Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Vienna, Austria.
Department of Medicine III (Cardiology and Angiology), Medical University of Innsbruck, Innsbruck, Austria.
Wien Klin Wochenschr. 2020 Dec;132(23-24):742-761. doi: 10.1007/s00508-020-01781-z. Epub 2020 Dec 3.
The prevalence and significance of cardiac amyloidosis have been considerably underestimated in the past; however, the number of patients diagnosed with cardiac amyloidosis has increased significantly recently due to growing awareness of the disease, improved diagnostic capabilities and demographic trends. Specific therapies that improve patient prognosis have become available for certain types of cardiac amyloidosis. Thus, the earliest possible referral of patients with suspicion of cardiac amyloidosis to an experienced center is crucial to ensure rapid diagnosis, early initiation of treatment, and structured patient care. This requires intensive collaboration across several disciplines, and between resident physicians and specialized centers. The aim of this consensus statement is to provide guidance for the rapid and efficient diagnosis and treatment of light-chain amyloidosis and transthyretin amyloidosis, which are the most common forms of cardiac amyloidosis.
过去,心脏淀粉样变性的患病率和意义被严重低估;然而,由于对该病的认识不断提高、诊断能力的提高和人口趋势的变化,最近被诊断为心脏淀粉样变性的患者数量显著增加。某些类型的心脏淀粉样变性已经有了可以改善患者预后的特定疗法。因此,对于疑似心脏淀粉样变性的患者,尽早转至有经验的中心至关重要,以确保快速诊断、早期开始治疗和结构化的患者护理。这需要多个学科之间、住院医师和专业中心之间的密切协作。本共识声明的目的是为快速有效地诊断和治疗最常见的心脏淀粉样变性——轻链淀粉样变性和转甲状腺素蛋白淀粉样变性提供指导。