Pulsations of the Umbilicus as Sign of a Left Ventricle Diverticulum—Presentation of a Mild form of Pentalogy of Cantrell

Objectives: The pentalogy of Cantrell (POC) is a rare multiple congenital anomalies syndrome which often presents with lethal anatomic alterations. We present a mild case of POC with dextrocardia and left ventricle diverticulum reaching into the umbilicus.

Methods: The baby was born to a 32-year-old secundigravida at 40 weeks of gestation. Antenatal echocardiography revealed dextrocardia and a ventricular septal defect. Postnatal adaption was unremarkable and on examination no major alterations were obvious. Conspicious pulsations of the umbilicus and surrounding abdominal wall accompanied by a small vascular convolute at the root of the umbilical cord were noted postnatally.

Result: Echocardiography and MRI diagnostics showed a 3.7-cm long protrusion of the left ventricle apex directly under the sternum, reaching into a diverticulum in the area of the umbilical sac. Additional findings were dextrocardia, a small ventricular septal defect, aberrant right subclavian artery, and persistent left superior vena cava. Osseous malformations were not found. The baby underwent surgery on cardiopulmonary standby at 9 days of life; thereby resection of the diverticulum was performed in toto and abdominal wall hernia was closed. Postoperative recovery was unremarkable. Follow-ups revealed no complications up to the age of 9 months.

Conclusion: POC can present in very mild forms, which are not detected during usual prenatal workups. Alterations of umbilical cord appearance can be the only clinical sign after birth such as in the presented case with an unusual left ventricle diverticulum.

Publication History

Article published online:
21 February 2021