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Sudden cardiac death represents a common mode of death in patients with light chain cardiac amyloidosis (AL-CA). However, data evaluating implantable cardioverter defibrillator (ICD) in these patients are scarce. We aimed to describe follow-up and incidence of appropriate ICD therapy in AL-CA patients implanted with ICD. This is a retrospective single referral center study including all AL-CA patients implanted with ICD between 2015 and 2024. The primary endpoint was the cumulative incidence of first appropriate ICD therapy with non-arrhythmic death considered as competitive risk. Secondary endpoints included death, heart transplantation and ICD-related complications. A total of 145 patients (mean age 63.4 ± 9.2 years, 64% male) were included. All were implanted in primary prevention. During a median (interquartile range) follow-up of 14.9 (4.2–51.9) months, 22 patients (15.2%) received at least one appropriate ICD therapy (one-year and five-year cumulative incidence [95% CI] respectively 9.4% [5.6–15.9] and 17.1% [11.0–26.4], Fig. 1). Eight primary events (36.4%) occurred before one month following implantation while 10 (45.5%) occurred after one year of follow-up. During the study period, 10 patients (6.9%) underwent heart transplantation and 58 others (40.0%) died (estimated median survival time 62 months). Twelve patients (8.3%) experienced inappropriate shock, mostly due to atrial arrhythmias. Lead dysfunction occurred in 7 (4.8%) patients and infective endocarditis in 2 (1.4%) patients. AL-CA patients with prophylactic ICD experience significant rate of appropriate therapy but maintain high incidence of non-arrhythmic death. Additional ongoing analyzes to identify predictors of ventricular arrhythmias may help to improve candidate selection. Further studies are needed to evaluate impact of ICD on mortality in this patient population. [ABSTRACT FROM AUTHOR]

Background: Early cardiac amyloidosis (CA) diagnosis enables patients to access effective treatments for better long-term outcomes, yet it remains under-recognised, misdiagnosed and inadequately managed., Aim: To reduce diagnostic delays, we aimed to describe the epidemiological and clinical characteristics and changes over an 11-year period., Methods: This was a retrospective, observational cohort study of all patients referred to the Henri-Mondor Hospital for suspected CA., Results: Overall, 3194 patients were identified and 3022 were included and analysed. Our patients came from varied ethnic backgrounds, and more than half (55.2%) had confirmed CA. Over 11 years, referrals increased 4.4-fold, mostly from cardiologists. Notably, wild-type transthyretin amyloidosis (ATTRwt) became the predominant diagnosis, with referrals increasing 15-fold from 20 in 2010-2012 to 308 in 2019-2020. The number of amyloid light chain (AL) diagnoses increased, whilst variant transthyretin amyloidosis (ATTRv) numbers remained relatively stable. Concerning disease severity, AL patients presented more frequently with severe cardiac involvement whereas an increasing number of ATTRwt patients presented with National Amyloid Centre stage I (22.0% in 2013-2014 to 45.9% in 2019-2020). Lastly, among patients diagnosed with ATTRv in 2019-2020, 83.9% had ATTR Val122Ile cardiac phenotype., Conclusions: This study shows that increasing cardiologist awareness and referrals have increased CA diagnoses. With improved awareness and non-invasive diagnostic techniques, more patients with ATTRwt with milder disease and more ATTRv Val122Ile mutations are being referred and diagnosed. Although more AL cases are being recognised, patients are diagnosed with severe cardiac involvement., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Assessment of potential donors is an essential part of heart transplantation. Despite the shortage of donor hearts, donor heart procurement from brain-dead organ donors remains low in France, which may be explained by the increasing proportion of high-risk donors, as well as the mismatch between donor assessment and the transplant team's expectations. Improving donor and donor heart assessment is essential to improve the low utilization rate of available donor hearts without increasing post-transplant recipient mortality. This document provides information to practitioners involved in brain-dead donor management, evaluation and selection, concerning the place of medical history, electrocardiography, cardiac imaging, biomarkers and haemodynamic and arrhythmia assessment in the characterization of potential heart donors., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)