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2013-06-22-22 Early pulmonary valve anomalies © Bronshtein www.TheFetus.net


Early pulmonary valve anomalies

Moshe Bronshtein
, MD

Haifa, Israel.

Case 1: Pulmonary stenosis and tricuspid regurgitation

This was a low risk pregnancy of a 28 year-old woman. Her ultrasonographic examination at 15 weeks showed normal gray scale image of the heart and outflow tracts, but color Doppler revealed a reversed turbulent flow at the level of the mid portion of the pulmonary artery and tricuspid regurgitation (see videos 1, 2, and 3). At 20 weeks hypoplastic right heart syndrome had been developed and pregnancy was terminated.

The early isolated pulmonary artery stenosis has usually good prognosis [1]. However, associated reversed flow within the pulmonary artery and tricuspid regurgitation are ominous prognostic factors.

Videos 1, 2, and 3: 15 weeks of gestation - pulmonary stenosis; Video 1 shows communication of the pulmonary artery with the aortic arch via ductus arteriosus (yellow V) and turbulent reverse flow within the midportion of the pulmonary artery.  Video 2 shows spectral Doppler with aliasing at the level of the pulmonary artery. Video 3 shows tricuspid regurgitation.

 



 

Case 2: 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. Additionally color Doppler imaging found tricuspid regurgitation.

At 19 weeks hypoplastic right heart syndrome was present and pregnancy was terminated.


Videos 4, 5: 15 weeks of gestation; Video 4 shows tricuspid regurgitation and normal size of the right ventricle. Video 5 shows a tiny membrane localized above the semilunar valve of the pulmonary artery (green arrow).

 

Video 6: 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 7, 8, and 9: 19 weeks of pregnancy; transvaginal sonography - the videos show hypoplastic right ventricle with markedly diminished inflow (video 7). The video 8 shows tiny orifice in the supravalvular membrane within the pulmonary artery (green arrowhead). Video 9 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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