CTICasesPentalogy of Cantrell
Imaging findings
Cardiac CT in an adult shows transposition of the great arteries, a repaired VSD, a Cor Triatriatum, a severe sternal cleft/deficiency, and a large anterior diaphragmatic defect. The apical portions of both ventricles herniate through the diaphragmatic and sternal defects into the upper abdominal wall, representing a partial ectopia cordis.
Key takeaways
Pentalogy of Cantrell is a rare congenital syndrome characterized by a midline developmental defect consisting of five features: a midline supraumbilical abdominal wall defect, a lower sternal defect, an anterior diaphragmatic defect, a diaphragmatic pericardial defect, and congenital intracardiac anomalies (frequently ectopia cordis, VSD, or Tetralogy of Fallot). Survival into adulthood is rare and typically requires timely surgical reconstruction and management of the associated complex congenital heart disease.
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