This is a case of a 26-year-old woman, G1 P0, who was referred to our antenatal unit at 31 weeks of gestation for a fetal cardiac scan. The previous ultrasound performed at 24 weeks of gestation was reported as normal.
Our ultrasound detected the following:
- Marked right cardiomegaly with dilated right atrium
- Dysplastic tricuspid valve with regurgitation
- Dilated pulmonary artery with normal branches
A follow-up scan was performed at 33 weeks of gestation. Cardiomegaly was still present. The tricuspid valve was markedly dysplastic, with severe regurgitation. Doppler images revealed "to-and-fro" flow between the right atrium and ventricle. The right ventricular wall was hypertrophic. The pulmonary artery was markedly dilated and pulmonary valve dysplasia seemed present. Pulmonary valve was thickened and stenotic. A pulmonary artery reverse flow was noted. The Doppler of the ductus venosus showed a reversed flow.
After extensive counselling, the patient decided for an amniocentesis with a normal result, karyotype 46XX. The screening tests for fetal infection were negative (Toxoplasmosis, Rubeola, CMV, Herpes, Coxsackie, Enterovirus and Chikungunya).
The patient continued in her pregnancy. She delivered at term and the postnatal cardiac scan confirmed our diagnosis. The cardiac function was abnormal, with a severe pulmonary stenosis. The neonate was transferred to a pediatric cardiac surgery unit in Paris, France for the surgical intervention.
Videos 1,2: Video 1 shows the dilated right atrium, thickened right ventricular wall and the dysplastic tricuspid valve with tricuspid regurgitation. Video 2 shows a right outflow tract with dilated main pulmonary artery and the thickened pulmonary valve.