EDUCATION IN HEART
Congenital heart disease
Ebstein’s anomaly of the tricuspid valve: from fetus to adult
Pediatric Cardiology, Children’s Hospital, Toulouse, France
Correspondence to:
Professor Philippe Acar, Cardiologie Pédiatrique, Hôpital des Enfants, 330 av de Grande-Bretagne, 31000 Toulouse, France; acar.p@chu-toulouse.fr
| The first 150 words of the full text of this article appear below. |
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
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
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