title: Biventricular Badness: Rare Images of Ebstein Anomaly of the Tricuspid Valve in a Patient with Hypoplastic Left Heart Syndrome.
abstract: 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.
authors: C.S. Mott Congenital Heart Center, University of Michigan, 1540 E Hospital Dr, Ann Arbor, MI, 48109, USA, dwerho@med.umich.edu.
authors: David K
authors: DK
authors: Werho
authors: Thor
authors: T
authors: Thorsson
authors: Sonal T
authors: ST
authors: Owens
authors: Carlen
authors: C
authors: Fifer
mesh_terms: D001024:Aortic Valve Stenosis / Q000175:diagnosis* / Q000000981:diagnostic imaging; D004437:Ebstein Anomaly / Q000175:diagnosis* / Q000000981:diagnostic imaging; D004452:Echocardiography; D005260:Female; D005865:Gestational Age; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis* / Q000000981:diagnostic imaging; D007231:Infant, Newborn; D011247:Pregnancy; D014261:Tricuspid Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging; D016216:Ultrasonography, Prenatal / Q000379:methods*