A 2 week old girl, born at 38 weeks gestation, had been noted on prenatal ultrasonic screening to have an omphalocele. Subsequent to birth, a superficial pulsatile mass was present, extending from the subxiphoid region into the omphalocele. Computed tomographic scanning with contrast (Fig. 1) showed a septated diverticulum (black arrow, white arrow indicates septum) which extended caudally from the left ventricle through the anterior defect in the diaphragm. Cross-sectional echocardiography with colour Doppler in the four chamber view (Fig. 2) demonstrated a midline heart, patency of the oval fossa, and the pulsatile diverticulum (arrow, arrowhead indicates septum) extending from the left ventricle (LV) into the omphalocele. The septum within the diverticulum was separate from the true ventricular septum. The right ventricle (RV) was also seen in this view.

Figure 1

Figure 2

On the 21st day of life, elective operative repair of the diverticulum was performed on cardiopulmonary bypass through a midline sternal incision. There was no abnormality in the sternum itself. The diverticulum (LVD) was exposed at its junction with the left ventricular apex (Fig. 3), resected, and closed with pledgeted sutures (Fig. 4, arrow) with the expectation that the devitalized tissue will eventually atrophy. The diaphragmatic and pericardial defects were also repaired. Postoperative recovery was unremarkable, and the patient was discharged five days after operation.

Figure 3

Figure 4

This patient satisfied four of the five criterions for the syndrome first described by Cantrell in 1958, which is characterized by midline thoracoabdominal defects involving the abdominal wall, diaphragm, sternum, pericardium, and heart. These are thought to be due to a developmental failure of the mesoderm during the third and fourth weeks of gestation. A left ventricular diverticulum is found in approximately one-third of cases.Reference Vazquez-Jimenez, Muehler and Daebritz¹ Operative repair was indicated in our patient due to the risks of diverticular incarceration within the diaphragmatic defect, arrhythmias, formation of thrombus, diverticular rupture, and cardiac failure.