Syndrome of congenital ventricular diverticulum and midline thoraco abdominal defects

Abstract

The long term follow up of a 30 yr old patient who had a correction of the syndrome of left ventricular diverticulum and thoraco abdominal defects is presented. The main features of the syndrome include a diverticulum of the left ventricle, a ventricular septal defect, and sometimes other cardiac anomalies. The thoraco abdominal defects consist of foreshortened sternum, pericardial and diaphragmatic defects, and umbilical hernia. The association of these anomalies is thought to be due to a developmental failure of the primitive paramidline mesoderm. The diagnosis can be made clinically by the presence of a pulsatile, epigastric mass associated with signs of cardiac septal defects and dextrocardia. The prognosis of patients with this syndrome depends mainly on the associated abnormalities although rupture of the diverticulum can be a fatal complication. The treatment of choice is resection of the diverticulum combined with repair of associated anomalies which can give good early and late results.