Ectopia Cordis Seoul National University Hospital

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Presentation transcript:

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

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

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

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

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