Pentalogy of Cantrell with a double-outlet right ventricle: 3.5-year follow-up in a prenatally diagnosed patient

Eur J Pediatr. 2008 Jan;167(1):103-5. doi: 10.1007/s00431-007-0579-8. Epub 2007 Aug 4.


Pentalogy of Cantrell is a rare congenital defect associated with five ventral midline anomalies and high mortality. An obstetric sonogram revealed a fetus with a body wall defect suggesting a diagnosis of this condition. Soon after birth, the infant underwent a closure of the upper abdominal wall defect followed by the successful repair of double-outlet right ventricle and pulmonary valve stenosis at the age of 5 months. The patient is currently alive and well 3.5 years after surgery. It is concluded that a deliberate therapeutic strategy based on the intrauterine diagnosis may alter the natural history of this devastating disorder.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abdominal Wall / abnormalities*
  • Abdominal Wall / surgery
  • Adult
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Hearing Loss / etiology*
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / surgery
  • Hernia, Umbilical / complications*
  • Hernia, Umbilical / surgery
  • Humans
  • Infant, Newborn
  • Male
  • Pregnancy
  • Ultrasonography, Prenatal*