Development of High-Grade Ventricular Arrhythmias in Familial Amyloid Polyneuropathy
To determine which subsets of patients with amyloid heart disease are particularly susceptible to the development of clinically significant ventricular arrhythmias, 16 patients with familial amyloid polyneuropathy (FAP) were studied by serial 24-hour electrocardiographic monitorings and echocardiography. During a mean follow-up period of 26.6 months, seven patients (44%) developed repetitive premature ventricular complexes (PVCs), including ventricular couplets in four and ventricular tachycardia in three. One of them showed syncope at the time when the arrhythmia was identified. Although there were no significant differences in the mean age, duration of the illness before the beginning of the study, and the initial echocardiographic results, the mean follow-up periods were more prolonged and neurologic disabilities were more advanced in patients with repetitive PVCs compared with those without any significant arrhythmias. In addition, patients who developed repetitive PVCs showed significantly greater percent changes in ventricular wall thickness with resultant wall hypertrophy than those who did not. One of the three patients who died within one year of the final study experienced sudden cardiac death and had showed frequent episodes of ventricular tachycardia at the final study. We conclude that (1) high-grade ventricular arrhythmias develop frequently in patients with FAP in the far-advanced stage in whom progressive and marked myocardial amyloid infiltration occurs and (2) identification of subsets of patients with repetitive PVCs may be helpful for the treatment as well as management of amyloid heart disease.
KeywordsVentricular Tachycardia Sudden Cardiac Death Cardiac Amyloidosis Posterior Wall Thickness Sick Sinus Syndrome
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