Affiliation:
1. Department of Cardiovascular Surgery, Division of Congenital Cardiac Surgery, Rajaie Cardiovascular Medical and Research Institute, Iran University of Medical Science Tehran Iran
2. Echocardiography Research Center, Rajaie Cardiovascular Medical and Research Center Iran University of Medical Sciences Tehran Iran
3. Student Research Committee, Faculty of Medicine Hormozgan University of Medical Sciences Bandar Abbas Iran
Abstract
Key Clinical MessageThis case report presents an uncommon variant of Ebstein anomaly, where all three tricuspid valve leaflets exhibited apical displacement—a rare finding. It illustrates the complexities in diagnosing and managing such atypical presentations, with successful surgical correction through annuloplasty. The report adds valuable insights to the limited literature on this congenital heart disease.AbstractEbstein anomaly (EA), a rare congenital heart disorder, presents with diverse clinical spectrums. This case report explores a distinctive manifestation of EA, where all three tricuspid valve (TV) leaflets exhibited apical displacement, highlighting a novel aspect in the presentation of this condition. A 44‐year‐old woman, under long‐term medical surveillance for EA, showcased an atypical clinical trajectory marked by the apical displacement of all TV leaflets, which is uncommon in EA. Despite a predominantly asymptomatic course, recent exacerbation of symptoms prompted further evaluation. Diagnostic modalities, including echocardiography and cardiac magnetic resonance imaging, revealed severe tricuspid regurgitation concomitant with unprecedented apical displacement of the anterior, septal, and posterior tricuspid leaflets. The displacement of the anterior leaflet was contrary to typical embryonic valvular formation expectations, indicating a unique presentation within EA. The patient underwent annuloplasty surgery, which successfully rectified the anomalous TV architecture. Postoperative evaluation demonstrated mild residual tricuspid regurgitation, and the patient was discharged in stable condition. This case underscores the variability in EA presentations and accentuates the significance of tailored surgical interventions. The observation of apical displacement involving all TV leaflets adds a unique dimension to the existing EA literature, reinforcing the need for careful diagnosis and personalized treatment approaches.