The American Association for Thoracic Surgery (AATS) 2025 Expert Consensus Document: Management of Ebstein anomaly in children and adults

Abstract

Objectives: Patients with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic, focusing on preoperative assessment, indications for intervention, and medical management before and after surgical intervention in older children and adults. This is a companion document to a recently issued document regarding management of EA in neonates and infants. This document addresses children older than 1 year of age and adults.

Methods: The EA Writing Group of the Congenital Clinical Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was conducted using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members' votes with at least 75% agreement on each statement.

Results: In patients with EA, the presence of congestive heart failure, cyanosis, significant left ventricular dysfunction, severe cardiomegaly, and persistent arrhythmia increases the risk for morbidity and mortality. Asymptomatic patients with EA should undergo exercise stress testing with measurement of oxygen consumption to unmask occult exercise intolerance. Cardiac magnetic resonance imaging is recommended for comprehensive imaging of EA for reliable volume assessment of both ventricles, evaluation of tricuspid regurgitation fraction, and right ventricle stroke volume. Surgery is recommended for symptoms that include fatigue, decreased objective exercise tolerance, decreased arterial oxygen saturation (cyanosis), and exertional dyspnea. Surgery can be beneficial in asymptomatic patients with severe tricuspid regurgitation, moderate right ventricular enlargement, and valve anatomy favorable for repair. Bidirectional cavopulmonary shunt is reasonable when there is severe right ventricular dilation, severe right ventricular systolic dysfunction, right atrial pressure: left atrial pressure ratio >1.5, or failure to separate from cardiopulmonary bypass after repair. Concomitant maze procedure at the time of surgery is reasonable when there is paroxysmal or continuous atrial fibrillation. In EA patients with evidence of systolic left ventricular dysfunction, concomitant acquired disease should be excluded, and goal-directed medical therapies is recommended.

Conclusions: Management of EA requires a tailored and multidisciplinary approach. The introduction of the cone procedure has revolutionized surgical management, providing excellent outcomes and durability across a wide range of anatomical variations. The timing of intervention, especially in asymptomatic patients, remains a topic of ongoing research and debate. Comprehensive preoperative evaluations, including exercise testing and arrhythmia assessment, are critical for optimizing surgical outcomes. In complex cases, adjunct procedures like bidirectional cavopulmonary shunt or the maze procedure offer additional therapeutic benefits.

Keywords: Ebstein anomaly; cone procedure; tricuspid valve.