title: Contemporary Outcomes and Factors Associated With Mortality After a Fetal or Neonatal Diagnosis of Ebstein Anomaly and Tricuspid Valve Disease.
abstract: Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare anomalies and data on outcomes after a fetal or neonatal EA/TVD diagnosis are conflicting.
  
  
  
  To examine the outcome and identify markers predictive of mortality, we reviewed our single-centre experience from 2000-2014. Variables were analyzed separately for cases diagnosed in utero without pregnancy termination and for all live-born patients.
  
  
  
  Of 47 fetal cases, 8 (17%) died in utero and 10 (21%) as neonates. Independent predictors associated with fetal demise included severe tricuspid regurgitation with a Doppler gradient < 40 mm Hg (odds ratio, 1.22 per mm Hg deduction; P = 0.003) and pulmonary regurgitation (odds ratio, 11.4; P = 0.03) at the baseline examination. A novel prognostic score (range, 0-10) combining the severity of 5 echocardiographic findings was independently associated with overall mortality (hazard ratio [HR], 1.39 per point increase; P = 0.01). Survival rates of 66 live births at 1 month, 1 year, and 5 years were 86%, 82%, and 80% respectively, and 75%, 60%, and 55% remained free from surgery at the same points in time. Factors associated with postnatal death in multivariate analysis included a younger gestational age at birth (HR per week, 1.59; P < 0.001), tricuspid annulus diameter (HR per z-score increase, 1.76; P = 0.004), and no pulmonary forward flow (HR, 4.63; P = 0.03).
  
  
  
  Our experience with fetal and neonatal EA/TVD shows better survival rates than previously reported. Mortality after a fetal diagnosis was significantly associated with hemodynamic changes indicative of a circular shunt, including pulmonary and tricuspid regurgitation severe enough to cause diastolic umbilical arterial flow reversal.
authors.affiliation: Labatt Family Heart Center, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Dagmar
authors.initials: D
authors.lastname: Wertaschnigg
authors.affiliation: Labatt Family Heart Center, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Cedric
authors.initials: C
authors.lastname: Manlhiot
authors.affiliation: Labatt Family Heart Center, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Michelle
authors.initials: M
authors.lastname: Jaeggi
authors.affiliation: Labatt Family Heart Center, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Mike
authors.initials: M
authors.lastname: Seed
authors.affiliation: Labatt Family Heart Center, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Andreea
authors.initials: A
authors.lastname: Dragulescu
authors.affiliation: Labatt Family Heart Center, Department of Critical Care Medicine, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Schwartz
authors.affiliation: Labatt Family Heart Center, Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Glen
authors.initials: G
authors.lastname: van Arsdell
authors.affiliation: Labatt Family Heart Center, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: edgar.jaeggi@sickkids.ca.
authors.forename: Edgar T
authors.initials: ET
authors.lastname: Jaeggi
mesh_terms: D002170:Canada / Q000453:epidemiology; D004437:Ebstein Anomaly / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology; D015150:Echocardiography, Doppler / Q000379:methods*; D005260:Female; D005865:Gestational Age; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D054238:Perinatal Mortality; D011237:Predictive Value of Tests; D011247:Pregnancy; D011379:Prognosis; D012189:Retrospective Studies; D014261:Tricuspid Valve / Q000000981:diagnostic imaging / Q000503:physiopathology; D014262:Tricuspid Valve Insufficiency / Q000151:congenital / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology; D016216:Ultrasonography, Prenatal / Q000379:methods