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Amyloidosis is a systemic disease caused by low molecular weight protein accumulation in the extracellular space, which can lead to different degrees of damage, depending of the organ or tissue involved. The condition is defined cardiac amyloidosis (CA) when heart is affected, and it is associated with an unfavorable outcome. Different types of CA have been recognized, the most common (98%) are those associated with deposition of light chain (AL-CA), and the form secondary to transthyretin deposit. The latter can be classified into two types, a wild type (transthyretin amyloidosis wild type (ATTRwt)-CA), which mainly affects older adults, and the hereditary or variant type (ATTRh-CA or ATTRv-CA), which instead affects more often young people and is associated with genetic alterations. The atrial involvement can be isolated or linked to CA with a nonspecific clinical presentation represented by new onset atrial fibrillation (AF), diastolic dysfunction and heart failure with preserved ejection fraction, or thromboembolism and stroke. Untreated patients have a median survival rate of 9 years for AL-CA and 7 years for ATTR-CA. By contrast, AL-CA and ATTR-CA treated patients have a median survival rate of 24 and 10 years, respectively. Atrial involvement in CA is a common but poor studied event, and alterations of performance can anticipate the anatomical damage. Recently, numerous advances have been made in the diagnostic field with improvements in the available techniques. An early diagnosis therefore allows a more effective therapeutic strategy with a positive impact on prognosis and mortality rate. A multimodality approach to the diagnosis of atrial involvement from CA is therefore recommended, and standard echocardiography, advanced Doppler-echocardiography (DE) and cardiac magnetic resonance (CMR) can be useful to detect early signs of CA and to estabilish an appropriate treatment. [ABSTRACT FROM AUTHOR]

Amyloidosis is a systemic disease characterized by the accumulation of insoluble aggregates in various organs, leading to parenchymal damage. When these amyloid fibrils are deposited in the extracellular matrix of the cardiac structures, the condition is referred to as cardiac amyloidosis (CA). The extent of organ involvement determines the degree of cardiac impairment, which can significantly impact prognosis. The two most implicated proteins in CA are transthyretin and misfolded monoclonal immunoglobulin light chains. These proteins give rise to two distinct clinical forms of CA: transthyretin amyloidosis (ATTR-CA) and light-chain amyloidosis (AL-CA). ATTR-CA is further classified into two subtypes: ATTRm-CA, which occurs at a younger age and is caused by hereditary misfolded mutated proteins, and ATTRwt-CA, which is an acquired wild-type form more commonly observed in older adults, referred to as senile amyloidosis. While AL-CA was considered the most prevalent form for many years, recent autopsy studies have revealed an increase in cases of ATTRwt-CA. This narrative review aims to describe the clinical and imaging features of CA, with a particular focus on cardiac complications and mortality associated with the AL form. Early identification and differentiation of CA from other disorders are crucial, given the higher risk and severity of cardiac involvement in AL-CA. Furthermore, emphasis is placed on the potential utility of cardiovascular magnetic resonance imaging in detecting early cases of CA. [ABSTRACT FROM AUTHOR]

Sarcomeric hypertrophic cardiomyopathy (HCM) is a prevalent genetic disorder characterised by left ventricular hypertrophy, myocardial disarray, and an increased risk of heart failure and sudden cardiac death. Despite advances in understanding its pathophysiology, treatment options for HCM remain limited. This narrative review aims to provide a comprehensive overview of current clinical practice and explore emerging therapeutic strategies for sarcomeric HCM, with a focus on cardiac myosin inhibitors. We first discuss the conventional management of HCM, including lifestyle modifications, pharmacological therapies, and invasive interventions, emphasizing their limitations and challenges. Next, we highlight recent advances in molecular genetics and their potential applications in refining HCM diagnosis, risk stratification, and treatment. We delve into emerging therapies, such as gene editing, RNA-based therapies, targeted small molecules, and cardiac myosin modulators like mavacamten and aficamten, which hold promise in modulating the underlying molecular mechanisms of HCM. Mavacamten and aficamten, selective modulators of cardiac myosin, have demonstrated encouraging results in clinical trials by reducing left ventricular outflow tract obstruction and improving symptoms in patients with obstructive HCM. We discuss their mechanisms of action, clinical trial outcomes, and potential implications for the future of HCM management. Furthermore, we examine the role of precision medicine in HCM management, exploring how individualised treatment strategies, including exercise prescription as part of the management plan, may optimise patient outcomes. Finally, we underscore the importance of multidisciplinary care and patient-centred approaches to address the complex needs of HCM patients. This review also aims to encourage further research and collaboration in the field of HCM, promoting the development of novel and more effective therapeutic strategies, such as cardiac myosin modulators, to hopefully improve the quality of life and outcome of patients with sarcomeric HCM. [ABSTRACT FROM AUTHOR]

