A study of implanted cardiac rhythm recorders in advanced cardiac AL amyloidosis

Sayed, Rabya; Rogers, Dominic; Khan, Fakhar Z.; Wechalekar, Ashutosh; Lachmann, HJ; Fontana, Marianna; Mahmood, Shameem; Sachchithanantham, Sajitha; Patel, Ketna; Hawkins, Philip N.; Whelan, Carol; Gillmore, Julian D.

doi:10.1093/eurheartj/ehu506

Cited by 143 publications

(109 citation statements)

References 25 publications

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“…5 Patients in G1 had increased PR interval (mean of 0.230s ± 0.060), which was significantly different from G2 (p=0.015), G3 (p=0.044) and G4 (p=0.005). Sayed et al 22 also showed an association between disturbances in electrical conduction and decreased longitudinal strain, and a negative prognostic value for this association, although the study was conducted with cardiac AL amyloidosis.…”

Section: Discussionmentioning

confidence: 98%

Speckle Tracking and Transthyretin Amyloid Cardiomyopathy

Rocha¹,

Ferreira²,

Nacif³

et al. 2016

Arquivos Brasileiros De Cardiologia

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BackgroundAmyloidosis is a disease caused by deposits of insoluble fibrils in extracellular spaces. The most common type of familial amyloidosis is mediated by mutation of transthyretin, especially Val30Met. Symptoms and ejection fraction decrease may occur in cardiac amyloidosis only in case of poor prognosis. Myocardial strain detected by two-dimensional speckle tracking echocardiography can indicate changes in myocardial function at early stages of the disease.ObjectiveTo determine the accuracy of left ventricular longitudinal strain by two-dimensional speckle tracking echocardiography in patients with familial amyloidosis caused by Val30Met transthyretin mutation.MethodsEighteen consecutive patients, carriers of transthyretin mutation, were evaluated by two-dimensional speckle tracking echocardiography, by which myocardial strain curves were obtained, following the American Society of Echocardiography recommendations.ResultsPatients were divided into three groups: 1- Val30Met with cardiac amyloidosis; 2-Val30Met with extracardiac amyloidosis; 3 - Val30Met without evidence of disease. As the three groups were compared by the Mann-Whitney test, we found a statistically significant difference between groups 1 and 2 in the mean longitudinal tension (p=0.01), mean basal longitudinal strain (p=0.014); in mean longitudinal tension and mean longitudinal strain between groups 1 and 3 (p=0.005); and in the ratio of longitudinal strain of apical septum segment to longitudinal strain of basal septum (p=0.041) between groups 2 and 3.ConclusionLeft ventricular longitudinal strain detected by two-dimensional speckle tracking echocardiography is able to diagnose left ventricular dysfunction in early stages of familial amyloidosis caused by transthyretin Val30Met mutation.

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Section: Discussionmentioning

confidence: 98%

Speckle Tracking and Transthyretin Amyloid Cardiomyopathy

Rocha¹,

Ferreira²,

Nacif³

et al. 2016

Arquivos Brasileiros De Cardiologia

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“…However, pacemaker insertion failed to salvage patients with bradyarrhythmias in a study of newly diagnosed patients with severe symptomatic cardiac AL amyloidosis [59]. …”

Section: Treatmentmentioning

confidence: 99%

Recent Advances in the Diagnosis, Risk Stratification, and Management of Systemic Light-Chain Amyloidosis

Vaxman

Gertz

2019

Acta Haematol

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The term amyloidosis refers to a group of disorders in which protein fibrils accumulate in certain organs, disrupt their tissue architecture, and impair the function of the effected organ. The clinical manifestations and prognosis vary widely depending on the specific type of the affected protein. Immunoglobulin light-chain (AL) amyloidosis is the most common form of systemic amyloidosis, characterized by deposition of a misfolded monoclonal light-chain that is secreted from a plasma cell clone. Demonstrating amyloid deposits in a tissue biopsy stained with Congo red is mandatory for the diagnosis. Novel agents (proteasome inhibitors, immunomodulatory drugs, monoclonal antibodies, venetoclax) and autologous stem cell transplantation, used for eliminating the underlying plasma cell clone, have improved the outcome for low- and intermediate-risk patients, but the prognosis for high-risk patients is still grave. Randomized studies evaluating antibodies that target the amyloid deposits (PRONTO, VITAL) were recently stopped due to futility and currently there is an intensive search for novel treatment approaches to AL amyloidosis. Early diagnosis is of paramount importance for effective treatment and prognosis, due to the progressive nature of this disease.

