Key result
Contemporary management including cone reconstruction and Starnes procedure improves prognosis in Ebstein's anomaly.
Advancements in diagnostic and surgical techniques, such as cone reconstruction and the Starnes procedure, have significantly improved the prognosis for patients with Ebstein's anomaly.
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May inform individualized imaging and surgical planning across presentations; leaves open comparative long-term data on cone versus other repairs.
Ebstein’s anomaly is an uncommon congenital defect of the tricuspid valve, marked by the apical displacement of the septal and posterior leaflets, leading to the “atrialization” of the right ventricle and subsequent dysfunction on the right side. Clinical manifestations vary from asymptomatic adults to neonates exhibiting severe cyanosis and heart failure. Advancements in diagnostic imaging, therapeutic interventions, and surgical techniques have significantly enhanced the prognosis for individuals afflicted by this historically serious condition. This review provides an in-depth examination of Ebstein’s anomaly, discussing its embryological origins, underlying pathophysiology, clinical manifestations, diagnostic assessment, medical treatment protocols, and surgical interventions. Particular emphasis is placed on contemporary surgical options, including cone reconstruction, the Starnes procedure, one-and-a-half ventricle repair, and valve replacement.
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Levchuck et al. (2026) conducted a review in Ebstein's anomaly. Contemporary medical and surgical management strategies was evaluated. Contemporary medical and surgical management strategies, including cone reconstruction and the Starnes procedure, have significantly enhanced the prognosis for individuals with Ebstein's anomaly.
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