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Congenital Anomalies of the Cardiac Valves

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Pathology of Cardiac Valve Disease
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Abstract

Congenital malformations are seen in all cardiac valves, both at atrioventricular and ventricular arterial connections.

At the mitral level, the most frequent forms are: the parachute, the arcade (known as the congenital mitral stenosis), the cleft of the anterior leaflet, the double orifice, and the supravalvular-valvular ring. Rare forms are mucoid dysplasia of all cardiac valves and short/absent chordae tendineae.

A peculiar malformation is the Ebstein anomaly of the tricuspid valve, which consists of a downward displacement of the septal and posterior leaflets with incompetence and auricularization of the right ventricle, with thin wall.

A right-to-left shunt across a patent foramen ovale may occur, accounting for cyanosis. An anomalous septal av connection may be associated, accounting for ventricular preexcitation.

As far as the aortic and pulmonary semilunar valves, malformations mostly consist of anomalous number of the semilunar cusps (unicusp, bicuspid, quadricuspid).

Bicuspid aortic valve may be associated with aortopathy of the ascending (both sinusal and tubular) aorta, with degenerative changes of the tunica media, at risk of progressive dilatating and dissecting aneurysm with rupture and sudden death.

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Frescura, C., Thiene, G. (2023). Congenital Anomalies of the Cardiac Valves. In: Thiene, G., Basso, C., Rizzo, S., Della Barbera, M., Valente, M., Bortolotti, U. (eds) Pathology of Cardiac Valve Disease. Springer, Cham. https://doi.org/10.1007/978-3-031-35498-4_5

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