Ebstein Anomaly Fetal Echocardiogram Parameters are Associated with Illness Severity on Admission to the Cardiac Intensive Care Unit.

backgroundEbstein anomaly is associated with a broad spectrum of presentations. Many fetal factors have been associated with intrauterine fetal demise and early neonatal mortality. Little is described about how fetal factors may influence illness severity in the immediate postnatal period.

objectivesTo identify the relationship between fetal echocardiogram measures and severity of illness on admission utilizing pSOFA scores.

methodsRetrospective chart review of 32 neonates with prenatally diagnosed Ebstein anomaly. Fetal echocardiographic factors assessed included right ventricular pressure (RVp), presence of antegrade pulmonary blood flow, ductus arteriosus (DA) flow direction, and pulmonary regurgitation (PR). pSOFA score was calculated on admission from available data. Patients were evaluated for the composite postnatal outcome of cardiac arrest, need for ECMO, or mortality.

resultsMedian RVp on the last fetal echocardiogram was 26 [22, 35] mmHg. In 40.6% of infants, no antegrade pulmonary blood flow was demonstrated and DA flow was left-to-right. There was more than mild PR in 31.3% of patients. Lower fetal RVp (p = 0.016), lack of antegrade pulmonary blood flow (p = 0.002), left-to-right DA flow (p = 0.001) and PR (p < 0.001) on the last fetal echocardiogram were associated with a higher pSOFA score on admission. In addition, lower fetal RVp (p = 0.003) and lack of antegrade pulmonary blood flow (p = 0.041) were associated with the composite outcome of cardiac arrest, ECMO or mortality.

conclusionsFetal echocardiographic parameters are associated with severity of illness on CICU admission as assessed by pSOFA score. Both lower fetal RVp and lack of antegrade pulmonary blood flow remain associated with neonatal morbidity and mortality.