The essence of the Ebstein's malformation is that the tricuspid valve leaflets do
not attach normally to the valve annulus, and the effective orifice is displaced downward
into the right ventricular cavity at the junction of the inlet and trabecular components
of the right ventricle. Only the septal and posterior leaflets are displaced and divide
the right ventricle into two portions. The inlet portion is usually integrated functionally
with the right atrium (“atrialized portion”), while the other, including the trabecular
and outlet portions, constitutes the functional right ventricle. The proximal atrialized
right ventricle often has a wall thinner than the distal functional right ventricle,
due to partial congenital absence of myocardium. An atrial septal defect is present
in more than one-third of hearts, and the majority of the remainder has a patent foramen
ovale resulting in a right-to-left shunt. The downward displacement of the septal
tricuspid valve leaflet is associated with discontinuity of the central fibrous body
and septal atrioventricular ring, thus creating a potential substrate for accessory
atrioventricular connections and ventricular pre-excitation making the patient at
risk of sudden death. Angiography has demonstrated that a significant number of
patients with Ebstein's anomaly also have morphofunctional abnormalities of the left
ventricle, which may be explained by increased fibrosis in the left ventricular wall
and ventricular septum as demonstrated by histological studies. Regarding embryology,
the leaflets and tensile apparatus of the tricuspid valve are believed to be formed
mostly by a process of delamination of the inner layers of the inlet zone of the right
ventricle. The downward displacement of the leaflets in Ebstein's anomaly suggests
that delamination from the inlet portion failed to occur.
Key words:
- Congenital heart disease - Sudden death - Tricuspidvalve dysplasia
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Gaetano Thiene
Istituto di Anatomia Patologica
Via A. Gabelli 61
35121 Padova
Italy
Phone: +++ 39 049 8272283
Fax: +++39 049 8272284
Email: cardiopath@ux1.unipd.it
