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 2016-08-06  Case of the week # 431  © Fabrice Cuillier  www.TheFetus.net
Answer to the case of the week # 431
October 13, 2016 - October 27, 2016

Dr Cuillier F.1, Dr Arsac L.A.2, Dr Fernandez C.3, Dr Alessandri J.L.4, Pr Doray B.5.   

1. Department of Gynecology, Félix Guyon’Hospital, 97400 Saint-Denis, Ile de la Réunion, France. 
2. Department of Cardiology, Félix Guyon’Hospital, 97400 Saint-Denis, Ile de la Réunion, France.
3. Department of Anatomo-pathology, Félix Guyon’Hospital, 97400 Saint-Denis, Ile de la Réunion, France. 
4. Department of Neonatology, Félix Guyon’Hospital, 97400 Saint-Denis, Ile de la Réunion, France.
5. Department of Genetic, Félix Guyon’Hospital, 97400 Saint-Denis, Ile de la Réunion, France. 



Case report

A 26-year old (G1P0) with unremarkable history and  low risk first trimester screening was sent to our unit for routine examination at 23 weeks of gestational age.
Our sonography revealed the following revealed a large interventricular septal defect, only one vessel arising from the right ventricle, and two pulmonary arteries behind this vessel. No other abnormalities were found.
Our prenatal diagnosis was isolated truncus arteriosus from the right ventricle.

The parents were informed about the prognosis, the lack of surgical reparation and the high risk of in utero death. An interruption of pregnancy was performed at 25 weeks of gestational age just after the amniocentesis (normal karyotype) and fetal lysis.
The anatomo-pathologist examination confirmed our diagnosis. 


Images 1-4:  2D images show normal atrio-ventricular concordance and a large interventricular septal defect. 

  



Videos 1 and 2: 2D images show normal atrio-ventricular concordance and a large interventricular septal defect.

 



Image 5, video 3:  2D sagital images of the fetal hear show abnormal ventriculo-arterial concordance with only one vessel arising from the anterior right ventricle. 

 



Images 6, 7 and 8.  Doppler images show only one vessel from the right ventricle. There was a post-valvular dilation and valve abnormal movements during the examination. The speed was 120 cm/s.

 



Videos 4 and 5:  2D images show only one vessel from the right ventricle. There was a post-valvular dilation and valve abnormal movements during the examination. The descendant aorta present a normal diameter. Inferior and superior pulmonary veins flows on the right atrium.
 

 



Images 9 and 10: two pulmonary arteries were present. Inferior and superior pulmonary veins flows on the right atrium.
 

 



Video 6: visualization of the two pulmonary arteries.





Image 11:  Superior and inferior vena cava were normal.





Image 12-15: post-mortem images confirmed the diagnosis of truncus arteriousus arising from the right ventricle and large interventricular septal defect.

  

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