Narrative review of Ebstein’s anomaly beyond childhood: Imaging, surgery, and future perspectives

Abstract

Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets adhere to the endocardium due to failure of delamination. This leads to apical displacement of their hinge points with a shift of the functional tricuspid valve annulus towards the right ventricular outflow tract with a possibly restrictive orifice. Frequently, a coaptation gap yields tricuspid valve regurgitation and over time the "atrialized" portion of the right ventricle may dilate. The highly variable anatomy determines the clinical presentation ranging from asymptomatic to very severe with need for early operation. Echocardiography and magnetic resonance imaging are the most important diagnostic modalities to assess the tricuspid valve as well as ventricular morphology and function.While medical management of asymptomatic patients can be effective for many years, surgical intervention is indicated before development of significant right ventricular dilatation or dysfunction. Onset of symptoms and arrhythmias are further indications for surgery. Modified cone reconstruction of the tricuspid valve is the state-of-the-art approach yielding the best results for most patients. Alternative procedures for select cases include tricuspid valve replacement and bidirectional cavopulmonary shunt depending on patient age and other individual characteristics. Long-term survival after surgery is favorable but rehospitalization and reoperation remain significant issues. Further studies are warranted to identify the optimal surgical strategy and timing before adverse right ventricular remodeling occurs. It is this article's objective to provide a comprehensive review of current literature and an overview on the management of Ebstein's Anomaly. It focuses on imaging, cardiac surgery, and outcome. Additionally, a brief insight into arrhythmias and their management is given. The "future perspectives" summarize open questions and fields of future research.

Clinical features and echocardiographic findings in children with ebstein’s anomaly: A descriptive case-series of 48 cases.

“…The literature reports that cyanosis, dyspnea and poor exercise tolerance to be key symptoms among patients with EA. 25 Cyanosis is reported in almost half of EA patients while studies have reported that severe cyanosis is further related to poor exercise intolerance. 26,27 Chest pain and syncope are the other commonly reported symptoms by other researchers in the past.…”

Tactics of managing children-patients with post-coagulopathy cardiac surgery interventions

“…In some cases, it can even save a child's life if left untreated. After surgery, children typically need to stay in the hospital for several days for monitoring and recovery before they can go home [29].…”