[Heterotaxy syndrome, analysis of 13 cases and review of the literature].

Abstract:

INTRODUCTION: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. AIM OF THE STUDY:The most frequency of heterotaxy syndrome's concomitant anomalies during a five year period in own fetopathology material. MATERIALS AND 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). CONCLUSION: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.

journal_name

Orv Hetil

journal_title

Orvosi hetilap

authors

Marton T,Cesko I,Hajdú J,Hargitai B,Papp Z

subject

Has Abstract

pub_date

2002-02-10 00:00:00

pages

299-301

issue

6

eissn

0030-6002

issn

1788-6120

journal_volume

143

pub_type

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