Echocardiogram in Ebstein's anomaly with Wolff-Parkinson-White preexcitation syndrome, type B.

SUMMARY The echocardiographic features of Ebstein's anomaly include a large anterior chamber, para-doxic ventricular septal motion (type B), increased velocity and amplitude of anterior tricuspid leaflet (ATL) motion, and delayed closure of the ATL. This delayed closure has been thought to be secondary to right bundle-branch block (RBBB). We have studied two patients by simultaneous echocardiography and phonocardiography. The findings in both patients were similar. One patient had RBBB and therefore resembles the previously reported patients. However, the other patient had W-P-W preexcitation syndrome, type B, and is reported here in more detail. The ATL closure of this patient was markedly delayed in spite of right ventricular preexcitation. This finding gives further insight into the altered pathophysiology in Ebstein's anomaly and implies that the delayed ATL closure is probably not due to RBBB and may be a specific feature of this anomaly. Additional Indexing Words: Ultrasound Tricuspid valve Anomalous pulmonary venous connection Tricuspid regurgitation Atrioventricular canal Phonocardiogram IN EBSTEIN'S ANOMALY, abnormal tricuspid valve and ventricular septal motion with a large anterior chamber have been described.' Simultaneous echocardiographic and phonocardiographic recordings have led to some pathophysiologic correlations.2 Because of its associated accelerated A-V conduction, the present case suggests new correlations for these observed abnormalities, confirms what would appear to be characteristic findings in Ebstein's anomaly, and suggests that delayed closure of the ATL is not due to RBBB but rather is a specific abnormality found in this syndrome. Case Report The patient was a 20-year-old white woman who was referred to the Mayo Clinic for cardiac evaluation. A dfagnosis of Ebstein's anomaly had been made at age 10 years at another institution. She had remained asymptomatic until 3 years ago, when she began to have frequent episodes of tachycardia for which digitalis therapy was started. Three months prior to her present examination she noted increased fatiguability, Atrial septal defect Interventricular septum cyanosis of digits, swelling of ankles, and loss of appetite.