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Articles » Cardiovascular » Situs anomalies » Right atrial isomerism
2010-04-07-11 Right atrial isomerism © Cuillier

Right atrial isomerism

Fabrice Cuillier, MD.*,  Alice Bertha, MD**

* Service de Gynécologie-Obstétrique, Hôpital Félix Guyon, Saint-Denis de La Réunion, France.
** Department of Gynecology, Seattle, USA.

Case report

This is a case of a 30-year-old woman (G4P3), who was referred to our antenatal unit at 32 weeks of gestation for an ultrasound examination. There was no known family history of congenital disease. Patient lived in Mayotte’s Island in the Indian ocean. 

A cardiac malformation was first detected by the sonographer. The patient was then sent to our antenatal unit. An amniocentesis was performed with a normal result, karyotype 46XY. The following images were obtained during the examination at 32 and 34 weeks of gestation. Our diagnosis based on the ultrasound examination was a right atrial isomerism. The patient delivered at 38 weeks of gestation and right atrial isomerism was confirmed postnatally. Aside of the cardiac malformation, the spleen was absent and there was hypospadias. The prognosis for the neonate was unfavorable. Mother returned home with her baby 15 days after delivery. The baby died 30 days after delivery.

The ultrasound findings were following:

  • Stomach on the right and liver on the left side
  • Spleen not detected
  • Heart on the left side
  • Incomplete atrioventricular septal defect with two atrioventricular valves
  • Aorta shifted to the right and inferior vena cava on the left, in front of the aorta
  • Hypospadias

Images 1,2
: Image 1 shows heart on the left and stomach on the right side, aorta is shifted to the right and inferior vena cava is on the left in front of the aorta. Image 2 shows a atrioventricular septal defect.

Images 3,4: Image 3 shows a ventricular septal defect and large communication between left and right atria. Image 4 shows a Doppler imaging of the blood flow through the atrioventricular septal defect. There are 2 atrioventricular valves.

Images 5,6: Image 5 shows a left outflow tract. Image 6 shows both pulmonary arteries.

Images 7,8: Image 7 shows a fetal heart. Image 8 shows a Doppler of the aorta.

Images 9,10: Image 9 shows superior and inferior vena cava. Image 10 shows corpus callosum and pericallosal artery.

Images 11,12: Sagittal and transverse view of the choanas ruling out a choanal atresia.

Images 13-16: Images of the genitalia showing the hypospadia.

Video 1,2: Videos of the fetal heart showing incomplete atrioventricular septal defect with two atrioventricular valves.

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