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Congenital heart disease
Ebstein’s anomaly of the tricuspid valve: from fetus to adult
  1. Soizic Paranon,
  2. Philippe Acar
  1. Pediatric Cardiology, Children’s Hospital, Toulouse, France
  1. Professor Philippe Acar, Cardiologie Pédiatrique, Hôpital des Enfants, 330 av de Grande-Bretagne, 31000 Toulouse, France; acar.p{at}chu-toulouse.fr

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Ebstein’s anomaly was first described by Wilhelm Ebstein in 1866. At autopsy of a young man with a history of palpitations’ and dyspnoea followed—before his death—by cyanosis, cardiomegaly and systolic murmur, the Berlin based doctor described a tricuspid valve abnormality. This congenital malformation is characterised by any degree of inferior displacement of the proximal attachments of the septal and posterior leaflets of the tricuspid valve from the atrioventricular ring. It occurs in about 1–5 per 200 000 births and represents <1% of all congenital heart disease.1 Clinical presentation, treatments and outcome are varied and individual management is required.

MORPHOLOGY

Tricuspid valve anatomy

The rules of cardiac anatomy state that the valve belongs to the ventricle. The ventricle is divided into three components: the outlet, the apical trabecular and the inlet component which extends from the atrioventricular junction. During systole, the atrioventricular valve has to stay closed and resist the contraction of the ventricle to prevent backflow of blood. The tricuspid valve is attached to the atrioventricular junction of the right ventricle. The three leaflets are distinguished by their position: septal, anterior, and inferior leaflets. These leaflets are suspended from the muscular atrioventricular junction apart from a small area of membranous septum. They are joined by papillary muscles: the medial papillary muscle between the septal and anterior leaflets, the inferior between the septal and posterior leaflets, and the anterior between the posterior and the anterior leaflets.2

The embryologic origin of the tricuspid valve is the delamination from the ventricular myocardium at the atrioventricular junction. In Ebstein’s anomaly, the delamination, primarily of the septal and inferior leaflets, is incomplete. There are no cords to suspend the leaflets, so they arise from the cavity of the ventricle and are attached to its wall. An apical displacement of leaflets has been described, but a recent concept of …

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