Ebsteins anomaly of the tricuspid valve pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Claudia P. Hochberg, M.D. [3];Priyamvada Singh, MBBS [4]

Overview

The pathophysiology of Ebstein's anomaly depends on the morphology of tricuspid valve and the right ventricle. The annulus of the valve is in normal position. The valve leaflets however, are to a varying degree attached to the walls and septum of the right ventricle. There is subsequent atrialization of a portion of the morphologic right ventricle (which is then contiguous with the right atrium). This causes the right atrium to be large and the anatomic right ventricle to be small in size. 50% of cases involve an atrial shunt (either a PFO or an ASD).

Tricuspid valve

• The anterior leaflet of the tricuspid valve is malformed in this condition and it is attached to the tricuspid valve annulus or to the right ventricular endocardium
• The septal and posterior leaflets of the tricuspid valve are absent most of the time
• Due to these changes, the tricuspid valve may become funnel-shaped and incompetent or leaky

Right ventricle

The right ventricle changes secondary to the malformed tricuspid valves. The right ventricle can be divided into two parts by the malformed valve:

• The downward extension of the tricuspid valve causes 'atrialization' of the proximal part of the right ventricle
• The small distal part, the right ventricle proper, thus is reduced in size and sometimes comprises only of the right ventricular outflow tract

Associated Conditions

Commonly associated conditions include:

• 50% of patients have an ASD or patent foramen ovale
• 25% have an accesory pathway

A complete list of associated conditions includes the following:

• Aortic coarctation
• Cleft anterior leaflet of the mitral valve
• Coarctation of the aorta
• Congenital deaf-mutism
• Corrected transposition of the great arteries
• Hypertensive pulmonary vascular disease
• Hypoplastic pulmonary arteries
• Left ventricular outflow obstruction
• Mitral valve prolapse
• Parachute mitral valve
• Partial atrioventricular canal
• Patent ductus arteriosus
• Pulmonary atresia with an intact ventricular septum
• Pulmonary stenosis
• Subaortic stenosis
• Tetralogy of Fallot
• Ventricular septal defect (VSD)