Ectopia Cordis Case Report

1. ECTOPIA CORDIS
CASE REPORT AND
LITERATURE REVIEW
Professor
Abdulsalam Y Taha
2020
University of Sulaimani
Faculty of Medical Sciences
School of Medicine

2. Ectopia Cordis
21-Sep-22 Prof. Abdulsalam Y Taha 2
This child
underwent
surgery soon
after birth.
There was a
defect in the
sternum.
The defect
was enlarged
into median
sternotomy.
The heart was
relocated in
the chest.
Unfortunately, the child expired soon after surgery.
Courtesy of Prof. Abdulsalam Y Taha Courtesy of Prof. Abdulsalam Y Taha

3. Definition
 Ectopia Cordis (Latin: ˝outside/away
̏
+heart) is a congenital malformation in
which the heart is abnormally located
either partially or totally outside the
thorax. The ectopic heart can be found
along a spectrum of anatomical locations,
including the neck, thorax, or abdomen. In
most cases, the heart protrudes outside
the chest through a split sternum.
21-Sep-22 Prof. Abdulsalam Y Taha 3

4. Epidemiology and Classification
 The occurrence of ectopia cordis is 8 per
million births.
 It is typically classified according to
location of the ectopic heart, which
includes: cervical, thoracic, thoraco-
abdominal and abdominal.
 Thoracic and thoraco-abdominal ectopia
cordis constitute the vast majority of
known cases.
21-Sep-22 Prof. Abdulsalam Y Taha 4

5. Pathology
 Ectopia cordis results from failure of
midline mesoderm and ventral body wall
formation during embryonic development.
The exact pathology remains unknown,
but abnormalities in the lateral body wall
folds are believed to be involved.
Normally, the lateral body walls are
responsible for fusion at the midline to
form the ventral wall.
21-Sep-22 Prof. Abdulsalam Y Taha 5

6. Pathology…
 Corruption of this process may underline
ectopia cordis. Defective ventral body wall
formation yields a heart unprotected by
the pericardium, sternum, or skin. Other
organs may also have formed outside the
skin as well. Many cases of ectopia cordis
have associated congenital heart defects,
in which the heart has failed to properly
form.
21-Sep-22 Prof. Abdulsalam Y Taha 6

7. Pathology…
 Defects more commonly associated with
ectopia cordis include:
 Intra-cardiac defects such as ASD, VSD,
TOF, tricuspid atresia and DORV.
 Non-cardiac malformations such as:
Pentalogy of Cantrell, Omphalocele,
Anterior diaphragmatic hernia and cleft
palate.
21-Sep-22 Prof. Abdulsalam Y Taha 7

8. Prognosis
 The prognosis of ectopia cordis depends
on:
1. Its location (cervical, thoracic, thoraco-
abdominal or abdominal).
2. Extent of cardiac displacement.
3. Presence or absence of intra-cardiac
defects.
21-Sep-22 Prof. Abdulsalam Y Taha 8

9. Prognosis…
 Some studies have suggested a better
prognosis with surgery in cases of
thoraco-abdominal ectopia cordis or less
severe pentalogy of Cantrell.
 In general, the prognosis of ectopia cordis
is poor; most cases result in death shortly
after birth due to infection, hypoxemia or
cardiac failure.
21-Sep-22 Prof. Abdulsalam Y Taha 9

10. Treatment
 Due to rarity and rapid post-partum
mortality of ectopia cordis, limited
treatment options have been developed.
 Successful surgeries have been
performed, but the mortality rate remains
high.
21-Sep-22 Prof. Abdulsalam Y Taha 10

11. 21-Sep-22 11
Prof. Abdulsalam Y Taha

12. Any
Questions or
Comments?
21-Sep-22 12
Prof. Abdulsalam Y Taha