Abstract Cardiac amyloidosis (CA) can lead to progressive heart failure (HF) by depositing insoluble amyloid fibrils within the myocardial extracellular space, resulting in an infiltrative and restrictive cardiomyopathy. Although CA was previously perceived as rare and incurable, recent advances in diagnostics and emerging therapies have been changing this outlook. It is crucial to spread awareness about CA to facilitate earlier diagnosis and proper therapeutic interventions, enhancing patient prognosis and survival. Currently, there is an estimated delay of 2 years from […]

Abstract This article provides a critical review of the diagnostic value of several echocardiographic findings in cardiac amyloidosis (CA). The importance of early and accurate detection of CA is emphasized, considering its challenging diagnosis and the need for a high index of suspicion by clinicians. Echocardiography is often the first choice for imaging assessment of cardiac structure and function when CA is suspected. The article encompasses several conventional echocardiographic features and speckle-tracking echocardiography-derived deformation parameters. Some of these indexes are […]

Introduction Cardiac amyloidosis (CA) manifests as a gradually progressive infiltrative condition characterized by the accumulation of insoluble protein aggregates within the myocardium and cardiac conduction system. The majority of CA cases are associated with precursor proteins, particularly immunoglobulin light chains (AL-CA) and transthyretin (ATTR-CA), which represent over 95% of cases. ATTR precursor substances may originate from a broad spectrum, encompassing both wild-type (ATTRwt) and variant (ATTRv) genes. Arrhythmias in CA result from a combination of factors. The deposition of amyloid […]

Abstract Cardiac amyloidosis (CA) is a progressive condition marked by the accumulation of amyloid fibrils in cardiac tissue, resulting in cardiac dysfunction and, ultimately, heart failure. Pyrophosphate scintigraphy has emerged as a promising tool for the early detection and monitoring of CA, providing valuable insights into the disease’s extent and severity. This review examines the current use of pyrophosphate scintigraphy in distinguishing between different types of amyloidosis, assessing risk levels, and monitoring patients with CA. It underscores the clinical significance […]

Abstract Transthyretin amyloidosis (TTRA) is a systemic infiltrative disease where cardiac involvement is paramount for prognosis. The strain technique plays a crucial role in non-invasive cardiac screening for this condition. Research on the prognostic utility of cardiac deformation in TTRA indicates the significance of right ventricular (RV) free wall strain, global longitudinal strain (GLS), left and right atrium reservoir strain, in descending order of publication, respectively. The GLS has emerged as a promising tool for therapeutic monitoring. Analyzing myocardial deformation […]

Abstract Cardiomyopathy is characterized by structural and functional abnormalities in the heart muscle that are not influenced by coronary disease, hypertension, valve disease, or congenital heart defects. Genetic testing, which helps diagnose the molecular bases of cardiomyopathy, can affect prognostication and clinical decisions. Recent guidelines advocate classifying cardiomyopathies based on phenotypic categories, which encompass a range of clinical presentations, underlying causes, and outcomes. Beyond deepening our grasp of the disease, genetic testing can distinguish between diseases with similar presentations, known […]

Abstract Amyloidosis results from deposition of insoluble fibrillar protein compounds in various organs, including the heart. Cardiac involvement occurs in both atria and ventricles, leading to restrictive cardiomyopathy, heart failure (HF), and arrhythmias. Five amyloid proteins affect cardiac tissue: light and heavy chain forms of immunoglobulin, transthyretin (TTR), apolipoprotein 1, and amyloid A. Approximately 95% of cardiac involvement stems from light chain of immunoglobulin and TTR deposition in heart. Chamber stiffness and rhythm disturbances occur in cardiac amyloidosis (CA), encompassing […]

Abstract Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy that affects the heart mostly in two forms: immunoglobulin light chain amyloidosis (AL-CM) and transthyretin amyloidosis. Transthyretin amyloid cardiomyopathy (ATTR-CM) predominantly affects the elderly population, mainly men (about 70-80%), and usually manifests with symptoms of heart failure (HF). Despite technological advances in medicine, CA is still an underdiagnosed condition, especially in women, partly due to the known male predominance and high level of suspicion required for diagnosis. It is important to remember […]

Abstract Background: In transthyretin amyloid cardiomyopathy (ATTR-CM), the assessment of functional capacity is of great importance. A distance < 300m covered in the 6-minute walk test (6MWT) is linked to a poorer prognosis in heart failure (HF) patients. However, in ATTR-CM, this association is not well-established, particularly among its various presentations and variants. Objectives: To evaluate functional capacity and, the prognostic value of the 6MWT in patients with ATTR-CM and to identify their clinical characteristics according to the type of […]

Abstract Amyloidosis is caused by the extracellular deposition of insoluble protein arrangements, formed from structural changes in different precursor proteins. The prominent clinical and epidemiological relevance of transthyretin amyloid cardiomyopathy (ATTR-CM), whose increased incidence is associated with the aging of the global population, given its high prevalence in the elderly population, has motivated the intensification of the search for specific therapeutic targets capable of altering the natural course of the disease. The past few years have been especially prolific with […]

Abstract Background: Aortic Stenosis (AS) is one of the most common valvular heart diseases in adults, especially the elderly. The persistence of heart failure symptoms and worse outcomes despite valve replacement treatment may be associated with the coexistence of Cardiac Amyloidosis (CA). Objective: To determine the prevalence of CA in patients with calcific AS. Methods: This was a cross-sectional study. Elderly patients with moderate to severe AS underwent cardiac scintigraphy with bone radiotracer for diagnosis of CA. Patients diagnosed with […]