Treatment by pacemaker in familial amyloid polyneuropathy.

Amyloid deposits often involve the heart and cause disturbances in conduction and impulse formation in patients with familial amyloid polyneuropathy (FAP). Seven patients with FAP required pacemaker treatment during eight years. The most frequent bradyarrhythmias requiring pacing were sinus node dysfunction with junctional failure. Our seven patients had attacks and symptoms of bradyarrhythmias. A pacemaker relieved symptoms of bradycardias with recurrence of dislocation of electrode, exit block, and relatively high threshold. However, pacing did not improve the ultimate prognosis of FAP, because of progressive inanition of FAP. In our series, pacing should have been started earlier before advanced stage according to the ECG findings as in other diseases with bradycardias.