Biventricular Badness: Rare Images of Ebstein Anomaly of the Tricuspid Valve in a Patient with Hypoplastic Left Heart Syndrome
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We report a rare case of hypoplastic left heart syndrome coexisting in a patient with Ebstein anomaly of the tricuspid valve, which has previously been described only in pathological studies. A fetal echocardiogram at 27-weeks gestation showed severe aortic stenosis with evolving hypoplastic left heart syndrome, significant endocardial fibroelastosis, a dysplastic tricuspid valve with moderate regurgitation, right atrial and ventricular dilation, and signs of fetal congestive heart failure. Due to inadequate left heart size, the patient was not a candidate for fetal intervention for critical aortic stenosis, and repeat studies showed progression of the lesion through the pregnancy. The infant was delivered at 36-weeks gestation with signs of hydrops, and a postnatal echocardiogram confirmed hypoplastic left heart syndrome as well as severe Ebstein anomaly of the tricuspid valve. The infant did not survive to intervention.
KeywordsHypoplastic left heart syndrome Ebstein anomaly Hydrops Congenital heart disease Echocardiography
Conflict of interest
The authors declare that they have no conflicts of interest.
Supplementary material 1 Video 1 2D Fetal sweep: L indicates left; P, posterior; R, right. Sweeping through the fetal heart from apex to base, we first see the dilated right ventricle and the apically displaced orifice of the dysplastic tricuspid valve en face, and finally the dilated right atrium and the left ventricle with severely depressed systolic function. (MPG 6328 kb)
Supplementary material 2 Video 2 2D Subcostal coronal sweep: Sweeping from posterior to anterior, we first see the dilated right atrium with redundant Eustachian valve tissue and the atrialized right ventricle as well as a patent foramen ovale, followed by the apically displaced tricuspid valve and functionally small right ventricle as well as the severely depressed and echobright left ventricle with endocardial fibroelastosis. Finally, we see the dysplastic tricuspid valve en face. (MPG 4700 kb)
Supplementary material 3 Video 3 Color Doppler sweep through the tricuspid valve in the parasternal short axis: This view demonstrates the broad-based jet of tricuspid regurgitation. Toward the end of the sweep, we see the nearly akinetic left ventricle and then the stenotic aortic valve with a small amount of antegrade flow. (MPG 4762 kb)