Management of Pentalogy of Cantrell with complete ectopia cordis and Double Outlet Right Ventricle.
Pentalogy of Cantrell (PoC) is a rare congenital midline defect. We present a case and its treatment of PoC with complete ectopia cordis and congenital heart disease. Postnatally the congenital heart defect was surgically corrected and the ectopic heart was covered by musculous mobilized flap. Due to cephalic orientation of the heart and limited intrathoracic space, replacement of the heart into the thoracic cavity was initially not performed. After 11 years of follow up our patient now is without relevant limitations solely wearing a thoracic shelter. This case elucidates the complexity of further management. The potential risk of disastrous hemodynamic compromise by intrathoracic shift is to compare with the limited safety of the ectopic heart.
Department of Pediatric Cardiology, University Heart Center Hamburg, University Hospital Hamburg- Eppendorf, Hamburg, Germany.
Gesa
G
Harring
Jochen
J
Weil
Christian
C
Thiel
Rainer
R
Schmelzle
Goetz C
GC
Mueller
D002648:Child; D019468:Disease Management; D004310:Double Outlet Right Ventricle / Q000150:complications* / Q000175:diagnosis / Q000601:surgery*; D054083:Ectopia Cordis / Q000150:complications* / Q000175:diagnosis / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D058502:Pentalogy of Cantrell / Q000150:complications* / Q000175:diagnosis / Q000601:surgery*; D011247:Pregnancy; D011296:Prenatal Diagnosis; D016896:Treatment Outcome
Pentalogy of Cantrell; congenital heart disease; double outlet right ventricle; ectopia cordis