Prevalence and Prognostic Value of Conduction Disturbances at the Time of Diagnosis of Cardiac AL Amyloidosis

Boldrini, Michele; Salinaro, Francesco; Mussinelli, Roberta; Raimondi, Ambra; Alogna, Alessio; Musca, Francesco; Palladini, Giovanni; Merlini, Giampaolo; Perlini, Stefano

doi:10.1111/anec.12032

Cited by 48 publications

(29 citation statements)

References 45 publications

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“…Conventional 12-lead electrocardiograms were also recorded with standard instrument sensitivity of 10 mm ¼ 1 mV. If no QRS deflection was greater than 5 mm in any limb lead, the definition of 'low QRS voltages' by limb lead criteria was satisfied [23][24][25]. Patients with cardiac involvement and clinical signs or symptoms of heart failure whose ejection fraction was above 50% were defined as presenting diastolic heart failure or heart failure with normal (preserved) ejection fraction (HFpEF) [9].…”

Section: Methodsmentioning

confidence: 99%

Prognostic value of depressed midwall systolic function in cardiac light-chain amyloidosis

Perlini¹,

Salinaro²,

Musca³

et al. 2014

Journal of Hypertension

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Section: Methodsmentioning

confidence: 99%

Prognostic value of depressed midwall systolic function in cardiac light-chain amyloidosis

Perlini¹,

Salinaro²,

Musca³

et al. 2014

Journal of Hypertension

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“…16 The presence of intraventricular conduction delays or blocks, particularly as they progress over time, reflect worsening of the disease and involvement of the conduction system and high likelihood of requiring a permanent pacemaker, but also higher mortality in AL subtype. 20 Reduced heart rate variability on a 24 hour Holter predicts high short-term mortality in AL, but limited data in ATTR subtypes on mortality is currently available. 18 Despite its widespread availability and low cost, ECG has limited overall diagnostic accuracy and data on accurately differentiating amyloid subtypes.…”

Section: Prognostic Valuementioning

confidence: 99%

Role of imaging in the diagnosis and management of patients with cardiac amyloidosis: State of the art review and focus on emerging nuclear techniques

AlJaroudi

Desai

Tang

et al. 2014

Journal of Nuclear Cardiology

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Amyloidosis is an infiltrative disease characterized by deposition of amyloid fibrils within the extracellular tissue of one or multiple organs. Involvement of the heart, cardiac amyloidosis, is recognized as a common cause of restrictive cardiomyopathy and heart failure. The two major types of cardiac amyloidosis are cardiac amyloid light-chain (AL) and transthyretin-related cardiac amyloidosis (ATTR, mutant and wild types) (Nat Rev Cardiol 2010;7:398-408). While early recognition of cardiac amyloidosis is of major clinical importance, so is the ability to differentiate between subtypes. Indeed, both prognosis and therapeutic options vary drastically depending on the subtype. While endomyocardial biopsy with immunostaining is considered the gold standard, advances in imaging provide an attractive non-invasive alternative. Currently, electrocardiography, echocardiography, and cardiac magnetic resonance imaging are all used in the evaluation of cardiac amyloidosis with varying diagnostic and prognostic accuracy. Yet, none of these modalities can effectively differentiate the cardiac amyloid subtypes. Recent data with 99m Tc-phosphate derivatives, previously used as bone seeking radioactive tracers, have shown promising results; these radiotracers selectively bind ATTR, but not AL subtype, and can differentiate subtypes with high diagnostic accuracy. This review will initially present the non-radionuclide imaging techniques and then focus on the radionuclide imaging techniques, particularly 99m Tc-DPD and 99m Tc-PYP, mechanism of action, performance and interpretation of the study, diagnostic accuracy, prognostic value, future clinical perspective, and outlook.

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“…However, tafamidis treatment resulted in an increase ≥10% in the LV mass index in 43% of patients [6,7]. In the present patient, IVS thickness increased by 6 mm, myocardial mass increased by 10%, and the QRS interval was prolonged, which together suggested the progression of amyloid deposition in the myocardial wall [9]. Hemodynamic function analysis showed a decrease in peak filling rate and an increase in mean pulmonary artery pressure, which demonstrated decreased diastolic function and increased LV end-diastolic pressure.…”

Section: Discussionmentioning

confidence: 80%

Tafamidis for the Treatment of Hereditary Transthyretin Amyloid Cardiomyopathy: A Case Report

Fujita

Inomata²,

Kaida³

et al. 2017

Cardiology

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Tafamidis meglumine is a novel medicine that has been shown to slow the progression of peripheral neurological impairment in patients with hereditary transthyretin amyloidosis (ATTR). However, the efficacy of tafamidis against ATTR-related cardiac amyloidosis remains unclear. A 72-year-old woman had cardiac hypertrophy and axonopathy in her lower legs. Endomyocardial biopsy revealed an infiltrative cardiomyopathy consistent with amyloidosis. Immunostaining and genetic studies confirmed the diagnosis of ATTR, and tafamidis was started subsequently. Two years after the initiation of tafamidis treatment, electromyography demonstrated no change in the axonopathy in her lower legs; however, electrocardiography displayed QRS prolongation, and echocardiography disclosed an increase in interventricular septal thickness. Endomyocardial biopsy indicated that transthyretin amyloid infiltration of the myocardium was not reduced. In this case, there was no apparent progression of axonopathy, although there were signs of worsening amyloid cardiomyopathy during the treatment with tafamidis.

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Prevalence and Prognostic Value of Conduction Disturbances at the Time of Diagnosis of Cardiac AL Amyloidosis

Abstract: Intraventricular conduction delays have a negative prognostic impact in patients with cardiac AL amyloidosis. Their presence should not be overlooked in the diagnostic workup, prompting a more accurate cardiological support.

Cited by 48 publications

References 45 publications

Prognostic value of depressed midwall systolic function in cardiac light-chain amyloidosis

Prognostic value of depressed midwall systolic function in cardiac light-chain amyloidosis

Role of imaging in the diagnosis and management of patients with cardiac amyloidosis: State of the art review and focus on emerging nuclear techniques

Tafamidis for the Treatment of Hereditary Transthyretin Amyloid Cardiomyopathy: A Case Report

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