Pacemaker treatment in familial amyloidosis with polyneuropathy

Involvement of the heart is common in familial amyloidosis with polyneuropathy (FAP) and is manifested by disturbances of atrioventricular and intraventricular conduction and dysfunction of the sinus node. Pacemaker implantation was performed in 20 patients with FAP in northern Sweden between 1968 and 1983 for the following indications: complete heart block (11), second degree heart block (1), sinus node dysfunction (5), and atrial fibrillation with a slow ventricular rate (3). There was a prompt improvement of symptoms attributable to a slow heart rate. Dislodgement of the electrode occurred in four patients; no other serious complications were observed. From our experience we conclude that at least one out of ten patients with FAP will eventually require pacemaker treatment. Cardiac pacing is an effective adjunct in the symptomatic treatment of FAP, although the ultimate prognosis depends mainly on the underlying disease.