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Treatment by pacemaker in familial amyloid polyneuropathy
N Anzai1, K Akiyama, K Tsuchida
1Department of Cardiology, Higashi Nagano National Hospital, Japan.
Chest
|July 1, 1989
Summary
Pacemakers can alleviate bradyarrhythmia symptoms in familial amyloid polyneuropathy (FAP) patients. However, early pacemaker implantation is recommended before advanced FAP stages to manage cardiac conduction issues effectively.
Area of Science:
- Cardiology
- Neurology
- Genetics
Background:
- Familial amyloid polyneuropathy (FAP) can affect the heart, leading to conduction and impulse formation disturbances.
- Amyloid deposits in the heart are a known complication of FAP.
Purpose of the Study:
- To evaluate the role and outcomes of pacemaker treatment in patients with familial amyloid polyneuropathy (FAP).
Main Methods:
- Retrospective analysis of seven FAP patients requiring pacemaker implantation over eight years.
- Monitoring of bradyarrhythmias, pacemaker function, and clinical symptoms.
Main Results:
- Sinus node dysfunction with junctional failure was the most frequent bradyarrhythmia requiring pacing.
- Pacemakers relieved bradycardia symptoms but did not alter the overall prognosis due to progressive FAP.
- Complications included electrode dislocation, exit block, and high pacing thresholds.
Conclusions:
- Pacemaker therapy effectively manages symptomatic bradyarrhythmias in FAP patients.
- Earlier pacemaker implantation, guided by ECG findings, may be beneficial before advanced FAP stages.
- Pacemaker implantation does not improve the ultimate prognosis of FAP, which is limited by progressive inanition.