Management of Pentalogy of Cantrell with complete ectopia cordis and Double Outlet Right Ventricle
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Management of Pentalogy of Cantrell with complete ectopia cordis and Double Outlet Right Ventricle. / Harring, Gesa; Weil, Joachim; Thiel, Christian; Schmelzle, Rainer; Müller, Götz.
In: CONGENIT ANOM, Vol. 55, No. 2, 2015, p. 121-123.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Management of Pentalogy of Cantrell with complete ectopia cordis and Double Outlet Right Ventricle
AU - Harring, Gesa
AU - Weil, Joachim
AU - Thiel, Christian
AU - Schmelzle, Rainer
AU - Müller, Götz
N1 - This article is protected by copyright. All rights reserved.
PY - 2015
Y1 - 2015
N2 - Pentalogy of Cantrell (P.o.C.) is a rare congenital midline defect. We present a case and its treatment of P.o.C. 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.
AB - Pentalogy of Cantrell (P.o.C.) is a rare congenital midline defect. We present a case and its treatment of P.o.C. 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.
U2 - 10.1111/cga.12096
DO - 10.1111/cga.12096
M3 - SCORING: Journal article
C2 - 25385246
VL - 55
SP - 121
EP - 123
JO - CONGENIT ANOM
JF - CONGENIT ANOM
SN - 0914-3505
IS - 2
ER -