1. Ectopia Cordis Seoul National University Hospital
Department of Thoracic & Cardiovascular Surgery

2. Ectopia Cordis Definition
Ectopia cordis means that the heart is partially or totally outside the heart
History
1st reported in 1671 by Stensen
1st successful operation in 1975 by Saxena
in Philadelphia

3. Ectopia Cordis Incidence : 5.5-7.9/million live birth Etiology :
Failure of maturing of midline mesodermal component of the chest and abdomen results from early rupture of amniotic sac and formation of amniotic bands
3. Associated defects :
Diaphragmatic hernia
Omphalocele & diastasis recti
Sternal and pericardial defect
Congenital heart disease
Partial displacement of ventricle

4. Ectopia Cordis Classification
Cervical : very rare , only in fetus
Cervicothoracic : most common
Thoracoabdominal: common
Abdominal: very rare
* For practical purpose, there are
essentially only two types of ectopia

5. Strategy for Repair for Ectopia Cordis
Urgent soft tissue coverage and hemodynamic palliation if necessary
Intracardiac repair with concomitant chest wall reconstruction and reduction of into the thoracic cavity