title: Tricuspid valve dysplasia with severe tricuspid regurgitation: fetal pulmonary artery size predicts lung viability in the presence of small lung volumes.
abstract: Congenital tricuspid valve disease (Ebstein's anomaly, tricuspid valve dysplasia) with severe tricuspid regurgitation and cardiomegaly is associated with poor prognosis. Fetal echocardiography can accurately measure right atrial enlargement, which is associated with a poor prognosis in the fetus with tricuspid valve disease. Fetal lung volumetric assessments have been used in an attempt to predict viability of fetuses using ultrasonogram and prenatal MRI. We describe a fetus with tricuspid dysplasia, severe tricuspid regurgitation, right atrial enlargement and markedly reduced lung volumes. The early gestational onset of cardiomegaly with bilateral lung compression raised the possibility of severe lung hypoplasia with decreased broncho-alveolar development. Use of fetal echocardiography with measurement of pulmonary artery size combined with prenatal MRI scanning of lung volumes resulted in an improved understanding of this anomaly and directed the management strategy towards a successful Fontan circulation.
authors.affiliation: Cardiac Center, Children's Hospital of Philadelphia, University of Pennsylvania, 19104, USA.
authors.forename: A T
authors.initials: AT
authors.lastname: Nathan
authors.forename: B S
authors.initials: BS
authors.lastname: Marino
authors.forename: T
authors.initials: T
authors.lastname: Dominguez
authors.forename: S
authors.initials: S
authors.lastname: Tabbutt
authors.forename: S
authors.initials: S
authors.lastname: Nicolson
authors.forename: D D
authors.initials: DD
authors.lastname: Donaghue
authors.forename: T L
authors.initials: TL
authors.lastname: Spray
authors.forename: J
authors.initials: J
authors.lastname: Rychik
mesh_terms: D000328:Adult; D006332:Cardiomegaly / Q000000981:diagnostic imaging / Q000196:embryology / Q000601:surgery; D002315:Cardiopulmonary Bypass; D004437:Ebstein Anomaly / Q000175:diagnosis* / Q000601:surgery; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging; D005865:Gestational Age; D006325:Heart Atria / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000000981:diagnostic imaging / Q000196:embryology*; D008279:Magnetic Resonance Imaging; D011247:Pregnancy; D011296:Prenatal Diagnosis / Q000379:methods*; D011651:Pulmonary Artery / Q000196:embryology* / Q000601:surgery; D014261:Tricuspid Valve / Q000002:abnormalities* / Q000196:embryology / Q000601:surgery; D014262:Tricuspid Valve Insufficiency / Q000000981:diagnostic imaging* / Q000601:surgery; D016216:Ultrasonography, Prenatal