Fetal echocardiography in ectopia cordis

Ectopia cordis is an extremely rare congenital abnormality occurring in 5.5 to 7.9 per 1 million live births with high lethality. Between January 1995 and October 1997 eight cases of ectopia cordis were diagnosed at our insti tute before birth. On the basis of echocardiography the fetal heart anatomy was categorized as either normal heart anatomy (NHA; n = 3) or congenital heart defect (CHD; n = 5). In the majority of cases (seven of eight) other abnormalities were present. Some reports have described ectopia cordis bein g diagnosed in the first trimester of pregnancy. In our study group the ave rage gestational age at diagnosis was 26 weeks. The prenatal diagnosis of i solated ectopia cordis is easy; counseling the patient, the perinatal manag ement including term, place, and method of delivery, and optimal care of th e newborn are more difficult. Ectopia cordis is a malformation that pediatr icians rarely encounter, even at pediatric cardiology centers. Much more fr equently it is a problem for sonographers and obstetricians; however, pedia tric cardiologists should be aware of diagnostic algorithm for such cases, especially when additional abnormalities are present.