Updates for the diagnosis and management of cardiac amyloidosis.

Abstract

A substantial increase in the interest in transthyretin cardiac amyloidosis (ATTR-CA) is a result of the constantly growing number of patients, the use of clear diagnostic protocols and the availability of the first selective drug for these patients. This has also raised the awareness of the disease among physicians of all specialties. The topic is particularly relevant to cardiologists, who use non-invasive multimodal imaging in their daily practice. The differential diagnosis of the causes of myocardial hypertrophy includes arterial hypertension, hypertrophic cardiomyopathy, aortic stenosis (AS), athletic heart syndrome, Fabry disease, and cardiac amyloidosis (CA). It turns out that in patients with myocardial hypertrophy >15 mm, amyloidosis is the most common cause of left ventricular (LV) hypertrophy. In parallel, CA is one of the most common infiltrative diseases leading to a clinical picture that may mimic heart failure with preserved ejection fraction (HFpEF). The accumulation of amyloid in the extracellular space impairs the diastolic function of the myocardium, which is observed as the restrictive cardiomyopathy phenotype. In advanced cases, the LV systolic function is also impaired. Moreover, protein deposits contribute to the disturbances of calcium metabolism and cell metabolism as well as to cardiotoxicity, leading to edema and damage to cardiomyocytes.

• Authors: Piotr Gościniak, Tomasz Baron, Sławomir Milczarek, Magdalena Kostkiewicz, Bogusław Machaliński
• Journal: Advances in clinical and experimental medicine : official organ Wroclaw Medical University (Adv Clin Exp Med) Vol. 31 Issue 2 Pg. 175-185 (Feb 2022) ISSN: 1899-5276 [Print] Poland
• PMID: 35195962 (Publication Type: Journal Article, Review)

Topics

• Amyloidosis (diagnosis, therapy)
• Cardiomyopathies (diagnosis, therapy)
• Heart Failure (diagnosis)
• Humans
• Hypertrophy, Left Ventricular
• Stroke Volume
• Ventricular Function, Left