Tafamidis in the Treatment of ATTR-related Cardiomyopathy
Transthyretin amyloid cardiomyopathy (ATTR-CM) is caused by the myocardial extracellular deposition of amyloid fibrils formed from the dissociation of TTR tetramer into monomers. The rate-limiting step in TTR amyloidogenesis is the dissociation of the TTR tetramer into monomers: Tafamidis is an effective TTR-stabilizer in its native homotetrameric structure. Tafamidis is a safe and effective drug in reducing symptoms, hospitalization and mortality in accurately selected patients affected by hereditary and wild-type transthyretin amyloid cardiomyopathy. (Source: Heart Failure Clinics)
Source: Heart Failure Clinics - April 12, 2024 Category: Cardiology Authors: Teresa Maria Capovilla, Andrea Lalario, Maddalena Rossi, Aldostefano Porcari, Alberto Aimo, Giuseppe Limongelli, Michele Emdin, Marco Merlo, Gianfranco Sinagra Source Type: research
Genotype-Phenotype Correlations in ATTR Amyloidosis
Hereditary transthyretin-related amyloidosis (hATTR) is the most common form of familial amyloidosis. It is an autosomal dominant disease caused by a pathogenic variant in the TTR gene. More than 140 TTR gene variants have been associated with hATTR, with the Val30Met variant representing the most common worldwide. The clinical phenotype varies according to the gene variant and includes predominantly cardiac, predominantly neurologic, and mixed phenotypes. The present review aims to describe the genotype-phenotype correlations in hATTR. Understanding these correlations is crucial to facilitate the early identification of t...
Source: Heart Failure Clinics - April 10, 2024 Category: Cardiology Authors: Emanuele Monda, Chiara Cirillo, Federica Verrillo, Giuseppe Palmiero, Luigi Falco, Alberto Aimo, Michele Emdin, Marco Merlo, Giuseppe Limongelli Source Type: research
Pathophysiology of Cardiac Amyloidosis
Amyloidosis refers to a heterogeneous group of disorders sharing common pathophysiological mechanisms characterized by the extracellular accumulation of fibrillar deposits consisting of the aggregation of misfolded proteins. Cardiac amyloidosis (CA), usually caused by deposition of misfolded transthyretin or immunoglobulin light chains, is an increasingly recognized cause of heart failure burdened by a poor prognosis. CA manifests with a restrictive cardiomyopathy which progressively leads to biventricular thickening, diastolic and then systolic dysfunction, arrhythmias, and valvular disease. The pathophysiology of CA is m...
Source: Heart Failure Clinics - April 8, 2024 Category: Cardiology Authors: Paolo Morfino, Alberto Aimo, Maria Franzini, Giuseppe Vergaro, Vincenzo Castiglione, Giorgia Panichella, Giuseppe Limongelli, Michele Emdin Source Type: research
The Role of Scintigraphy with Bone Radiotracers in Cardiac Amyloidosis
Cardiac amyloidosis (CA) is caused by the myocardial deposition of misfolded proteins, either amyloid transthyretin (ATTR) or immunoglobulin light chains (AL). The paradigm of this condition has transformed, since CA is increasingly recognized as a relatively prevalent cause of heart failure. Cardiac scintigraphy with bone tracers is the unique noninvasive technique able to confirm CA without performing tissue biopsy or advanced imaging tests. A moderate-to-intense myocardial uptake (Perugini grade ≥2) associated with the absence of a monoclonal component is greater than 99% specific for ATTR-CA, while AL-CA confirmation...
Source: Heart Failure Clinics - April 8, 2024 Category: Cardiology Authors: Paolo Morfino, Alberto Aimo, Assuero Giorgetti, Dario Genovesi, Marco Merlo, Giuseppe Limongelli, Vincenzo Castiglione, Giuseppe Vergaro, Michele Emdin Source Type: research
Standard Therapy in Cardiac Amyloidosis
This study aims to highlight the most compelling issues concerning patients ’ clinical management with HF and CA. (Source: Heart Failure Clinics)
Source: Heart Failure Clinics - April 6, 2024 Category: Cardiology Authors: Annamaria Del Franco, Giulia Biagioni, Carlotta Mazzoni, Alessia Argir ò, Mattia Zampieri, Francesco Cappelli Source Type: research
Clinical Use of Biomarkers in Cardiac Amyloidosis
Amyloidosis is a systemic condition characterized by multiple organs involvement. A multidisciplinary and multimodal approach in assessing patients is pivotal and recommended by the international scientific societies. Biomarkers represent an essential noninvasive tool to increase the suspicion of disease and orient further workup and clinical management of patients. This review provides an updated contemporary focus on the clinical use of biomarkers in cardiac amyloidosis, emphasizing their role in both the diagnostic and prognostic setting and discussing future perspective of emerging biomarkers. (Source: Heart Failure Clinics)
Source: Heart Failure Clinics - April 4, 2024 Category: Cardiology Authors: Andrea Lalario, Riccardo Saro, Gianfranco Sinagra, Marco Merlo, Aldostefano Porcari Source Type: research
Cardiovascular Magnetic Resonance in the Management of Cardiac Amyloidosis
Cardiac magnetic resonance represents the gold standard imaging technique to assess cardiac volumes, wall thickness, mass, and systolic function but also to provide noninvasive myocardial tissue characterization across almost all cardiac diseases. In patients with cardiac amyloidosis, increased wall thickness of all heart chambers, a mildly reduced ejection fraction and occasionally pleural and pericardial effusion are the characteristic morphologic anomalies. The typical pattern after contrast injection is represented by diffuse areas of late gadolinium enhancement, which can be focal and patchy in very early stages, circ...
