[Current aspects of Ebstein's disease].

Ebstein's disease is a rare affection which is more and more frequently discovered since the advent of sonocardiography. As a matter of fact, except in the loud but less frequent neonatal forms, Ebstein's disease appears, most of the time, in the first three decades of life under various, misleading forms. The diagnosis is, today, greatly facilitated by bi-dimensional sonocardiography. This technic enables in fact to precisely define, in typical forms, the different abnormalities of the entire tricuspid valve complex; however, there are also atypical forms which should not be ignored. The prognosis of Ebstein's disease is dominated by rhythm disorders, which should be systematically looked for, and will benefit from modern treatments, and cardiac insufficiency. In this field, recent surgical techniques have also greatly improved so that surgery should be considered as soon as the symptoms become worse (Class III of NYHA).