RESUMO
Resumen
The Ivemark syndrome is one of the heterotaxy syndromesor also named cardiosplenic syndromes. During the normal embryological development, as the body left-right axis is established also the organs assume their definitive positions. When variation of the body organs arrangement occurs precociously in embryogenesis, there is a tendency toward symmetric development of normally asymmetric organs or organ systems, with concomitant complex cardiac defects. The most significant forms of heterotaxy syndromes are the polysplenia syndrome and the Ivemark syndrome. Both are rare syndromes and seem to be responsible for 1% of all congenital cardiac defects. The Ivemark syndrome is usually manifested as asplenia, bilaterally trilobed lungs, symmetric liver, with a central position in the upper abdomen, gallbladder centrally positioned or in the left side, and complex cardiopathy. The authors describe a case report of a second gestation of an healthy young couple, to whom a diagnosis of Ivemark syndrome was made at 20th gestacional week. In her obstetric history she had a term pregnancy, with an Ivemark syndrome newborn -anatomo-pathological diagnosis-, who died of cardio-pulmonary insufficiency in the neonatal period. In the current pregnancy an increased nuchal translucency was detected in the first trimester ultrasound. Amniocentesis for fetal cariotype revealed a normal cariotype for a male fetus. In the ultrasound at 21th gestational week a complex cardiopathy was identified and echocardiography confirmed the diagnosis of right isomerism. The couple decided for a pregnancy interruption, which was accepted. The anatomo-pathological exam confirmed the diagnosis of Ivemark syndrome
