Rare congenital anomaly (0.1% of all congenital heart defects) where the heart is located wholly or partly outside the thoracic cavity. It is considered by some experts as an extreme form of the pentalogy of Cantrell (see this term)
The following are often associated:  Turner syndrome (XO) or trisomy 18, omphalocele, cleft lip palate, hydrocephaly. Frequent VSD (100 %), ASD (53 %) or tetralogy of Fallot (20 %). 

Four types according to the region where the heart is exteriorized:

       1:        cervical 3 %

       2:        thoracic 60 %

       3:        abdominal 30 %

       4:        thoracoabdominal 7 %

The purpose of the initial surgery is to cover the heart with skin or synthetic material without compressing the heart or the great vessels. Mortality is important.

Anesthetic implications: 

neonatal surgery at high risk, cardiac malformations, difficult monitoring (ECG)

References : 

-        Diaz JH. 
Perioperative management of neonatal ectopia cordis : report of three  cases. 
Anesth Analg 1992; 75: 833-7

-        Pandey R, Darong V, Punj J, Kumar A, Arora MK, Shende D. 
Anesthetic management of thoracic type of complete ectopia cordis. 
Pediatr Anesth 2008; 18: 1137-9.

Updated: November 2018