Impact of prenatal haemodynamic and functional abnormalities in Ebstein's anomaly on survival

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.