Ebstein’s Anomaly
Report:
Sinus rhythm 152/min
Right axis deviation +140o
Incomplete right bundle branch block
Probable right ventricular hypertrophy
QRV1, RAD
Comment:
This is one of the ECG forms of Ebstein’s anomaly; most of them have a relatively small, somewhat atypical RBBB in V1 and extremely variable P waves. The latter are quite unremarkable in this trace. Not so when the infant was four days old (Fig 41a below): there was a typical P congenitale, largest in L1, and 2mm tall one in V2. Also, the qR RBBB pattern was more obvious.
His tricuspid valve was 1.4 cm displaced from the annulus, with only mild incompetence; there was also an ASD with bidirectional shunt.
At the other end of age spectrum, Fig 41b shows an 89 year old, in junctional rhythm, probably the oldest Ebstein I will ever see. He had a huge, pulsatile liver to delight medical students. His RBBB is even more typical of Ebstein’s, with lower voltage than in the preceding example.
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