[Value of echocardiography in the diagnosis of Uhl's disease. Apropos of a case in an adult]. / Intérêt de l'échocardiographie dans le diagnostic de la maladie de Uhl. A propos d'une observation chex l'adulte.

ABSTRACT

Uhl's anomaly, or paper thin right ventricle is a rare congenital malformation which may present with variable degrees of hypoplasia. The most severe forms are encountered in infants and children, but a less serious form occurs in adolescents and adults which, clinically. There are a number of echocardiographic features common to both diseases but paper-thin right ventricle may be distinguished by the finding of premature opening of the pulmonary value. The increased atrial contraction in these patients with right ventricles of reduced compliance is directly transmitted to the pulmonary artery, leading to end diastolic opening of the pulmonary value before tricuspid closure. The case presented underlines the value of M mode echocardiography in the diagnosis of Uhl's anomaly and in the differential diagnosis with Ebstein's anomaly.