Eine Ebstein-Anomalie als seltene Ursache eines nicht immunologischen Hydrops fetalis (NIHF): pränatale Diagnose mittels Doppler-Echokardiographie

Abstract

The Ebstein's malformation occurs in 0.5% of patients with congenital heart disease. The prenatal diagnosis of such a malformation in the 33rd week of pregnancy is reported. The fetal echocardiography was performed owing to a severe nonimmune hydrops fetalis. The typical distal displacement of the annular attachment of the tricuspid valve leaflets could be viewed in the apical four-chamber view. The application of the pulsed Doppler ultrasound enables the analysis of the cardial haemodynamics and thus the assessment of the severity and prognosis of the diagnosed malformation. In our case the prognosis was interdisciplinary estimated as being very poor (severe cardiac lesion with congestive heart failure already in utero); In the following days intrauterine death occurred. The autopsy confirmed all the prenatal findings.