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2018-12-20  Tetralogy of Fallot (TOF) with absent pulmonary valve syndrome (APVS) and absent ductus arteriosus (ADA)  © Hitanshu Bhatt

Absent pulmonary valve syndrome
Dr. Hitanshu Bhatt; Dr. Gouri Nagraj Bhatt

Consultant Fetal Maternal medicine, Surat, India

Case report

Primigravida 18-year-old with non contributory history, was referred for second opinion for suspected congenital Heart anomaly at 22 weeks of gestation.
Our examination revealed the following findings:
-Situs Solitus;
-Veno-atrial connections: concordant;
-Atrio-ventricular connections: concordant;
-Ventriculo-arterial connections: concordant;
-LVOT: Perimembranous ventricular septal defect with overriding of Aorta (TOF variant);
-RVOT: small main pulmonary artery with absent/ unguarded/ dysplastic pulmonary valve resulting into dilated branched pulmonary arteries suggestive of absent pulmonary valve syndrome;
-Arch view: absent ductus arteriosus (ADA);
-No significant MAPCAs noted;

The diagnosis was confirmed after birth.

Images 1-3: 2D and color Doppler images showing dilated right and left pulmonary arteries.

Image 4: spectral Doppler shows increased velocities in the main pulmonary artery, which is stenotic and incompetent.

Images 5 and 6: 2D and color Doppler of 4-chamber view showing normal pulmonary veins draining into left atrium.

Images 5 and 6: 2D and color Doppler showing the perimembranous ventricular septal defect.

Images 7 and 8: color Doppler images showing absent ductus arteriosus.

Videos 1-3: 2D images of the fetal heart showing dilated right and left pulmonary arteries, dysplastic pulmonary valve, perimembranous septal defect and overriding aorta.

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