The study investigates the incidence, mechanisms, and predictors of sudden death (SD) in patients with cardiac amyloidosis, a serious heart condition.
It analyzed data from 784 patients with either ATTR or AL cardiac amyloidosis, finding that SD is more common in AL patients compared to those with ATTR.
Key risk factors for SD include previous pacemaker implantation for ATTR patients and the use of beta-blockers and advanced heart failure symptoms (NYHA III-IV) for AL patients.
The study aimed to explore the prevalence of transthyretin amyloidosis variant cardiomyopathy (ATTRv-CM) among relatives at risk, assess the effectiveness of repeated evaluations, and analyze first-line diagnostic methods like ECG and echocardiogram.
Among 159 relatives evaluated, 25% were diagnosed with ATTRv-CM, and half of those diagnosed exhibited heart failure symptoms, showcasing a high negative predictive value for screening based on specific criteria.
The findings emphasize the need for ongoing monitoring, as 13% of patients with ATTRv-CM showed no initial cardiac signs, suggesting regular follow-up testing is crucial for early detection and management.