Ultraschall Med 2008; 29 - PO_43
DOI: 10.1055/s-2008-1080868

„Failing“ right ventricle – follow up after severe dysfunction of the fetal right ventricle

U Herberg 1, U Gembruch 2, C Berg 2, A Geipel 2, A Heep 3, C Schmitz 4, J Breuer 1
  • 1Abteilung für Kinderkardiologie, Universitätsklinikum Bonn, Bonn
  • 2Department of Obstetric and Gynecology, Prenatal Medicine, University of BOnn, Germany, Bonn
  • 3Department of Neonatology, University Bonn, Germany, Bonn
  • 4Dep. Ped. Cardiac Surgery, University Bonn, Germany, Bonn

Introduction: In the fetus, the right ventricle (RV) is the dominant ventricle, contributing more than 50% to the combined cardiac output. RV dysfunction results in severe fetal cardiac failure with sometimes unpredictable postnatal outcome.

Methods: Postnatal follow up of 8 fetuses with severe RV cardiac failure (i.e. absent antegrade flow across the pulmonary trunc, RV hypocontractility, severe tricuspid regurgitation) with following diagnosis: Ebstein´s anomaly with functional pulmonary atresia (3), RV-cardiomyopathy (6), severe hypoplasia of the RV and Uhl´s anomaly with functional pulmonary atresia.

Results: Postnatally all cases showed severe RV dysfunction and ductal depend pulmonary perfusion, 6/7 required mechanical ventilation, 4/7 positive inotropic support, 2 therapy of life threatening arrhythmia.. In fetuses with primarily myocardial disease RV function improved due to the postnatal decrease of pulmonary resistance and anterograde flow across the pulmonary valve was achieved after a mean of 2 days. Even in a fetus with severe RV hypoplasia and prenatal recommendation towards an univentricular approach, RV function improved without need of further intervention. 2 of 3 fetuses with Ebstein´s anomaly and functional pulmonary atresia required interventional pulmonary valve ballooning. One neonate was diagnosed as long QT syndrome, requiring a pacemaker at the age of 28 weeks and weight of 800g.

Conclusions: Postnatally, the prognosis of severe RV dysfunction is dependend on the physiologic decrease of the pulmonary resistance. Sophisticated perinatal therapy including mechanical ventilation, inhaled NO, application of prostaglandines and catecholamines for even a prolonged periode of time allows the recovery of right ventricular function. Prenatal counceling and interdisciplinary planning of the perinatal therapy including gynecologists, neonatologists, pediatric cardiologists and cardiac surgeons is necessary to reassure optimal perinatal outcome.

Ebstein - cardiomyopathy - fetal right heart dysfunction - outcome - perinatal management