Case of the Week #604

Frantisek Grochal
Femicare, Center of prenatal ultrasonographic diagnostics, Martin, Slovakia

Posting Dates: Apr 30, 2024 - May 14, 2024

A 29-year-old G2P1 presented to our office at 36 weeks, 5 days gestation due to an abnormal cardiac finding.

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Answer

We present a case of pulmonary atresia with intact ventricular septum (PAIVS) and severe tricuspid dysplasia and regurgitation.

Our examination identified the following findings:

Marked cardiomegaly / wall-to-wall heart (cardiothoracic area ratio 0,82)
Severe tricuspid regurgitation with striking enlargement of the right atrium and ventricle
Large atrial septal defect – common atrium
Small pulmonary artery and branches
Tortuous ductus arteriosus with retrograde flow
Pulmonary hypoplasia
Oligohydramnios

A female infant with birthweight 2730g was born spontaneously two days later after our examination and immediately transported to the National Institute for Cardiovascular Diseases. Diagnosis of pulmonary atresia with intact ventricular septum (PAIVS) and severe tricuspid dysplasia was confirmed. The infant has undergone several surgical operations including Starnes' operation, thymectomy, placement of the Laks' shunt, resection of the right atrium, right atrial appendage ligation, and Dual Chamber Pacemaker DDD placement due to the third-degree, and later second-degree atrioventricular block. The postoperative course was complicated by Pseudomonas aeruginosa sepsis, Enterococcus faecalis bacteremia, artificial ventilation, laryngomalacia, cardiac decompensation, hepatomegaly, lung hypoplasia, and pneumopathy requiring intensive care. The baby is 9 months old and is doing relatively well; awaiting potential bidirectional Glenn shunt procedure.

Image 1 and Video 1 show a transverse sweep through the fetal abdomen and thorax showing a four-chamber-view of the heart with marked cardiomegaly (wall-to-wall heart) with dominant enlarged right ventricle and atrium. Left and right atria communicate via large defect (common atrium). A dysplastic tricuspid valve can also be seen. Lung hypoplasia is also evident. LA – left atrium, RA – right atrium, RV – right ventricle, LV – left ventricle, Ao – aorta, St - stomach.

Image 2 and Video 2 show a 3D/4D imaging of the fetal thorax showing a four-chamber-view of the heart with marked cardiomegaly (wall-to-wall heart) with dominant enlarged right ventricle and atrium. Left and right atria communicate via large defect (common atrium). A dysplastic tricuspid valve can also be seen. Lung hypoplasia is also evident. LA – left atrium, RA – right atrium, RV – right ventricle, LV – left ventricle, Ao – aorta.

Video 3 and Video 4 represent a transverse scan of the fetal thorax showing marked cardiomegaly (wall-to-wall heart) with dominant enlarged right ventricle and atrium. Due to severe cardiomegaly classical views of the left and right outflows and three-vessel-trachea view were not typically depicted, but as the sweep of the probe goes through the heart, aorta (Ao), tortuous ductus arteriosus (DA), hypoplastic pulmonary artery (PA) and its branches can be seen.

Video 5 and Image 3 represent a sagittal image of the heart demonstrating the aortic arch (Ao arch) and reverse flow within ductus arteriosus (DA).

Video 6 and Image 4 represent a color Doppler and spectral Doppler scan of the fetal thorax showing marked cardiomegaly (wall-to-wall heart) with severe tricuspid regurgitation.