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Autosomal Dominant Myofibrillar Myopathy with Arrhythmogenic Right Ventricular Cardiomyopathy Linked to Chromosome 10q Ann Neurol 46:684-692,681, Melberg,A.,et al, 1999 See this aricle in Pubmed Article Abstract Twenty-one members of a Swedish family suffering from myopathy and cardiomyopathy underwent neurological and cardiological investigations. Three men had arrhythmogenic right ventricular cardiomyopathy. Nonsustained ventricular tachycardia , atrial flutter, and dilatation of the ventricles mainly affecting the right ventricle were documented. Two of them had a pacemaker implanted because of atrioventricular block and sick sinus syndrome. Inheritance is autosomal dominant with variable ons et and severity of skeletal muscle and cardiac involvement. Linkage analysis of candidate chromosomal regions showed a maximum 2-point LOD score of 2.76 for marker locus D10S1752 on chromosome 10q. A multipoint peak LOD score of 3.06 between markers D10 S605 and D10S215 suggests linkage to chromosome 10q22.3, and this region may harbor a genetic defect for myofibrillar myopathy with arrhythmogenic right ventricular cardiomyopathy.

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(click to filter results - removes previous filter) arrhythmia,cardiac atrioventricular block cardiomyopathy desmin familial heart block muscle biopsy myopathy myopathy,myofibrillar pacemaker,cardiac-transvenous sick sinus syndrome

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