Abstract

MORA, Guillermo; LAVERDE, Ligia P  and  PALACIO, Ana C. Progressive familial atrio-ventricular block type I: clinical description of a family. Rev. Colomb. Cardiol. [online]. 2011, vol.18, n.5, pp.279-281. ISSN 0120-5633.

We present the case of a family with atrio-ventricular block that involves several generations, with dominant autosomal mendelian inheritance that progressively affects the cardiac conduction system leading to syncope and sudden death in early life. This entity corresponds to progressive familial atrio-ventricular block type I, described mainly in South Africa . Treatment with permanent pacemaker therapy was successful.

Keywords : familial heart block; progressive cardiac conduction defect; hereditary bundle branch block.

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