298: Ebstein's disease in adulthood

Abstract

Ebstein's disease is a rare congenital malformation with many clinical manifestations depending on the age at diagnosis and the severity of the anatomical pattern. In this study we try to describe the clinical manifestations, the echocardiographic patterns and the outcome of this malformation in adulthood. Retrospective study about 8 cases of Ebstein's anomaly in adult patients (5 women and 3 men); the mean age is 34 years old. The clinical manifestations were absent in 6% of cases but the majority of patients have exertional dyspnea or palpitations in 56% of cases. Arrhythmias and congestive heart failure were observed respectively in 6% and 13% of cases. Transthoracic echocardiography demonstrated the presence of Ebstein anomaly in all cases, showing apical displacement of the septal leaflet of the tricuspid valve. Five patients had the A grade of the Carpentier's classification and the others had the grade B. After an outcome of 11 years, one patient died by refractory heart failure. Clinical manifestations and outcome of Ebstein's anomaly depend on the degree of tricuspid valve malformation and many cases are discovered in adults. The prognosis is worsening by arrhythmia and heart failure. Surgical intervention with tricuspid valve repair should be proposed for patients with severe heart failure and intractable arrhythmia. Conservative surgery and anti arrhythmic therapy had enhanced the prognosis of this congenital malformation.