SPC365

Clinical Outcomes in Familial Amyloid Polyneuropathy Patients: The Effect of Pacemaker Implantation

Session: SESSÃO DE POSTERS 06 - AMILOIDOSE CARDÍACA
Speaker: Diana Ribeiro

Congress: CPC 2025
Topic: F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme: 17. Myocardial Disease
Subtheme: 17.2 Myocardial Disease – Epidemiology, Prognosis, Outcome
Session Type: Cartazes
FP Number: ---
Authors: Diana Ribeiro; André Alexandre; David Sá Couto; Mariana Pereira Santos; Pedro Monteiro; Tiago Peixoto; Sara Lopes Fernandes; Bruno Brochado; Hipólito Reis; Severo Torres

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Arial,sans-serif"><span style="color:#000000"><strong><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:black">Introduction</span></span></span></strong><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:black">: Familial amyloid polyneuropathy (FAP) is an autosomal dominant disorder with possible cardiac involvement, including rhythm and conduction disturbances. Pacemakers are frequently needed, but there is still no evidence or specific guidelines regarding indications in this group of patients. In addition to conventional indications, several centers have performed prophylactic implantations in pre-surgical scenarios and/or followed a lower threshold for implantation. The aim of this study was to compare the progression of the disease&#39;s various manifestations between PAF patients with and without pacemaker implantation.</span></span></span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Arial,sans-serif"><span style="color:#000000"><strong><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:black">Methods</span></span></span></strong><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:black">: A retrospective study <span style="background-color:white">of </span>FAP patients followed at a referral center in Portugal was performed using data from clinical, electrocardiographic, and echocardiographic records, with descriptive and comparative statistical methods applied for analysis.</span></span></span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Arial,sans-serif"><span style="color:#000000"><strong><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:black">Results</span></span></span></strong><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:black">: A total of 296 FAP patients were included, 71.6% with pacemakers and 58.1% male. The mean age at pacemaker implantation was 41.5 &plusmn; 14.0 years. Of the pacemaker implants, 69.2% were prophylactic, 23.7% for atrioventricular block, and 7.1% for sinus node dysfunction. Diabetes (11.9% vs 2.8%; p=0.002) and dyslipidemia (25.0% vs 11.8%; p=0.006) were more prevalent in non-pacemaker patients, while hypertension (31.0% vs 23.1%; p=0.162) and smoking (34.5% vs 35.8%; p=0.830) did not differ significantly. Symptom onset was later in non-pacemaker patients (46.9 vs 36.5 years; p&lt;0.001). More patients with pacemakers were in stage 1 of Coutinho&rsquo;s (p&lt;0.001). Polyneuropathy was more common in pacemaker patients (p&lt;0.001), while cardiomyopathy was slightly more frequent in non-pacemaker patients, but the difference was not significant (p=0.880). Conduction abnormalities were observed in 35.1% of patients, with a higher prevalence in pacemaker carriers (38.7% vs. 26.2%, p=0.042). During an 11 &plusmn; 6.5 years follow-up, conduction disturbances (47.1% vs 30.1%; p=0.008) and polyneuropathy (94.7% vs 74.7%; p&lt;0.001) were more frequently observed in pacemaker patients, and no significant differences in cardiomyopathy were observed (24.5% vs 14.6%; p=0.066). Mortality was higher in non-pacemaker patients, though not significantly (40.5% vs 36.8%; p=0.556).</span></span></span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Arial,sans-serif"><span style="color:#000000"><strong><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:black">Conclusions</span></span></span></strong><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:black">: While pacemaker patients had more conduction disturbances and polyneuropathy, cardiomyopathy progression was similar between groups. Mortality was higher in non-pacemaker patients, but not significantly, suggesting pacemaker implantation aids symptom management but other factors affect survival.</span></span></span></span></span></span></p>