Ventricular late potentials in familial amyloidotic polyneuropathy

Area of Science:

• Cardiology
• Electrophysiology
• Genetics

Background:

• Familial amyloidotic polyneuropathy (FAP) is a progressive disease affecting nerves and organs.
• Ventricular arrhythmias are a potential complication in FAP patients.
• Late potentials (LPs) are markers of cardiac electrical instability.

Purpose of the Study:

• To investigate the prevalence of ventricular LPs in FAP patients.
• To assess the association between LPs, ventricular arrhythmia, and echocardiographic findings in FAP.
• To explore potential age-related differences in LP occurrence.

Main Methods:

• Studied 55 FAP patients and 94 healthy controls.
• Performed Holter electrocardiography to detect LPs and ventricular arrhythmia.
• Conducted echocardiography to assess cardiac structure and function.

Main Results:

• LPs were found in 46% of FAP patients >60 years vs. 15% of controls (P=.02).
• LP occurrence correlated with nonsustained ventricular arrhythmia in older FAP patients (P=.04).
• Older FAP patients with LPs showed increased ventricular septum and left posterior wall thickness.

Conclusions:

• Ventricular LPs are prevalent in older FAP patients (>60 years).
• LPs are associated with ventricular arrhythmia and cardiac structural changes in this population.
• Long-term studies are necessary to establish the prognostic value of these findings.