What is it about?
Ebstein's anomaly usually presents with severe valvular dysfunction in adults. In the present manuscript, we have shown an atypical variant of Ebstein anomaly, in which the overgrowth of the septal leaflet of the tricuspid valve caused preservation of normal valvular coaptation. This latter finding was demonstrated with 3D echocardiography.
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Why is it important?
This finding suggests that some variants of Ebstein's anomaly may present with preserved valve coaptation, so the valve has a normal or near-normal function. However, knowledge on the prognosis of this patient subset is severely limited and prospective studies are needed to ascertain whether this variants stay normally functioning during lifetime or progress to overt valvular dysfunction in time.
Perspectives
This article might raise interest on Ebstein's anomaly in general ppulation, since neither the morphological variants of the Ebstein's anomaly nor the prognosis of this condition were well-studied in adults. Also, we have observed that three-dimensional echocardiography could be clinically useful for a "real-time, surgical" view of the tricuspid valve, which may facilitate understanding the valve morphology in Ebstein's anomaly.
Dr Tolga Sinan Güvenç
Read the Original
This page is a summary of: A “congenitally corrected” variant of Ebstein’s anomaly, Cardiology in the Young, January 2018, Cambridge University Press,
DOI: 10.1017/s1047951117002712.
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