[Heterotaxy syndrome, analysis of 13 cases and review of the literature].
BACKGROUND
Heterotaxy syndrome (Ivemark syndrome, or asplenia-polysplenia syndrome) is a heterogeneous group of disease with disturbed body symmetry and malposition of internal organs. Heterotaxy syndrome is caused by the disturbance of the left/right axis in the early embryonic period.


OBJECTIVE
The most frequency of heterotaxy syndrome's concomitant anomalies during a five year period in own fetopathology material.


METHODS
Data of fetopathologic examination of 13 fetuses suffering from prenatally diagnosed heterotaxy syndrome.


RESULTS
Situs ambiguus was detected in 9 cases out of 13. In the remaining 4 cases situs inversus totalis was diagnosed. The most frequent and important associated malformation included congenital heart disease was AV channel (10/13) and great vessel anomaly (10/13).


CONCLUSIONS
In cases with prenatally detected complex cardiac anomalies (especially AV channel cases) heterotaxy anomaly must be taken into consideration, with main consequences in prenatal counselling.
Marton
Tamás
T
Semmelweis Egyetem, Budapest, Altalános Orvostudományi Kar, l. sz. Szülészeti és Nógyógyászati Klinika.
Cesko
Izabella
I
Hajdú
Júlia
J
Hargitai
Beáta
B
Papp
Zoltán
Z
D000818:Animals; D003376:Counseling; D004195:Disease Models, Animal; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging; D006801:Humans; D012189:Retrospective Studies; D012857:Situs Inversus / Q000000981:diagnostic imaging* / Q000473:pathology; D016216:Ultrasonography, Prenatal
Heterotaxia syndroma: 13 foetopathologiai eset elemzése és irodalmi áttekintés.