Molecular Mechanisms of Cardiac Amyloidosis

Saito, Yukihiro; Nakamura, Kazufumi; Ito, Hiroshi

doi:10.3390/ijms23010025

Cited by 33 publications

(25 citation statements)

References 164 publications

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“…Impaired myocardial efficiency suggests that the coupling of myocardial oxidative metabolism to contractile work deteriorates, possibly due to structural amyloid damage to the heart, as well as cardiac cell toxicity in the case of AL amyloidosis ( 13 , 14 ). Direct toxic effects of ATTR amyloid on cardiomyocytes have also been reported, such as oxidative stress, altered calcium cycling, and apoptotic pathways ( 15 ).…”

Section: Central Hemodynamic Adaptation To Exercise In Cardiac Amyloi...mentioning

confidence: 99%

Diagnostic and Prognostic Values of Cardiopulmonary Exercise Testing in Cardiac Amyloidosis

Banydeen

Monfort

Inamo

et al. 2022

Front. Cardiovasc. Med.

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Cardiac amyloidosis (CA) is a myocardial disease characterized by extracellular amyloid infiltration throughout the heart, resulting in increased myocardial stiffness, and restrictive heart wall chamber behavior. Its diagnosis among patients hospitalized for cardiovascular diseases is becoming increasingly frequent, suggesting improved disease awareness, and higher diagnostic capacities. One predominant functional manifestation of patients with CA is exercise intolerance, objectified by reduced peak oxygen uptake (VO2 peak), and assessed by metabolic cart during cardiopulmonary exercise testing (CPET). Hemodynamic adaptation to exercise in patients with CA is characterized by low myocardial contractile reserve and impaired myocardial efficiency. Rapid shallow breathing and hyperventilation, in the absence of ventilatory limitation, are also typically observed in response to exercise. Ventilatory inefficiency is further suggested by an increased VE-VCO2 slope, which has been attributed to excessive sympathoexcitation and a high physiological dead space (VD/VT) ratio during exercise. Growing evidence now suggests that, in addition to well-established biomarker risk models, a reduced VO2 peak is potentially a strong and independent predictive factor of adverse patient outcomes, both for monoclonal immunoglobulin light chain (AL) or transthyretin (ATTR) CA. Besides generating prognostic information, CPET can be used for the evaluation of the impact of therapeutic interventions in patients with CA.

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Section: Central Hemodynamic Adaptation To Exercise In Cardiac Amyloi...mentioning

confidence: 99%

Diagnostic and Prognostic Values of Cardiopulmonary Exercise Testing in Cardiac Amyloidosis

Banydeen

Monfort

Inamo

et al. 2022

Front. Cardiovasc. Med.

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“…3 Although many organ systems are typically involved, cardiac involvement is the leading cause of mortality and morbidity in 50-70% of cases. 6 Diagnosis is achieved by detecting free light chains or monoclonal immunoglobulins in blood and urine. Treatment is through specifically directed chemotherapy.…”

Section: Introductionmentioning

confidence: 99%

A review of recent advances in the diagnosis of cardiac amyloidosis, treatment of its cardiac complications, and disease-modifying therapies

Raval

Siddiq²,

Sharma³

et al. 2023

F1000Res

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Cardiac amyloidosis (CA), a significant condition resulting in infiltrative cardiomyopathy and heart failure with preserved ejection fraction (HFpEF), is caused by extracellular deposition of amyloid fibrils in the heart. Even though this has been known for an extended period, its prevalence in elderly patients with heart failure is increasingly being recognized. Recent advances in diagnosis with non-invasive methods like technetium pyrophosphate-labeled cardiac scintigraphy (i.e., Tc-PYP scan) and treatment options with tafamidis have played a pivotal role in awareness of the burden of this disease. Management of cardiac complications like heart failure, atrial arrhythmias, conduction block, ventricular arrhythmias, coronary artery disease, and aortic stenosis is now more critical than ever. We aim to review and outline the recent advances in diagnoses of CA. We also review management strategies for cardiac complications of CA with a brief summary of disease-modifying therapies.

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“…ATTRv, Genetic/mutant transthyretin amyloidosis; ATTRwt, wild-type transthyretin amyloidosis. 14,18,20,23 …”

Section: Introductionmentioning

confidence: 99%

Recent Progress in the Development and Clinical Application of New Drugs for Transthyretin Cardiac Amyloidosis

Zhou,

Li,

Geng

et al. 2023

Journal of Cardiovascular Pharmacology

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Transthyretin cardiac amyloidosis is a rare disease that has gained significant attention in recent years due to misfolding of transthyretine fibrils produced by the liver, leading to their deposition in the myocardium. The disease has an insidious onset, nonspecific clinical manifestations, and historically lacked effective drugs, making early diagnosis and treatment challenging. The survival time of patients largely depends on the extent of heart involvement at the time of diagnosis, and conventional treatments for cardiovascular disease do not provide significant benefits. Effective management of the disease requires treatment of its underlying cause. Orthotopic liver transplantation and combined hepato-heart transplantation have been clinically effective means of treating transthyretine cardiac amyloidosis mutants for many years. However, transplantation has many limitations in clinical practice. In recent years, the development of new drugs has brought new hope to patients. This review presents the latest advances in drug development and clinical application, to provide a reference for clinicians managing transthyretin cardiac amyloidosis.

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Molecular Mechanisms of Cardiac Amyloidosis

Cited by 33 publications

References 164 publications

Diagnostic and Prognostic Values of Cardiopulmonary Exercise Testing in Cardiac Amyloidosis

Diagnostic and Prognostic Values of Cardiopulmonary Exercise Testing in Cardiac Amyloidosis

A review of recent advances in the diagnosis of cardiac amyloidosis, treatment of its cardiac complications, and disease-modifying therapies

Recent Progress in the Development and Clinical Application of New Drugs for Transthyretin Cardiac Amyloidosis

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