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2013-09-10-09 Case of the week #354 © Bronshtein www.TheFetus.net 

 

Answer to the case of the week #354

September 26, 2013 - October 10, 2013

 

Moshe Bronshtein

Haifa, Israel.


Case report

Critical pulmonary stenosis secondary to supravalvular membrane 

This was a secundigravida with low risk pregnancy, first time seen at 15 weeks of her gestation. Gray scale ultrasound did not find any conspicuous cardiac abnormalities, but thorough observation revealed a tiny membrane above normal opening of the pulmonary semilunar valve - supravalvular pulmonary membrane. Additionally color Doppler imaging found tricuspid regurgitation. At 19 weeks hypoplastic right heart syndrome was present and pregnancy was terminated.


Videos 1, 2: 15 weeks of gestation; Video 1 shows tricuspid regurgitation and normal size of the right ventricle. Video 2 shows a tiny membrane localized above the semilunar valve of the pulmonary artery (green arrow).

 

Video 3: 17 weeks of gestation; the video shows a tiny membrane localized above the semilunar valve of the pulmonary artery (green arrow).



Images 1, 2: 17 weeks of gestation; the images show pulmonary artery with supravalvular membrane (blue arrow) localized above cusps of the semilunar valve (white arrows). RV - right ventricle; A - aorta; rpa - right branch of the pulmonary artery. 

 

Image 3: 17 weeks of gestation; the image shows detailed view at supravalvular membrane (green arrowhead) localized above cusps of the semilunar valve (white arrows) of the pulmonary artery. RV - right ventricle; A - aorta; rpa - right branch of the pulmonary artery. 



Images 4, 5, 6, 7, and 8: the images show color Doppler images of the pulmonary artery with supravalvular membrane during cardiac cycle:

Image 4 - first systolic phase: a
 narrow jet through the tiny intramembranous orifice (blue arrow) can be seen;
Image 5 - second systolic phase:  forward flow within the pulmonary artery with high speed in the midstream can be seen (blue arrow);
Image 6 - third systolic phase: mild pulmonary regurgitation (red arrow) can be seen;
Image 7 - fourth systolic phase: beginning of reversed flow from the aorta - two flows in the pulmonary artery can be seen in the same phase - one from the aorta (red one) and the second one from the right ventricle (blue one);
Image 8 - fifth systolic phase: end systolic flow within the pulmonary artery - only reversed flow from the aorta can be seen.


  

  



Videos 4, 5, and 6: 19 weeks of pregnancy; transvaginal sonography - the videos show hypoplastic right ventricle with markedly diminished inflow (video 4). The video 5 shows tiny orifice in the supravalvular membrane within the pulmonary artery (green arrowhead). Video 6 shows bidirectional flow within the pulmonary artery.

 



Image 9: Histological image of the supravalvular membrane within the pulmonary artery (blue rectangle - the supravalvular membrane 0.5 mm; the arrow points at the narrow intramembranous orifice).


 

References

1. Bronshtein M, Zimmer EZ, Blazer S, Blumenfeld Z. Prenat Diagn. 2012 May;32(5):444-9. doi: 10.1002/pd.3836. Epub 2012 Apr 12.Transient abnormal fetal cardiac flow patterns at 13 to 17 gestational weeks. Prenat Diagn. 2012 May;32(5):444-9. doi: 10.1002/pd.3836. Epub 2012 Apr 12. 

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