Echocardiographic diagnosis of Ebstein's disease. Apropos of 10 cases
Drobinski, G.; Lebuisson, A.B.; Evans, J.I.; Thomas, D.; Kelessides, C.; Grosgogeat, Y.
Archives des Maladies du Coeur et des Vaisseaux 73(2): 176-182
1980
ISSN/ISBN: 0003-9683 PMID: 6769407 Document Number: 154661
The M-mode echocardiographic diagnostic criteria of Ebstein's anomaly are: the recording of the tricuspid valve with the probe at a distance from the left sternal border, an interval of over 0.10 s between the Q wave of the ECG and tricuspid valve closure and a delay of 0.06 s or more of tricuspid over mitral valve closure. Ten cases were studied to evaluate the sensitivity of these signs and the limitations of M-mode ECG to assess the utility of cardiac catheterization in this disorder; catheterization was performed in 6 cases in this series. The echocardiographic criteria of Ebstein's anomaly were observed in 8 of 10 cases. The diagnostic difficulties and the discrepancies between the clinical examination and complementary investigations are discussed. The chronological criteria of tricuspid closure exclude ostium secundum atrial septal defect but may be present in Uhl's disaese. In 1 case, the echocardiographic changes did not satisfy the diagnostic criteria and in a 2nd case the echocardiogram was normal. In these 2 patients the ECG and radiological changes were minor and the diagnosis was made at cardiac catheterization by the demonstration of right atrial pressure curves with a right ventricular intracardiac ECG, and an abnormally situated tricuspid valve. In 3 cases, there was an associated Wolff-Parkinson-White syndrome which not affect the echocardiographic appearances of Ebstein's anomaly, the diagnosis of which was missed in 1 case. Cardiac catheterization as a diagnostic procedure is unnecessary when the M-mode echocardiographic appearances are characteristic, whether or not the clinical features are obvious. In intermediate forms, the diagnosis may be difficult and echocardiographic data insufficient. Endocavitary investigation is the only diagnostic method in these cases showing a right ventricular ECG in a cavity with right atrial pressures. These minor forms may be complicated by systemic embolism and echocardiography has a role in showing up change which, even if non-specific, orientate the diagnosis and suggest further investigation by cardiac catheterization. The development of cross-sectional echocardiography should lead to a more precise anatomical definition especially of the abnormal insertion of the tricuspid valve.