02 Ectopia cordis

Royal Victoria Hospital
Date: 1933
Size (H x W x D): 21 x 18 x 8

The anterior chest wall (with the trachea and larynx above) shows a 3 cm rudimentary, globoid heart protruding in the midline.

History: Male infant pronounced dead 10 minutes after birth.

Comment: Ectopia cordis is defined as the presence of the heart or a portion thereof outside the thoracic cavity. It is believed to arise from failure of the lateral aspects of the embryonic disc to fuse in the midline Several locations of the aberrant heart have been described, including cervical, abdominal and, as in this case, the anterior chest wall associated with a sternal defect (ectopia cordalis pectoralis). The abnormality is rare (about 5 - 8 per million births). Most cases are associated with cardiac structural anomalies.

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