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 2017-02-27  Case of the week # 441  © Fabrice Cuillier
Answer to the case of the week # 441
March 16, 2017 - March 30, 2017

Fabrice Cuillier*, Dr Arsac L.A.**, Dr Alessandri J.L.***, Pr Doray B. ****  

* Department of Gynecology, Félix Guyon’Hospital, 97400 Saint-Denis, Ile de la Réunion, France. 
** Department of Pediatric cardiology, Félix Guyon’Hospital, 97400 Saint-Denis, Ile de la Réunion.  
*** Department of Neonatology, Félix Guyon’Hospital, 97400 Saint-Denis, Ile de la Réunion. 
**** Department of Genetic, Félix Guyon’Hospital, 97400 Saint-Denis, Ile de la Réunion, France. 

Case report

This is a case of a 26-year old woman (G1P0), who was referred to our antenatal unit at 33 weeks of gestational age. Patient had non-contributive personal or family history. The previous ultrasound was performed at 13 weeks and 22 weeks and didn't show any anomalies. 

At 33 weeks, our ultrasound examination  and a postnatal echocardiography performed at 3 months (images 7 and 8) revealed
one little echogenic tumor attached inside the right atrium. The left atrium was normal, as the right and left ventricle. Both outflow tracts were normal. At 34 and 38 weeks of pregnancy, the cardiac anomaly wasn´t change.

Our diagnosis based on the ultrasound examination was  cardiac fibroma, rhabdomyomas or myxoma.  

We scanned the fetal brain to exclude the presence of "tubers" as a typical sign for Tuberous Sclerosis. The brain and the others organs (as kidney) looked normal without any anomalies. The patient refused any other investigation.

Patient continue her pregnancy and delivered at term without any complications. The postnatal adaptation was uneventful. Postnatal echocardiography confirmed our diagnosis (isolated right atrium tumor), but 
three months later, an another echocardiography showed a new intracardiac tumors suggesting the diagnosis of rhabdomyomas.
Finally, Tuberous Sclerosis was confirmed.

Video 1
video of the fetal heart showing right intra-auricular tumor. 

Images 1, 2, 3 and 4: 2D views of the fetal heart shows normal right and left ventricular at 33 weeks of gestation.
Our ultrasound examination revealed one little echogenic tumor attached into the right atrium. Color and pulsed doppler ultrasound showed normal flow from left ventricule and from right ventricle (not shown).


Images 5 and 6: 2D images of the fetal heart at 34 and 38 weeks, without changes.



Images 7 and 8: post-natal ecocardiography images at 3 months of life showed a new intracardiac tumor. The diagnosis of rhabdomyomas in the context of Tuberous Sclerosis was confirmed.



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