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“…46,47 The benefit of ILR-guided therapy in patients at high risk of sudden cardiac death, 2 Data on ILR use in these rare diseases are limited and mainly retrospective [48][49][50] and the safety of an ILR approach for distinguishing benign causes of syncope and palpitations from lifethreatening arrhythmias requiring an ICD or a pacemaker should be evaluated in clinical trials.…”

Section: Ongoing Studies On the Prognostic Value Of Loop Recordersmentioning

confidence: 99%

Holter Monitoring and Loop Recorders: From Research to Clinical Practice

Galli¹,

Ambrosini²,

Lombardi³

2016

Arrhythmia &Amp; Electrophysiology Review

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Since the 1960s, Holter monitoring has been a cornerstone for diagnosing suspected arrhythmias in patients of all ages. 1 The most common monitoring systems allow the continuous registration of three or more leads for 24-48 hours; newer Holter monitors allow continuous electrocardiogram (ECG) registration for 2 weeks.1 Extending the time of ECG registration will increase the diagnostic yield of Holter monitoring, especially for those rhythm disturbances that are infrequent but recurrent.1,2 This need for a prolonged ECG monitoring has been addressed by event recorders, which can monitor patients for up to 3 years, storing the ECG obtained a few minutes before and after the onset of an arrhythmia in its memory and transmitting data to the cardiac unit. 1,2 When interpreting the results of the ECG, the cardiologist has to determine whether symptoms reported by the patient could be linked to significant disturbances in heart rhythm.2 In other circumstances the detection of atrial or ventricular arrhythmias may alert the cardiologist, even if they occur asymptomatically, thus prompting a specific therapeutic decision such as starting antiarrhythmic or anticoagulant drugs or implanting a pacemaker or a cardioverter defibrillator. Cardiac Monitoring SystemsCardiac rhythm monitoring has an established diagnostic and prognostic role in different circumstances: syncope, palpitations and monitoring of patients with known or suspected episodes of atrial fibrillation (AF), e.g. those with stroke of uncertain aetiology (cryptogenic stroke).1-3 ECG monitoring may also play a role in identifying ventricular tachycardia (VT) in patients with recognised risk of sudden cardiac death. 4 As many devices with different characteristics are available, the choice of the most suitable monitoring system largely depends on the likelihood of detecting a significant correlation between symptoms and ECG findings. Holter MonitorsTraditional ambulatory Holter monitors are simple devices that usually have three leads and continuously register the ECG. They can be short-(24-48 hours) or long-duration (1-2 weeks) devices.1 Two-week Holter monitoring is preferred when diagnosing or excluding AF in cryptogenic stroke, but loop recorders and outpatient telemetry have even higher detection rates. Loop Recorders and Post-event RecordersNewer generation monitors are the so-called event recorders.According to their specific functions they can be divided into two categories: loop recorders, which include external loop recorders (ELRs) and implantable loop recorders (ILRs); and post-event recorders (non-looping recorders). 1 AbstractHolter monitors are tools of proven efficacy in diagnosing and monitoring cardiac arrhythmias. Despite the fact their use is widely prescribed by general practitioners, little is known about their evolving role in the management of patients with cryptogenic stroke, paroxysmal atrial fibrillation, unexplained recurrent syncope and risk stratification in implantable cardioverter defibrillator or pacemaker candidates. New Holt...

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A study of implanted cardiac rhythm recorders in advanced cardiac AL amyloidosis

Abstract: The discovery that bradyarrhythmias heralded terminal cardiac decompensation in most patients with severe cardiac AL amyloidosis supports a study of prophylactic pacemaker insertion in this patient population.

Cited by 143 publications

References 25 publications

Speckle Tracking and Transthyretin Amyloid Cardiomyopathy

Speckle Tracking and Transthyretin Amyloid Cardiomyopathy

Recent Advances in the Diagnosis, Risk Stratification, and Management of Systemic Light-Chain Amyloidosis

Holter Monitoring and Loop Recorders: From Research to Clinical Practice

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