Complete Ectopia Cordis: A Case Report and Literature Review

Abstract

Ectopia cordis is a congenital heart exposure defined as complete or partial protrusion of heart through ventral defect in the thoracoabdominal wall alone or with other viscera in cases of pentalogy of Cantrell. This condition was first described by Haller et al. in 1706; since then many advances have been made. Diagnosis of ectopia cordis is done prenatally in well-equipped health facility by antenatal ultrasound scan so that early diagnosis and management plan can be initiated. The index case was delivered to uneducated rural family and admitted at 3 days of life and survived for seven days, even though most literatures state that majority died within four days even with surgery. So, in view of this, we presented this case report to deliberately draw the attention of paediatrician/obstetrician to the fact that even though this condition is rare, proactive search and diagnosis should be made and early treatment should be instituted, so that such a child may be salvaged.

Ultrasonographic Diagnosis of Fetuses with Pentalogy of Cantrell and Turner Syndrome, Dual Case Reports and Review of Literature

“…Ectopic cordis is suspected when cardiac contractile activity is detected outside the chest cavity and can be confirmed by Doppler [10,11]. The thoracoabdominal type of ectopic cordis is specific to Pentalogy of Cantrell and is highly suggestive for diagnosis [5]. The thoraco-abdominal type of ectopic cordis is defined by a ventral wall defect which extends to the umbilicus causing the diaphragm to have a 'V' shaped defect [12].…”

“…Once born, Turner syndrome individuals face a host of abnormalities including metabolic, cardiac and reproductive. Compared to Pentalogy of Cantrell which requires immediate surgical repair and rarely live beyond the age of 10, Turner syndrome patients can live into adulthood [5,8,17].…”

“…Most importantly, mesoderm failure results in cardiac defects, which are the major determinant for severity and prognosis, most commonly ventral septal defect [4,5]. Pentalogy of Cantrell is divided into two classes; class one is complete malformation with the presence of all five defects and classes two contains only four defects [4].…”

“…Pentalogy of Cantrell is divided into two classes; class one is complete malformation with the presence of all five defects and classes two contains only four defects [4]. Although this syndrome commonly results in still births, there have been reported cases of live births requiring immediate surgical repair [5]. Yet, the survival rate is unknown as live cases are rare [5].…”

“…Although this syndrome commonly results in still births, there have been reported cases of live births requiring immediate surgical repair [5]. Yet, the survival rate is unknown as live cases are rare [5].…”