Bellofatto Ilaria Anna, Nikolaou Panagiota Efstathia, Andreadou Ioanna, Canepa Marco, Carbone Federico, Ghigo Alessandra, Heusch Gerd, Kleinbongard Petra, Maack Christoph, Podesser Bruno K, Stamatelopoulos Kimon, Stellos Konstantinos, Vilahur Gemma, Montecucco Fabrizio, Liberale Luca
Department of Internal Medicine, University of Genoa, 6 Viale Benedetto XV, 16132, Genoa, Italy.
Laboratory of Pharmacology, Faculty of Pharmacy, National and Kapodistrian University of Athens, Panepistimiopolis, Zografou, 15771, Athens, Greece.
Clin Res Cardiol. 2024 Aug 21. doi: 10.1007/s00392-024-02522-2.
The term cardiac amyloidosis (CA) refers to the accumulation of extracellular amyloid deposits in the heart because of different conditions often affecting multiple organs including brain, kidney and liver. Notably, cardiac involvement significantly impacts prognosis of amyloidosis, with cardiac biomarkers playing a pivotal role in prognostic stratification. Therapeutic management poses a challenge due to limited response to conventional heart failure therapies, necessitating targeted approaches aimed at preventing, halting or reversing amyloid deposition. Mechanisms underlying organ damage in CA are multifactorial, involving proteotoxicity, oxidative stress, and mechanical interference. While the role of inflammation in CA remains incompletely understood, emerging evidence suggests its potential contribution to disease progression as well as its utility as a therapeutic target. This review reports on the cardiac involvement in systemic amyloidosis, its prognostic role and how to assess it. Current and emerging therapies will be critically discussed underscoring the need for further efforts aiming at elucidating CA pathophysiology. The emerging evidence suggesting the contribution of inflammation to disease progression and its prognostic role will also be reviewed possibly offering insights into novel therapeutic avenues for CA.
心脏淀粉样变性（CA）一词是指由于不同情况导致细胞外淀粉样沉积物在心脏中积聚，这些情况通常会影响包括脑、肾和肝在内的多个器官。值得注意的是，心脏受累显著影响淀粉样变性的预后，心脏生物标志物在预后分层中起关键作用。由于对传统心力衰竭治疗反应有限，治疗管理面临挑战，因此需要采取针对性方法来预防、阻止或逆转淀粉样沉积。CA中器官损伤的潜在机制是多因素的，涉及蛋白毒性、氧化应激和机械干扰。虽然炎症在CA中的作用仍未完全了解，但新出现的证据表明其对疾病进展有潜在贡献，以及作为治疗靶点的效用。本综述报告了系统性淀粉样变性中的心脏受累情况、其预后作用以及如何进行评估。将对当前和新出现的治疗方法进行批判性讨论，强调需要进一步努力阐明CA的病理生理学。还将综述提示炎症对疾病进展有贡献及其预后作用的新证据，这可能为CA的新治疗途径提供见解。
