Ebstein anomaly in the adult: focus on pregnancy

Abstract

Ebstein anomaly (EA) is a rare cardiac congenital abnormality characterized by downward displacement of the posterior and septal leaflets of the tricuspid valve which results in atrialization of the right ventricle, enlargement of the right atrium and tricuspid regurgitation. Affected individuals experience a wide spectrum of clinical severity, ranging from heart failure in infants to asymptomatic adults and identification of the disease later in life. Other clinical presentations include cyanosis, arrhythmias and paradoxical emboli through atrial level shunts. Imaging modalities such as echocardiography and cardiac magnetic resonance are used for diagnosis. Appropriate surgical and medical management tailored to each patient's anatomy and hemodynamic status is necessary in order to ensure acceptable patient outcomes. Since most patients survive to childbearing age, understanding of the hemodynamic changes during pregnancy and careful planning of labor and delivery are paramount. The purpose of this review is to focus on Ebstein anomaly in the adult patient tailored for the adult cardiologist and to provide a systematic review of pregnancy outcomes in women with Ebstein anomaly.