Ebstein's malformation of the tricuspid valve: Report of 8 patients diagnosed in utero

Ebstein's malformation of the tricuspid valve can be easily recognized during routine prenatal obstetric ultrasonography, since it tends to produce significant regurgitation and cardiomegaly. These abnormalities should prompt the need for fetal echocardiographic evaluation. We describe 8 cases detected prenatally at the Fetal Cardiology Unit from January 1989 to November 1996. The diagnosis of Esbstein's anomaly was established between 24 and 36 weeks of postconceptional age. The main reason for referral was cardiomegaly detected during routine obstetric ultrasound scan. All 8 fetuses had severe tricuspid regurgitation, two with significant hydrops. Two fetuses presented severe pulmonary stenosis and one had mild or functional pulmonary stenosis. One fetus had an associated ASD, but none had VSD. Two patients showed supraventricular tachycardia, treated in utero with maternal digoxin. There were 4 intrauterine deaths despite treatment of heart failure and arrhythmias. One patient died in the neonatal period after attempted corrective surgery. Ebstein's anomaly remains a severe fetal disorder, with gross echocardiographic abnormalities, readily detectable by routine obstetrical scan. It carries a poor prognosis during intrauterine life, which stress the need for early monitoring and treatment.