[Prenatal diagnosis of right atrial isomerism (asplenia-syndrome): case report].
Right atrial isomerism (earlier known as asplenia) is a group of malformations including anomalies of the heart and the visceral organs. Complex defects of the heart are normally found, but these are non-specific. The suspected diagnosis can be confirmed most easily by focusing on a specific and segmental examination of the heart. We report on a fetus referred at 22 weeks in whom AV-septal defect was detected. Heart and stomach were on the left side, but the segmental approach allowed the detection of juxtaposed descending aorta and inferior vena cava as typical signs of right isomerism. The suspected malformation could be verified by the additional detection of infra-diaphragmatic total anomalous pulmonary venous drainage by using colour Doppler. Howell-Jolly bodies from fetal blood supported the diagnosis of asplenia. Autopsy after termination of pregnancy demonstrated the typical signs of right isomerism. The paper reviews the typical prenatal findings which lead to the detection of right isomerism in the fetus. Segmental approach should be performed in each fetus with an anomaly of the heart especially in the presence of complex defects.
Batukan
C
C
Abteilung "Pränatale Diagnostik und Therapie" Universitäts-Frauenklinik, Berlin.
Schwabe
M
M
Heling
K S
KS
Hartung
J
J
Chaoui
R
R
D000328:Adult; D001283:Atrioventricular Node / Q000002:abnormalities / Q000196:embryology; D005260:Female; D006325:Heart Atria / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000196:embryology; D006801:Humans; D011247:Pregnancy; D018615:Ultrasonography, Doppler, Color; D016216:Ultrasonography, Prenatal