The genetic connection

A young Caucasian male with long standing severe debilitating muscle weakness and chronic respiratory failure (ventilator dependant) was transferred to the Intensive care unit for management of ventilator associated pneumonia. A cardiology evaluation was sought in view of clinical and radiographic signs of heart failure. Family history was positive for a similar problem in one of his siblings. The patient’s ECG revealed the following characteristic findings (fig. 1). What is the probable diagnosis? Discussion