Prenatal Diagnosis of Severe Tricuspid Insufficiency in Ebstein's Anomaly with Pulmonary Atresia and Intact Ventricular Septum: A Case Report

Abstract

Pulmonary atresia with intact ventricular septum is an uncommon congenital cardiac anomaly which very often present varying degrees of downward displacement and dysplasia of the tricuspid valve. A 23-year-old woman was referred at 23 weeks' gestation because of fetal cardiomegaly detected by routine ultrasonography. Ebstein's anomaly with pulmonary atresia was diagnosed by fetal echocardiography. On pulsed Doppler examination, a severe tricuspid regurgitation was detected. Pulmonary atresia was suspected, as forward flow was not detected into pulmonary artery. The fetus was terminated at 25 weeks. At the postmorterm examination Ebstein's anomaly with pulmonary atresia and intact ventricular septum were confirmed. The finding of right atrial enlargement on an early sonogram should prompt the search for tricuspid valve abnormalities with tricuspid regurgitation and the presence or development of pulmonary atresia or stenosis. With the information provided, early detection of tricuspid valve disease should be possible so that the option of therapeutic abortion can be offered.

Fetal Heart Size

“…Comparisons between the point-to-point trace and electronic ellipse methods for measurements of the area and circumference were performed by using within-subject repeated measures analysis of variance (NCSS 10; NCSS, Ebstein's malformation 7,14,48,57,61,69 Tricuspid valve dysplasia 7,44,56 Tricuspid valve atresia 44…”

The attempt of pregnancy modification in a case of fetal Ebstein’s anomaly

“…The diagnosis in the prenatal period carries a poor prognosis for both fetus and neonate [10]. In many centres in the world, pregnant women diagnosed with Ebstein's syndrome in the fetus choose the option of termination of pregnancy [19].…”