Normal Chest X-Ray
Ectopia cordis is a rare congenital lesion affecting between 5.5 and 7.9 children per one million live births. Depending on the location of the heart, the lesion can be classified as cervical, thoracic, thoracoabdominal, or abdominal. Ectopia cordis is often associated with other midline defects, including the constellation of defects know as the pentalogy of Cantrell. The components of pentalogy of Cantrell are ectopia cordis, sternal cleft, midline abdominal defect or omphalocele, a pericardial defect allowing communication between the pericardial and peritoneal cavities, and one or more cardiac defects.
Management of ectopia cordis entails coverage of the bare heart and palliation or correction of any associated heart defects. Placement of the heart into the thorax with sternal or thoracic reconstruction is desirable when possible. Although previous reports have shown very poor outcomes for infants with ectopia cordis and associated cardiac defects, more recent reports have demonstrated improved survivals. Whereas this patient’s chest radiograph appeared normal (Figure 1), physical exam, Doppler/echocardiography, and cardiac catheterization revealed ectopia cordis, right atrioventricular valve atresia, and a single morphologically left ventricle (LV) entirely below the level of the diaphragm (Figures 2 and 3⇓). She subsequently underwent successful closure of her pericardium and abdominal fascia with polytetrafluoroethylene patches and primary skin closure. Currently, her pulmonary and systemic circulations are well balanced, with oxygen saturations in the range of 85%. Her anticipated management will include a hemi-Fontan procedure at 6 months of age and ultimately a Fontan procedure at 18 to 24 months.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.