Abstract
Results: a mean of 1284-53 points for each patient were acquired. Lower unipolar and bipolar vokages were found in MD 1 pts compared to controls in the interatrial septum (anterior: unipolar 1.774-1.25 mV vs 3.084-1.83 mV, p<0.0001; bipolar 1.094-0.96 mV vs 2.634-2.18 mV, p<0.0001; medium: unipolar 2.254-1.74 mV vs 3.204-1.43 mV, p=0.009; bipolar 1.554-1.08 mV vs 2.364-1.47 mV, p=0.001; posterior: unipolar 1.844-1.08 mV vs 2.374-0.70 mV, p=0.01; bipolar 1.154-0.79 mV vs 1.734-0.79 mV, p=0.0002) and anterolateral atrial wall (unipolar: 2.174-1.38 mV vs 2.954-1.69 mV, p<0.0001; bipolar: 1.934-1.51 mV vs 2.244-1.90 mV, p=0.03). Bipolar potentials duration was significantly prolonged in all regions of RA in MD 1 pts compared to controls. Atrial propagation time of the electrical impulse was longer in MD1 pts than controls (1314-25 ms vs 1004-19 ms, p=0.046). A significant relationship was documented between number of CTG triplets and the percentage of low vokages in the interatrial septum (r=0.6, p= 0.02) Conclusions: cardiac involvement in MD1 includes structural heart abnormalities. These resuks demonstrate the presence of widespread functional changes in the myocardium not necessarily limited to the conduction system, suggesting the development of a cardiac myopathy as part of the disease.
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