Source: Heart Failure Clinics - April 4, 2024 Category: Cardiology Authors: Andrea Barison, Ignazio Alessio Gueli, Fausto Pizzino, Chrysanthos Grigoratos, Giancarlo Todiere Source Type: research
Specific Therapy in Transthyretin Amyloid Cardiomyopathy
Transthyretin amyloid cardiomyopathy (ATTR-CM) is a relatively prevalent cause of morbidity and mortality. Over the recent years, development of disease-modifying treatments has enabled stabilization of the circulating transthyretin tetramer and suppression of its hepatic production, resulting in a remarkable improvement in survival of patients with ATTR-CM. Second-generation drugs for silencing are currently under investigation in randomized clinical trials. In vivo gene editing of transthyretin has been achieving unanticipated suppression of hepatic production in ATTR-CM. Trials of antibodies inducing the active remova...
Source: Heart Failure Clinics - April 4, 2024 Category: Cardiology Authors: Riccardo Saro, Valentina Allegro, Marco Merlo, Franca Dore, Gianfranco Sinagra, Aldostefano Porcari Source Type: research
Amyloidosis and Amyloidogenesis
Amyloidosis is a heterogenous group of disorders, caused by the deposition of insoluble fibrils derived from misfolded proteins in the extracellular space of various organs. These proteins have an unstable structure that causes them to misfold, aggregate, and deposit as amyloid fibrils with the pathognomonic histologic property of green birefringence when viewed under cross-polarized light after staining with Congo red. Amyloid fibrils are insoluble and degradation-resistant; resistance to catabolism results in progressive tissue amyloid accumulation. The outcome of this process is organ disfunction independently from the ...
Source: Heart Failure Clinics - April 3, 2024 Category: Cardiology Authors: Marco Pozzan, Carla Indennidate, Guerino Giuseppe Varr à, Gianfranco Sinagra, Marco Merlo, Linda Pagura Source Type: research
Pulmonary Hypertension in Adult Congenital Heart Disease –Related Heart Failure
Already a challenging condition to define, adult congenital heart disease (ACHD) -associated heart failure (HF) often incorporates specific anatomies, including intracardiac and extracardiac shunts, which require rigorous diagnostic characterization and heighten the importance of clinicians proactively considering overall hemodynamic impacts of using specific therapies. The presence of elevated pulmonary vascular resistance dramatically increases the complexity of managing patients with ACHD-HF. Total circulatory management in patients with ACHD-HF requires input from multidisciplinary care teams and thoughtful and careful...
Source: Heart Failure Clinics - March 9, 2024 Category: Cardiology Authors: Jonathan Kusner, Richard A. Krasuski Source Type: research
The Pathophysiology(ies) of Heart Failure in Adults with Congenital Heart Disease
There is a growing, aging population of adults with congenital heart disease (CHD) with an increasing incidence of heart failure. Unquestioning extrapolation of widely applicable definitions of heart failure and guidelines for managing heart failure in adults with acquired heart failure to adults with CHD can be problematic. A nuanced and flexible application of clinical judgment founded on a deep understanding of underlying pathophysiology is needed to most effectively apply the many recent advances in managing acquired heart failure to the care of adults with CHD. (Source: Heart Failure Clinics)
Source: Heart Failure Clinics - March 9, 2024 Category: Cardiology Authors: Alexander R. Opotowsky Source Type: research
The Role of Multimodality Imaging in the Evaluation of Heart Failure and Surgical Transplant Planning of Patients with Adult Congenital Heart Disease
Cardiac imaging is pivotal in evaluating ventricular function, residual lesions, and long-term complications in patients with adult congenital heart disease (ACHD). Longitudinal imaging in ACHD is key for the timely identification of patients requiring evaluation for advanced therapies. The guidelines recommend routine imaging surveillance. In all patients undergoing evaluation with cardiac imaging, it is critical that studies are performed at centers with expertise and that the imaging protocols are tailored to the specific condition. The authors briefly highlight the utility and diagnostic yield of different modalities, ...
Source: Heart Failure Clinics - March 9, 2024 Category: Cardiology Authors: Valeria E. Duarte, Saurabh Rajpal Source Type: research
Adult Congenital Heart Disease
HEART FAILURE CLINICS (Source: Heart Failure Clinics)
Source: Heart Failure Clinics - March 9, 2024 Category: Cardiology Authors: Saurabh Rajpal, Ragavendra R. Baliga Source Type: research
Copyright
ELSEVIER (Source: Heart Failure Clinics)
Source: Heart Failure Clinics - March 9, 2024 Category: Cardiology Source Type: research
Contributors
EDUARDO BOSSONE, MD, PhD, FCCP, FESC, FACC (Source: Heart Failure Clinics)
Source: Heart Failure Clinics - March 9, 2024 Category: Cardiology Source Type: research
