Valore prognostico della presenza di disturbi della conduzione cardiaca nei pazienti affetti da amiloidosi AL

Prognostic value of cardiac conduction delays in light-chain (AL) amyloidosis Among the different types cardiac amyloidoses, light-chain (AL) amyloidosis has the worst prognostic outcome. The mechanism of death is usually sudden cardiac death, due to tachyarrythmias, bradyarrythmias and/or electromechanical dissociation. To evaluate the possible association between these arrhythmic events and conduction delays detectable on a standard 12 lead ECG, prevalence and prognostic impact of conduction disturbances were assessed in 337 consecutive AL patients at the time of diagnosis; median follow up was 402 days. The prevalence of conduction delays was significantly higher in patients with cardiac AL amyloidosis than in patients without cardiac involvement (41% vs 24% respectively, p<0.05). Analysis of different morphologic, diastolic and systolic parameters (interventricular septum thickness, Doppler E/E’ ratio and mitral annulus longitudinal excursion or MAPSE) showed a worse echocardiographic presentation in cardiac AL patients with conduction delays. Furthermore, prognosis was markedly worse in patients with cardiac AL amyloidosis and intraventricular conduction delays.