Biventricular hypertrophy

Serum CK is consistently increased in all forms of AMD. Forearm ischemic exercise causes a normal rise of venous lactate concentration in patients with childhood or adult AMD. The electrocardiogram (ECG) is altered in Pompe s disease, with a short P-R interval, giant QRS complexes and left ventricular or biventricular hypertrophy, but is usually normal in the later-onset forms. The EMG shows myopathic features and fibrillation potentials, bizarre high-frequency discharges and myotonic discharges. 

There is no glycemic response to glucagon or epinephrine (Fig. 42-1), whereas a galactose load causes a normal glycemic response. Forearm ischemic exercise produces a blunted venous lactate rise or no response. Serum CK activity is variably, often markedly, increased. The ECG shows left ventricular or biventricular hypertrophy in most patients, and the EMG may show myopathic features alone or associated with fibrillations, positive sharp waves and myotonic discharges. This mixed EMG pattern in patients with weakness and distal wasting often reinforces... 

Biventricular hypertrophy. Voltage criteria for LVH found in frontal plane with tall R waves in lead VI Athletes heart , physiological LVH... 

It is possible to have enlargement of both ventricles, termed biventricular hypertrophy. This can be difficult to spot as the two may cancel each other out to some degree, and the ECG may appear normal. Biventricular hypertrophy may be present if the voltage criteria for LVH is present in limb leads, with tall R waves in lead Vi (Fig. 5.14). 

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