High Prevalence of Intracardiac Thrombi in Cardiac Amyloidosis

Martinez‐Naharro, Ana; González-López, Esther; Ćorović, Andrej; Mirelis, Jesus G; Baksi, A. John; Moon, James; García‐Pavía, Pablo; Gillmore, Julian D.; Hawkins, Philip N.; Fontana, Marianna

doi:10.1016/j.jacc.2019.01.035

Cited by 81 publications

(81 citation statements)

References 5 publications

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“…Atrial fibrillation alongside a controlled ventricular response is often present in these patients owing to the underlying conduction disease; when present, atrial fibrillations becomes persistent in most patients with ATTRwt 28 . Patients with cardiac amyloidosis have increased risk of intracardiac thrombus, which might even occur in sinus rhythm 29,30 . Some patients will also present with stroke or systemic embolization, usually because of unrecognized atrial fibrillation.…”

Section: [H1] Clinical Features and Biomarkersmentioning

confidence: 99%

Diagnostic imaging of cardiac amyloidosis

et al. 2020

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Systemic amyloidosis encompasses an underdiagnosed, debilitating, progressive but increasingly recognized group of disorders characterized by the extracellular deposition of misfolded proteins in one or more organs. Cardiac amyloid deposition leads to an infiltrative or restrictive cardiomyopathy and is the major driver of prognosis in systemic amyloidosis. In total, >30 proteins can form amyloid fibrils, but the two main types of amyloid that infiltrate the heart are monoclonal immunoglobulin light chain amyloidosis or transthyretin amyloidosis. Cardiac amyloidosis can be acquired in older individuals or inherited at a younger age. Given the nonspecific symptoms of these disorders, a high index of suspicion is paramount to make the correct diagnosis, using non-invasive imaging methods such as echocardiography, bone scintigraphy and cardiac magnetic resonance (CMR). In the past 10 years, the use of CMR with tissue characterization and bone scintigraphy to diagnose cardiac amyloidosis has revolutionized our understanding of the disease, leading to changes in patient care. However, there remains a need for better awareness, expertise and greater clinical suspicion, since the initial clues provided by electrocardiography and echocardiography might not be typical. With specific treatments now available, timely diagnosis of cardiac amyloidosis is more important than ever. In this Review, we discuss the current and novel approaches for the diagnosis and treatment of cardiac amyloidosis.

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Section: [H1] Clinical Features and Biomarkersmentioning

confidence: 99%

Diagnostic imaging of cardiac amyloidosis

et al. 2020

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“…However, patients with cardiac amyloidosis present more procedure-related complications [43]. Moreover, due to the higher frequency of left atrial thrombus, which was present in 28% of patients with cardiac amyloidosis despite anticoagulation, a transesophageal echocardiogram is recommended before AF cardioversion [44]. Nevertheless, anticoagulation is recommended in all patients with AF and cardiac amyloidosis, and the CHADS-VASC does not apply to that decision.…”

Section: Atrial Arrhythmiasmentioning

confidence: 99%

Advances in the Treatment of Cardiac Amyloidosis

Macedo

Schwartzmann

Gusmão

et al. 2020

Curr. Treat. Options in Oncol.

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This article is part of the Topical collection on Cardio-oncology Keywords Amyloidosis I Restrictive cardiomyopathy I Transthyretin I Light chain I Amyloid I Heart failure Opinion statement * The Author(s) 2020 available. Therapies that stabilize transthyretin, such as tafamidis, have been shown to improve outcomes for patients with ATTR-CM. Modern treatments that stop the synthesis of TTR through gene silencing, such as patisiran and inotersen, have shown positive results for patients with TTR amyloidosis. Significant progress has been made in the treatment of amyloid cardiomyopathy, and hopefully, we will see even more progress with the spread of those treatments. We now can be optimistic about patients with this disease.

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“…Echocardiographic features of CA include thickened and at times sparkling appearance of the interatrial septum and valves as well as the classical 'speckled' myocardium. Extracellular amyloid infiltration leads to stiffening, impaired relaxation, and dysfunction of the ventricles, which in combination with atrial infiltration by amyloid, can lead to atrial dilatation, reduced blood flow, and a higher risk of thrombus formation [24][25][26][27]. Despite CA being categorised as a cause of HFpEF, there is typically both systolic and diastolic dysfunction [28].…”

Section: Echocardiographymentioning

confidence: 99%

A review of the criteria for non-invasive diagnosis of cardiac transthyretin amyloidosis

Rezk

Fontana

Gillmore

2021

Expert Opinion on Orphan Drugs

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Introduction: Cardiac transthyretin (ATTR) amyloidosis is a progressive and fatal infiltrative cardiomyopathy (ATTR-CM) characterized by congestive cardiac failure, often with preserved left ventricular ejection fraction, and significant risk of conduction disease. Diagnosis is often delayed or missed due to poor specificity of echocardiography and the historical requirement for a histological diagnosis, frequently an endomyocardial biopsy. Areas covered: Following a detailed literature review focusing on peer reviewed articles (Pubmed, Cochrane Library, Google Scholar), from 1995 to 2020, alongside international diagnostic guidelines and expert opinion in the field, this article will explore the current non-invasive diagnostic criteria for ATTR-CM including the role of transthoracic echocardiography, cardiac MRI, bone scintigraphy, and assessment for exclusion of a clonal dyscrasia. Expert opinion: ATTR-CM is an emerging and increasingly diagnosed cause of heart failure, particularly in the elderly. Promising novel therapies make accurate and swift diagnosis of the disease vital. With the increasing use of cardiac MRI to investigate cardiomyopathy and repurposing of technetium-labeledbone scintigraphy, clinicians are now often able to diagnose ATTR-CM without recourse to an endomyocardial biopsy.

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High Prevalence of Intracardiac Thrombi in Cardiac Amyloidosis

Cited by 81 publications

References 5 publications

Diagnostic imaging of cardiac amyloidosis

Diagnostic imaging of cardiac amyloidosis

Advances in the Treatment of Cardiac Amyloidosis

A review of the criteria for non-invasive diagnosis of cardiac transthyretin amyloidosis

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