cordis, head and tail folds, lateral folds

How to cite this article:
Guanmei K, Golmei N, Ajita R, Devi AJ. Ectopia cordis : A case report. J Med Soc 2017;31:50-1

How to cite this URL:
Guanmei K, Golmei N, Ajita R, Devi AJ. Ectopia cordis : A case report. J Med Soc [serial online] 2017 [cited 2022 May 19];31:50-1. Available from:

  Introduction Top

Ectopia cordis , also known as exocardia or ectocardia, is a rare congenital anomaly where the heart is abnormally located outside the thoracic cavity. Haller et al. first reported ectopic cordis in 1706, and it was classified into different types by Weese et al. in 1818 and Todd et al. in 1836. [1],[2] Its prevalence rate is reported to be 5.5-7.9 per million live births. [3]

  Case Report Top

A 24-year-old primigravida mother delivered a full-term male baby of 3.8 kg weight in Regional Institute of Medical Sciences (RIMS), Imphal, Manipur, India by normal vaginal delivery (NVD) presenting a pulsatile mass of heart projecting outwards and upwards along with the liver and the umbilicus through 10-12 cm midline sternal defects. The vitals and reflexes were normal. The beating heart and major vessels were covered with a serous membrane. There were no other visible physical abnormalities [Figure 1]. The baby died after 9 days of conservative comfort care treatment.
Figure 1: Ectopia cord with associated anomalies

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  Discussion Top

Embryologically ectopia cordis is due to defect in the closure of lateral folds to form the ventral body wall. By the end of the 3 rd week the lateral plate mesoderm splits into outer somatic and inner splanchnic layers and created a space intervening the two layers. During the 4 th week, the sides of the embryo begin to grow ventrally and by the end of the 4 th week, the lateral folds meet in the midline and fuse to close the ventral body wall. This closure is aided by head and tail folds. The complete or incomplete failure of midline fusion and defect in mesoderm in embryonic state can result in a variety of disorders ranging from isolated ectopia cordis to complete ventral evisceration. [4]

Ectopia cordis may be of complete variety or partial variety. According to its location where the heart is present, the ectopia cordis may be of cervical (3%), cervicothoracic (<1%), thoracic (60%), thoracoabdominal (7%), or abdominal (30%) type. The thoracoabdominal type is frequently associated with Cantrell's pentalogy that includes bifid sternum, deficiency of the diaphragm, defect of the diaphragmatic pericardium, defect of the anterior abdominal wall, and intracardiac defects. [5]

Because of its rarity and other commonly associated abnormalities, ectopia cordis is a challenging congenital anomaly. The complete ectopia cordis is a neonate emergency. Many cases of these (80.2%) have associated intracardiac defects, [6] including ventricular septal defect (VSD, 100%), atrial septal defect (ASD, 53%), tetralogy of Fallot (TOF, 20%), left ventricular diverticulum (LVD, 20%), and pulmonary hypoplasia. [7]

The prognosis of ectopia cordis depends on its classification and associated intracardiac anomalies. [3] Reports of successful repair of thoracic ectopia cordis are rare, the cervical type is universally fatal, and the thoracoabdominal type has a higher rate of successful repair. [2],[3],[8],[9]

There are descriptions of ectopia cordis with chromosomal abnormalities, but there is no known genetic etiology in humans. [10]

It is important to distinguish true ectopia cordis from the failure of sternal fusion in which the heart is structurally normal. Many cases reported as thoracic ectopia, with intact skin, are more likely examples of isolated failure of sternal fusion.

If the diagnosis of ectopia cordis is confirmed during the pregnancy, an early plan should be made for elective atraumatic cesarean delivery. [11] Immediately after birth, the newborn should be stabilized and the lesion should be covered with saline-soaked gauze pads and wrapping to prevent desiccation and heat loss of the exposed viscera. After completing the preoperative evaluation, the patient should be taken promptly to the operation room for surgical repair of the defects. The overall objectives of ectopia cordis management are: Closure of the chest wall defect, including the sternal defect, repair of the associated omphalocele, placement of the heart into the thorax, and repair of the intracardiac defect. [12],[13]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Engum SA. Embryology, sternal clefts, ectopia cordis, and Cantrell's pentalogy. Semin Pediatr Surg 2008;17:154-60.  Back to cited text no. 1
Kaplan LC, Matsuoka R, Gilbert EF, Opitz JM, Kurnit DM. Ectopia cordis and cleft sternum: Evidence for mechanical teratogenesis following rupture of the chorion or yolk sac. Am J Med Genet 1985;21:187-202.  Back to cited text no. 2
Hornberger LK, Colan SD, Lock JE, Wessel DL, Mayer JE Jr. Outcome of patients with ectopia cordis and significant intracardiac defects. Circulation 1996;94(Suppl):II32-7.  Back to cited text no. 3
Larsen W. Embryonic folding. In: Sherman LS, Potter SS, Scott WJ, editors. Human Embryology. 3 rd ed. Philadelphia: Churchill Livingstone; 2001. p. 133-4.  Back to cited text no. 4
Abdallah HI, Marka LA, Balsara RK, Davis DA, Russo PA. Staged repair of pentalogy of Cantrell with tetralogy of Fallot. Ann Thorac Surg 1993;56:979-80.  Back to cited text no. 5
Amato JJ, Zelen J, Talwalkar NG. Single-stage repair of thoracic ectopia cordis. Ann Thorac Surg 1995;59:518-20.  Back to cited text no. 6
Morales JM, Patel SG, Duff JA, Villareal RL, Simpson JW. Ectopia cordis and other midline defects. Ann Thorac Surg 2000;70:111-4.  Back to cited text no. 7
Kim KA, Vincent WR, Muenchow SK, Wells WJ, Downey SE. Successful repair of ectopia cordis using alloplastic materials. Ann Plast Surg 1997;38:518-22.  Back to cited text no. 8
Shamberger RC, Welch KJ. Sternal defects. Pediatr Surg Int 1990;5:156-64.  Back to cited text no. 9
Brewer S, Williams T. Finally, a sense of closure? Animal models of human ventral body wall defects. Bioessays 2004;26:1307-21.  Back to cited text no. 10
Diaz JH. Perioperative management of neonatal ectopia cordis: Report of three cases. Anest Analg 1992;75:833-7.  Back to cited text no. 11
Dobell AR, William HB, Long RW. Staged repair of ectopia cordis. J Pediatr Surg 1982;17:353-8.  Back to cited text no. 12
Leca F, Thibert N, Khoury W, Fermont L, Laborde F, Dumez Y. Extrathoracic heart (ectopia cordis): Report of two cases and review of literature. Int J Cardiol 1989;22:221-8.  Back to cited text no. 13


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