[Electrophysiological study of a Portuguese case of amyloidosis with conduction disorders]

Cardiac involvement in the course of familial Portuguese amyloidosis, as apart from the other primary amyloidosis, is characterised by the early and wide-spread intracardiac conduction disorders, and the contrasting late presentation of the clinical signs. A case is presented of Portuguese amyloidosis which was typical from the viewpoint of the neurological disorders, the familial characteristics, and the positive biopsy; the main conduction defects found in primitive amyloidoses are also recalled. The patient described had for many years suffered from first degree heart block, and then presented with lipid changes with syncopal attacks which led to electrophysiological investigation of the conduction defect, no similar example of which has been found in the literature. The severity and widespread nature of the disorders which were found, together with the localisation of a sub-His block led us to implant the pacemaker. We have only found two other patients who had implants for disorders of conduction secondary to cardiac amyloidosis. Emphasis has been laid on the importance of this investigation which, when it leads to the positioning of a pacemaker, should avoid the onset of syncopal attacks and sudden death which together constitute one of the primary causes of mortality in primary amyloidosis. The length of follow-up in our case has been 14 months (April 1977).