Dagmar Wertaschnigg1, Cedric Manlhiot1, Michelle Jaeggi1, Mike Seed1, Andreea Dragulescu1, Steven M Schwartz2, Glen van Arsdell3, Edgar T Jaeggi4. 1. Labatt Family Heart Center, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. 2. Labatt Family Heart Center, Department of Critical Care Medicine, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. 3. Labatt Family Heart Center, Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. 4. Labatt Family Heart Center, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: edgar.jaeggi@sickkids.ca.
Abstract
BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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. Copyright Â
BACKGROUND: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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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. Copyright Â
Authors: Lindsay R Freud; Doff B McElhinney; Brian T Kalish; Maria C Escobar-Diaz; Rukmini Komarlu; Michael D Puchalski; Edgar T Jaeggi; Anita L Szwast; Grace Freire; Stéphanie M Levasseur; Ann Kavanaugh-McHugh; Erik C Michelfelder; Anita J Moon-Grady; Mary T Donofrio; Lisa W Howley; Elif Seda Selamet Tierney; Bettina F Cuneo; Shaine A Morris; Jay D Pruetz; Mary E van der Velde; John P Kovalchin; Catherine M Ikemba; Margaret M Vernon; Cyrus Samai; Gary M Satou; Nina L Gotteiner; Colin K Phoon; Norman H Silverman; Wayne Tworetzky Journal: J Am Heart Assoc Date: 2020-10-20 Impact factor: 5.501