The ECG of a Cardiomyopathy - 2

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Author(s) A.A.M. Wilde
NHJ edition: 2008:06,221
These Rhythm Puzzles have been published in the Netherlands Heart Journal and are reproduced here under the prevailing creative commons license with permission from the publisher, Bohn Stafleu Van Loghum.
The ECG can be enlarged twice by clicking on the image and it's first enlargement
Figure 1

A 36-year-old male comes to your outpatient clinic because of his family history. His father died suddenly at age 43 and his brother died suddenly at age 39. Both had documented dilated cardiomyopathy. He has no symptoms whatsoever. His ECG is presented in figure 1. Ten years before the current presentation he had an unremarkable cardiological check-up (ECG, echo, X-ECG).

What is the most likely diagnosis, what would be your next step and what would your eventual treatment be?

Answer