[Ectopia cordis - case report]

Object: Case report of ectopia cordis in a fetus at 23 weeks gestation.

Design: Case repor.

Setting: Obstetrics and Gynecology Clinic, University of Ostrava and University Hospital in Ostrava.

Methods and results: We report a case of pentalogy of Cantrell variant in a 23-week fetus with an ultrasound finding of ectopia cordis, associated intracardial defects and omphalocele containing liver and guts. The patient opted for termination of pregnancy by using prostaglandins. Autopsy of the fetus revealed a cleft sternum and thoracic wall defect with nude ectopic heart-thoracic type and omphalocele containing liver and guts. Detailed examination of the heart revealed a double outlet right ventricle with a complete atrioventricular septal defect. Our study describes typical ultrasound findings in a correlation with autopsy findings.

Conclusion: Ectopia cordis is a rare congenital malformation with an estimated incidence of 1:100 000 live births in developed countries. It is characterized by abnormal heart placement outside the thorax, mostly on the thoracoabdominal side. This form is often associated with pentalogy of Cantrell.