title: Ebstein's Anomaly of the Tricuspid Valve in the Fetus - A Multicenter Experience.
abstract: PurposeTo assess the spectrum of associated anomalies, the intrauterine course, the outcome and possible prognostic markers in prenatally diagnosed Ebstein's anomaly (EA).Materials and MethodsAll cases of EA diagnosed over a period of 13 years with a minimum follow-up of 1 year were retrospectively collected in 4 tertiary referral centers in Germany.ResultsIn the study period 76 cases of EA were prenatally diagnosed. The mean gestational age at diagnosis was 25.0 weeks (range: 13 - 35). 41 (53.9 %) cases were isolated and 35 (46.1 %) had other cardiac and/or extracardiac anomalies. 19 (25.0 %) pregnant women opted for termination of pregnancy, intrauterine fetal death occurred in 7 cases (9.2 %), neonatal death in 14 cases (18.4 %), death in infancy or childhood in 9 cases (11.8 %) and 27 children (35.5 %) were alive at the last follow-up. After exclusion of terminations, the only parameter inversely correlated with intrauterine survival was hydrops fetalis. Prognostic parameters significantly associated with postnatal non-survival were an abnormal Celermajer index (right atrium/heart ratio > 0.7), cardiomegaly (cardiothoracic circumference ratio > 0.5), absence of antegrade flow over the pulmonary valve and earlier diagnosis in pregnancy.ConclusionPrenatally diagnosed EA has a high morbidity and mortality with the highest loss rate in the intrauterine and neonatal period. In our study, hydrops fetalis was the only parameter significantly associated with intrauterine demise, while other prenatal markers were only significantly associated with postnatal mortality.
authors.affiliation: Division of Prenatal Medicine and Gynecologic Sonography, Department of Obstetrics and Gynecology, University of Köln, Germany.
authors.forename: Ingo
authors.initials: I
authors.lastname: Gottschalk
authors.affiliation: Division of Prenatal Medicine and Gynecologic Sonography, Department of Obstetrics and Gynecology, University of Köln, Germany.
authors.forename: Lea
authors.initials: L
authors.lastname: Gottschalk
authors.affiliation: praenatal.de Köln, Prenatal Medicine and Genetics, Köln, Germany.
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Stressig
authors.affiliation: praenatal.de Köln, Prenatal Medicine and Genetics, Köln, Germany.
authors.forename: Jochen
authors.initials: J
authors.lastname: Ritgen
authors.affiliation: Department of Pediatric Cardiology, University of Bonn, Germany.
authors.forename: Ulrike
authors.initials: U
authors.lastname: Herberg
authors.affiliation: Department of Pediatric Cardiology, University of Bonn, Germany.
authors.forename: Johannes
authors.initials: J
authors.lastname: Breuer
authors.affiliation: Department of Paediatric Cardiology and Congenital Heart Defects, German Heart Centre Munich, Technical University Munich, Germany.
authors.forename: Renate
authors.initials: R
authors.lastname: Oberhoffer
authors.affiliation: Department of Obstetrics and Prenatal Medicine, University of Bonn, Germany.
authors.forename: Arne
authors.initials: A
authors.lastname: Willruth
authors.affiliation: Department of Obstetrics and Prenatal Medicine, University of Bonn, Germany.
authors.forename: Brigitte
authors.initials: B
authors.lastname: Strizek
authors.affiliation: Department of Obstetrics and Prenatal Medicine, University of Bonn, Germany.
authors.forename: Annegret
authors.initials: A
authors.lastname: Geipel
authors.affiliation: Department of Obstetrics and Prenatal Medicine, University of Bonn, Germany.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Gembruch
authors.affiliation: Division of Prenatal Medicine and Gynecologic Sonography, Department of Obstetrics and Gynecology, University of Köln, Germany.|Department of Obstetrics and Prenatal Medicine, University of Bonn, Germany.
authors.forename: Christoph
authors.initials: C
authors.lastname: Berg
mesh_terms: D002648:Child; D004437:Ebstein Anomaly / Q000000981:diagnostic imaging; D005260:Female; D005333:Fetus; D005858:Germany; D006801:Humans; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012189:Retrospective Studies; D014261:Tricuspid Valve / Q000002:abnormalities / Q000000981:diagnostic imaging