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2007-12-23-03 Answer to the case of the week #219 © Cuillier www.TheFetus.net


Answer to the case of the week #219

May 22 - June 5, 2008

Fabrice Cuillier, MD*; P. Dussautoir, MD**; G. Kah, MD**; J. P. Riviere, MD***.

*     Department of Gynecology, Félix Guyon"Hospital, 97400 Saint-Denis, Ile de la Réunion, France;  
**   Gynecologist, Moufia"street, 97400 Saint-Denis, Ile de la Réunion, France; 
***  Department of Anatomopathology, Félix Guyon"Hospital, 97400 Saint-Denis, Ile de la Réunion, France.

 

Case report

A 35-year-old woman, G3P2, was referred to our prenatal unit at 23 weeks of pregnancy to assess a heart anomaly. Her two children are healthy. During our examination, the following findings were made:

  • Cardiomegaly with right ventricular dilatation;
  • Dysplastic, nearly absent tricuspid valve;
  • Hypoplastic pulmonary artery and pulmonary branches.

The remaining fetal morphology was normal. The following scan was done at 27 weeks and fetal morphology appeared the same. Doppler images revealed a to-and-fro flow across the right ventricle and right atrium. The right ventricle was dilated with a very thin myocardium. At the level of the hypoplastic pulmonary artery, reversed flow was also visible.

An amniocentesis was done, revealing normal karyotype (46,XX). Screening tests for fetal infections (toxoplasmosis, MNI, rubeola, CMV, herpes, Coxsackie, enterovirus and Chikungunya) were negative.

The findings implied a diagnosis of an unguarded tricuspid orifice. The parents opted to continue the pregnancy; the diagnosis was confirmed after delivery (the tricuspid valve was absent and the mitral valve was normal). The neonate died two days after delivery due to cardiac insufficiency.

Images 1, 2: Image 1 shows a four chamber view of the heart. Tricuspid valve is missing and the right ventricle and atrium are dilated.  Image 2 shows the left outflow tract of the heart. Dilatation of the right ventricle can be seen.

 

Images 3, 4: Color Doppler images showing the left outflow tract of the heart and dilated right side of the heart and atrial septal defect.

 

Images 5, 6: Color Doppler images showing the left outflow tract of the heart and dilated right side of the heart and atrial septal defect.

 

Images 7, 8: Color Doppler and gray scale images comparing the diameter of the aorta (Ao) and thin pulmonary artery (AP) and its branches.

 

Images 9, 10: The image 9 represents a gray scale image comparing the diameter of the aorta and thin pulmonary artery. The image 10 shows a comparison between the thin pulmonary artery and aorta at the level of the 3-vessel view.

 

Video 1: The video shows color Doppler and gray scale sequences showing the unguarded tricuspid orifice anomaly.

 

 

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