Fetal diagnosis and prognosis of Ebstein's anomaly

Currently fetal echocardiography may confirm the diagnosis in utero of Ebstein anomaly, as well as determine the perinatal outcome with high certainty. To review 16 cases diagnosed with Ebstein anomaly, by fetal echocardiography, analyzing prognostic echocardiographic parameters set by Pavlova and colleagues. A descriptive, observational, retrospective study was done in patients with fetal diagnosis of Ebstein anomaly, during January 2001 to December 2011. Echocardiographic parameters are analyzed and its correlation to perinatal evolution of 16 cases of Ebstein anomaly. Maternal age was of 27.94 + 5.7 years, gestational age was 31.3 +/- 3.6 weeks. In utero mortality represented 37.5% (n = 6), and neonatal mortality 50% (n = 8), two patients (12.5%) survived. Cardiothoracic index was of 0.61 +/- 0.074, ratio foramen ovale-atrial septal was of 0.6 +/- 0.015, obstruction of the outflow tract of the right ventricle was seen in 14 (87.5%) patients; 81% of the deceased had a degree of displacement valve > 2.5; ratio right ventricle-left ventricle 2.24 +/- 0.37. The umbilical vein was throbbing in 64% of the deceased, the tricuspid insufficiency was severe in 15 cases (94%, 21.62 +/- 2.82 mmHg), hydrops affected 18.7% of patients. The following factors are of bad prognosis in fetal stage of Ebstein anomaly: cardiothoracic index > 0.55, relative foramen ovale-atrial septal 2.5, absence of reverse flow in the duct arteriosus, ratio right ventricle-left ventricle > 2. The Ebstein anomaly diagnosed in utero has a perinatal mortality of 87.5%.