Displacement of all three leaflets of tricuspid valve: A rare variant of Ebstein anomaly

Abstract

Ebstein anomaly, a rare congenital heart disease, is defined as displacement of hinge points of septal or posterior tricuspid leaflets but not anterior leaflet. Here, we report the case of a 35‐year‐old woman with an extremely rare pattern of Ebstein anomaly (EA) with all three tricuspid leaflets displaced downward to the apex.

Uncommon manifestation of Ebstein anomaly: A case report of apical displacement involving all tricuspid valve leaflets

“…Existing literature has reported instances of isolated anterior TV leaflet apical displacement 12–14 . However, our investigation reveals that only one other documented case involves apical displacement alongside other leaflets 8 …”

“…Furthermore, the junctional hinge of the anterior leaflet is infrequently affected. 7,8 The anterior leaflet may exhibit variations, presenting as either large and sail-like or tethered by fibrous tissue, resulting in reduced mobility. 9 Mobility is intricately linked to chordal attachment, with very mobile anterior leaflets often lacking appropriate attachment, while tethered leaflets may directly insert into a papillary muscle or the myocardium.…”

“…Consequently, Ebstein's anomaly rarely affects the anterior tricuspid leaflet, and the attachment point of the septal and inferior leaflets seldom experiences apical displacement beyond the junction between the ventricular inlet and the apicotrabecular component of the RV. Furthermore, the junctional hinge of the anterior leaflet is infrequently affected 7,8 . The anterior leaflet may exhibit variations, presenting as either large and sail‐like or tethered by fibrous tissue, resulting in reduced mobility 9 .…”

“…[12][13][14] However, our investigation reveals that only one other documented case involves apical displacement alongside other leaflets. 8 Given the diverse spectrum of manifestations, ranging from asymptomatic to symptomatic necessitating drug or surgical intervention, a nuanced understanding of the disease's myriad expressions and the associated anatomical and congenital changes in the heart is imperative. This comprehension facilitates informed decisions regarding drug treatments and aids in mitigating complications.…”