Migraine and sarcoidosis are two distinct medical conditions that may have some common biological and clinical pathways. Sarcoidosis is a chronic granulomatous disease characterized by the formation of granulomas in various organs, including the lungs, skin, cardiovascular system, lymph nodes, and brain. Migraine is a common comorbidity in sarcoidosis patients and a common neurological disorder characterized by recurrent headaches that can be accompanied by other symptoms, such as nausea, vomiting, and sensitivity to light and sound. There have been several reports of individuals with neurosarcoidosis experiencing migraines, though the exact relationship between the two disorders is not well understood. Both conditions have been associated with inflammation and the activation of the immune system. In sarcoidosis, the formation of granulomas is thought to be an immune response to the presence of an unknown antigen. Similarly, the pain and other symptoms associated with migraines are thought to be caused by inflammation in the brain and the surrounding blood vessels. There is also evidence to suggest an interplay of environmental and genetic factors playing a role in both conditions, but evidence is inconsistent with the hypothesis of shared genetic susceptibility. This review aims to illustrate common clinical and biological pathways between migraine and sarcoidosis, including inflammation and dysregulation of the immune system, with a focus on the cumulative burden of concurrent disorders and therapeutic implications. [ABSTRACT FROM AUTHOR]

Introduction: The introduction of Cardiac Computed Tomography (CCT) has changed the paradigm in the field of diagnostic cardiovascular medicine. CCT is the primary tool in the assessment of suspected Coronary Artery Disease (CAD) and should be followed by functional assessment when needed to stratify disease and to plan potential interventional or surgical therapy.Areas Covered: We provided the current state of the knowledge on the main aspects of technique of examination, image interpretation and clinical indications. We have focused our attention on the basic routine applications and activities.Expert Opinion: The primary role of CCT in suspected CAD will progressively become the standard approach. In general, any situation in which anatomy of the heart and thoracic vessels/structures is mandatory must be approached using CT first, whenever possible. The quantity and quality of information that can be provided by CCT is big and the operators should learn how to deal with this information. On the other hand, CCT is only apparently a straightforward and simple examination. It is actually the most complex diagnostic procedure that can be performed on CT and requires highly skilled operators and state-of-art-technology. [ABSTRACT FROM AUTHOR]

The respiratory system may be involved in all systemic vasculitides, although with a variable frequency. The aim of our review is to describe radiographic and high-resolution CT (HRCT) findings of pulmonary vasculitides and to correlate radiological findings with pathological results. Lung disease is a common feature of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitides, including granulomatosis with polyangiitis (Wegener's), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and microscopic polyangiitis. Pulmonary involvement is less frequent in immune-complex-mediated small-vessel vasculitides, such as Behçet's disease and Goodpasture's syndrome. Pulmonary involvement associated to large-vessel (gigantocellular arteritis and Takayasu's disease) or medium-vessel (nodose polyarteritis and Kawasaki's disease) vasculitides is extremely rare. The present review describes the main clinical and radiological features of pulmonary vasculitides with major purpose to correlate HRCT findings (solitary or multiple nodules, cavitary lesions, micronodules with centrilobular or peribronchial distribution, airspace consolidations, "crazy paving", tracheobronchial involvement, interstitial disease) with pathological results paying particular attention to the description of acute life-threatening manifestations. A thorough medical history, careful clinical examination and the knowledge of radiological patterns are mandatory for a correct and early diagnosis. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a chameleon disease of unknown etiology, characterized by the growth of non-necrotizing and non-caseating granulomas and manifesting with clinical pictures that vary on the basis of the organs that are mainly affected. Lungs and intrathoracic lymph nodes are the sites that are most often involved, but virtually no organ is spared from this disease. Histopathology is distinctive but not pathognomonic, since the findings can be found also in other granulomatous disorders. The knowledge of these findings is important because it could be helpful to differentiate sarcoidosis from the other granulomatous-related diseases. This review aims at illustrating the main clinical and histopathological findings that could help clinicians in their routine clinical practice. [ABSTRACT FROM AUTHOR]

Cardiac sarcoidosis (CS) is an unusual, but potentially harmful, manifestation of systemic sarcoidosis (SA), a chronic disease characterized by organ involvement from noncaseating and nonnecrotizing granulomas. Lungs and intrathoracic lymph nodes are usually the sites that are most frequently affected, but no organ is spared and CS can affect a variable portion of SA patients, up to 25% from post-mortem studies. The cardiovascular involvement is usually associated with a bad prognosis and is responsible for the major cause of death and complications, particularly in African American patients. Furthermore, the diagnosis is often complicated by the occurrence of non-specific clinical manifestations, which can mimic the effect of more common heart disorders, and imaging and biopsies are the most valid approach to avoid misdiagnosis. This narrative review summarizes the main clinical features of CS and imaging findings, particularly of CMR and 18-Fluorodeoxyglucose Positron Emission Tomography (18F-FDG PET) that can give the best cost/benefit ratio in terms of the diagnostic approach. Imaging can be very useful in replacing the endomyocardial biopsy in selected cases, to avoid unnecessary, and potentially dangerous, invasive maneuvers. [ABSTRACT FROM AUTHOR]

The recent COVID-19 pandemic has dramatically changed the world in the last months, leading to a serious global emergency related to a novel coronavirus infection that affects both sexes of all ages ubiquitously. Advanced age, cardiovascular comorbidity, and viral load have been hypothesized as some of the risk factors for severity, but their role in patients affected with other diseases, in particular immune disorders, such as sarcoidosis, and the specific interaction between these two diseases remains unclear. The two conditions might share similar imaging findings but have distinctive features that are here described. The recent development of complex imaging softwares, called deep learning techniques, opens new scenarios for the diagnosis and management. [ABSTRACT FROM AUTHOR]