Francisco, A.R. Gaspar LopesDias, N. CortezMenezes, M. N.Guimarães, T.Silva, G. Lima daPlácido, R.Magalhães, A.Inácio, C.Pinto, Fausto J.Coutinho, C. Azevedo2018-11-052018-11-052016European Heart Journal, Volume 37, Issue suppl_1, 1 August 2016, Pages 599–9830195-668Xhttp://hdl.handle.net/10451/35268© 2016 European Society of Cardiology. All rights reserved.Background: Rhythm disturbances and conduction defects are common consequences of cardiac amyloid infiltration in familial amyloid polyneuropathy (FAP) V30M-TTR. The occurrence of dysrhythmias appears to increase with the severity of the disease but its prognostic value is still unknown. Purpose: To evaluate the occurrence of arrhythmias according to the patient's age and the duration of symptoms and to analyze its prognostic value. Methods: Prospective observational study of consecutive patients with FAP V30M-TTR who underwent annual cardiac evaluation including Holter recording. Results: During a median follow-up of 55 months, 223 patients were evaluated (44±14 years; 54.3% female) and a total of 777 Holter recordings were performed. It was found that with increasing age, the number of premature supraventricular (Pearson R=0.246, P<0.001, Spearman's Rho=0.246, P<0.001) and ventricular (Pearson R=0.244, P<0.001, Spearman's Rho=0.221, P<0.001) contractions increased, while the mean heart rate decreased (Pearson R=-0.448, P<0.001, Spearman Rho=-0.43, P<0.001). On the other hand, with increasing in symptoms duration, the number of premature ventricular contractions increased significantly (Pearson R=0.144, P=0.001, Spearman's Rho=0.165, P<0.001) (but not supraventricular) and the mean heart rate decreased (Pearson R=-0.153, P=0.001, Spearman's Rho=-0.185, P<0.001). Multivariate Cox regression analysis showed that the independent predictors of mortality were age (HR: 1.073, 95% CI 1.055–1.090), the presence of brady (HR: 1.615, 95% CI 1.071–2.436, P<0.001) or tachyarrhythmias (HR: 1.255, 95% CI 1.255–3.462, P<0.001) and the number of premature supraventricular contractions (HR: 1.439, 95% CI 1.168–1.774, P<0.001). Conclusions: Holter monitoring should be integrated in the periodic evaluation of FAP V30M-TTR patients. The presence of brady or tachyarrhythmias and the number of premature supraventricular contractions are associated with unfavorable prognosis in these patients and should be treated timely.engjournal article10.1093/eurheartj/ehw433