title: Impact of prenatal haemodynamic and functional abnormalities in Ebstein's anomaly on survival.
abstract: Predicting outcomes of foetuses with Ebstein's anomaly and tricuspid valve dysplasia continues to be challenging. Limited data exist on the prognostic significance of prenatal haemodynamic and functional parameters in this population. Our aim was to investigate the prognostic significance of haemodynamic and ventricular functional parameters in addition to associated morphometric parameters in patients with Ebstein's anomaly. We reviewed medical records of foetuses with Ebstein's anomaly and tricuspid valve dysplasia at All Children's Hospital Johns Hopkins Medicine and Johns Hopkins University between 2005 and 2012. The main outcome was survival past 30 days from birth; participants who died in utero or <30 days after birth were considered non-survivors. There were 13 survivors and seven non-survivors. We found that participants with abnormal right ventricular function predicted by low tricuspid regurgitation velocity (<2.3 m/second) (p=0.012) and low estimated right ventricular pressure (<24 mmHg) (p=0.029), a low (<7) cardiovascular profile score (p=0.029) and high (>0.53) cardiothoracic ratio (p=0.008) at the first foetal echocardiogram were less likely to survive. In addition, participants with a fossa ovalis/atrial septal length ratio <0.36 at the last foetal echocardiogram (p=0.051) were more likely to die, albeit of borderline statistical significance. Low tricuspid regurgitation velocity and low right ventricular estimated pressure, or a low cardiovascular profile score could be potential prognostic factors for Ebstein's anomaly and tricuspid valve dysplasia. However, future larger prospective studies are needed to confirm these initial findings.
authors: 1Johns Hopkins All Children's Heart Institute,All Children's Hospital,St Petersburg,Florida,United States of America.
authors: Grace
authors: G
authors: Freire
authors: 1Johns Hopkins All Children's Heart Institute,All Children's Hospital,St Petersburg,Florida,United States of America.
authors: Thieu
authors: T
authors: Nguyen
authors: 3Department of Cardiology,Johns Hopkins University,Baltimore,Maryland,United States of America.
authors: Priya
authors: P
authors: Sekar
authors: 1Johns Hopkins All Children's Heart Institute,All Children's Hospital,St Petersburg,Florida,United States of America.
authors: Marilyn
authors: M
authors: Wilhm
authors: 1Johns Hopkins All Children's Heart Institute,All Children's Hospital,St Petersburg,Florida,United States of America.
authors: Kathy
authors: K
authors: Arnold
authors: 2Clinical and Translational Research Office,Johns Hopkins All Children's Hospital,St Petersburg,Florida,United States of America.
authors: Jenny
authors: J
authors: Leshko
authors: 2Clinical and Translational Research Office,Johns Hopkins All Children's Hospital,St Petersburg,Florida,United States of America.
authors: Ernest K
authors: EK
authors: Amankwah
authors: 4Department of Perinatal Cardiology,All Children's Hospital,St Petersburg,Florida,United States of America.
authors: James
authors: J
authors: Huhta
mesh_terms: D015331:Cohort Studies; D004437:Ebstein Anomaly / Q000000981:diagnostic imaging / Q000401:mortality* / Q000503:physiopathology; D004452:Echocardiography; D005313:Fetal Death; D005865:Gestational Age; D006439:Hemodynamics / Q000502:physiology*; D006801:Humans; D007231:Infant, Newborn; D066087:Perinatal Death; D012189:Retrospective Studies; D014262:Tricuspid Valve Insufficiency / Q000000981:diagnostic imaging / Q000503:physiopathology*; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging / Q000503:physiopathology*; D016278:Ventricular Function, Right; D017725:Ventricular Pressure / Q000502:physiology
keywords: right ventricular pressure