Circulation. 2006;113:e757-e758
doi: 10.1161/CIRCULATIONAHA.105.575191
(Circulation. 2006;113:e757-e758.)
© 2006 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Complete Ectopia Cordis
Christian Lilje, MD;
Florian Weiss, MD;
F. Lacour-Gayet, MD, PhD;
K. Ntalakoura, MD;
Vit Rázek, MD;
J. Weil, MD, PhD
From the Departments of Pediatric Cardiology (C.L., V.R., J.W.) and Heart and Thoracic Surgery (F.L.-G., K.N.), University Heart Center, and Department of Diagnostic Radiology (F.W.), University Hospital Hamburg–Eppendorf, Hamburg, Germany.
Reprint requests to Christian Lilje, MD, Universitäres Herzzentrum, Kinderkardiologie, Universitäts-Klinikum Hamburg–Eppendorf, Martinistrasse 52, D-20246 Hamburg, Germany. E-mail lilje{at}europe.com
Complete ectopia cordis is a very rare condition. With few exceptions, it is uniformly considered fatal. Death usually occurs within the first days of life as a result of infection, cardiac failure, or hypoxemia. We report the case of a male infant with Cantrell’s syndrome. There was complete thoracic exteriorization of the heart, with cephalic orientation of the cardiac apex (Figure 1). The cardiac surface was covered by serous pericardium only. The intracardiac anatomy was that of a double-outlet right ventricle. Postnatal management was supported by prenatal diagnosis. Initially, the cardiac surface was continually moistened, and an associated omphalocele was surgically corrected. After temporary prosthetic reconstruction of the chest wall, the heart was eventually covered by skin after staged excessive mobilization of a latissimus dorsi musculocutaneous free flap and a mash skin autotrans-plant (Figure 2 and Figure 3). At the age of 5 months, the boy underwent extrathoracic repair of the intracardiac defects. The heart was left protruding from the chest wall as a result of the cephalic cardiac orientation, the excessive length of the supplying vessels, the anatomy of the diaphragm, and the small size of the thoracic cavity. A thoracic protector was adopted. Eventually, part of the lung or liver will need to be sacrificed to provide sufficient intrathoracic space. Currently, the patient has survived for 3.5 years.
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Figure 1. Aspect of the boy’s chest as a neonate. The heart is protruding from the thoracic cavity above an omphalocele. The cardiac apex is pointing cranially.
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Figure 2. MRI, sagittal scan, and high-resolution turbo spin echo. The heart is located outside the thorax. Note the small intrathoracic cavity. Ao indicates ascending aorta; RV, right ventricle; and PA, pulmonary artery.
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Figure 3. MRI, axial scan, and high-resolution spin echo. The aorta is overriding a ventricular septal defect (arrow). Note the distance between the heart and the posterior mediastinal space for the supplying vessels. Ao indicates ascending aorta; desc Ao, descending aorta; RV, right ventricle; RPA, right pulmonary artery; LPA, left pulmonary artery; and arrow, ventricular septal defect.
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Acknowledgments
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Disclosures
None.
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Issue Highlights
Circulation 2006 113: 2261.
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