Pulmonary Valve Dysplasia

Fabrice Cuillier, MD

Department of Gynecology, FĂ©lix Guyon Hospital, 97400 Saint-Denis, Ile de la RĂ©union, France.

Case report

This is a 30-year-old woman (G1P0) who was referred to our antenatal unit at 31 weeks of pregnancy for a routine examination. There was no family history of congenital disease.

In our antenatal unit, we noticed normal fetal morphology except for the heart.

After counselling, the patient accepted to perform amniocentesis and another sonography (which confirmed our suspected diagnosis at 35 weeks of gestation). Fetal karyotype was normal. Williams and Di-George deletions were rule out.

The patient delivered at 39 weeks. The baby had a normal post-natal adaptation without cyanosis.

Post-natal echocardiography confirmed the prenatal diagnosis of pulmonary valvular dysplasia with moderate stenosis (with intact septum). 

Here the findings we observed at 31 weeks of gestation:

Images 1, 2: 2D images of the fetal heart showing normal aortic and atrio-ventricular valves.

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Images 3, 4, 5: 2D images showing abnormal pulmonary artery enlargement without atrial or ventricular hypertrophy. 

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2B-Fon_J
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Images 6, 7 and 8: Doppler images of the outflow tracts showing normal aortic and atrio-ventricular valves (Image 6). Images 7 and 8 show abnormal post-valvular dilatation and flow acceleration (> 100 m/s) in pulmonary valve.

3-Fon_J
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4B-Fon_J_-jpg

Videos 1-4: 2D images of the pulmonary artery showing moderate valvular stenosis with post-valvular dilatation.

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