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Thoracoabdominal ectopia cordis with isolated infundibular atresia

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      Abstract

      Ectopia cordis is a rare congenital anomaly in which the heart is partially or totally outside the thorax. 1 The thoracic and thoracoabdominal forms of ectopia cordis have been considered to constitute together 97% (138 of 142) of the cases.2 The cervical form, found in 4 out of 142 cases, has been described only in malformed fetuses.2 Complete abdominal ectopia cordis was thought previously to be represented only by Deschamp's case that was reported in 1806 by Cullerier.3 However, whether or not complete abdominal ectopia cordis really exists and, if so, how it should be defined, remain unclear. We present a unique case of ectopia cordis that superficially resembles complete abdominal ectopia cordis. This patient has the following previously undescribed cardiac anomaly: isolated atresia of the subpulmonary infundibulum with an intact ventricular septum and a well-formed and patent pulmonary valve.
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