Clinical and prognostic utility of cardiovascular magnetic resonance imaging in myeloma patients with suspected cardiac amyloidosis

Bhatti, Sabha; Watts, Evan; Syed, Fahd; Vallurupalli, Srikanth; Pandey, Tarun; Jambekar, Kedar; Mazur, Wojciech; Hakeem, Abdul

doi:10.1093/ehjci/jew101

Cited by 45 publications

(28 citation statements)

References 27 publications

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“…Of the 2947 studies, we identified 27 meeting the inclusion criteria, including data on 3493 patients ( Figure ). Twenty‐two studies evaluated the performance of CMR in diagnosing either cardiac amyloidosis or ATTR amyloidosis, with a further five studies assessing the diagnostic performance of nuclear scintigraphy . Seventeen studies assessing the performance of index tests in the diagnosis of cardiac amyloidosis included patients with a range of cardiac conditions, whereas 9 out of 11 studies diagnosing ATTR amyloidosis included patients with ATTR or AL amyloidosis only.…”

Section: Resultsmentioning

confidence: 99%

“…Among the four studies using nuclear scintigraphy to differentiate ATTR from AL amyloidosis, three studies specified a monoclonal protein screen in the methodology, the complete results of which were available in only one study . Among 11 studies using EMB as the reference test, five obtained tissue samples from the right ventricle and one involved biventricular EMB; the remaining studies did not report on EMB technique. One study reported no complications following EMB in all subjects, whereas others did not comment on the incidence of complications.…”

Section: Resultsmentioning

confidence: 99%

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Diagnostic performance of imaging investigations in detecting and differentiating cardiac amyloidosis: a systematic review and meta‐analysis

et al. 2019

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AimsThe study aims to systematically assess the diagnostic performance of cardiac magnetic resonance (CMR) and nuclear scintigraphy (index tests) for the diagnosis and differentiation of subtypes of cardiac amyloidosis.Methods and resultsMEDLINE and Embase electronic databases were searched for studies evaluating the diagnostic performance of CMR or nuclear scintigraphy in detecting cardiac amyloidosis and subsequently in differentiating transthyretin amyloidosis (ATTR) from immunoglobulin light‐chain (AL) amyloidosis. In this meta‐analysis, histopathological examination of tissue from endomyocardial biopsy (EMB) or extra‐cardiac organs were reference standards. Pooled sensitivity, specificity, positive likelihood ratio, and negative likelihood ratio were calculated, and a random effects meta‐analysis was used to estimate diagnostic odds ratios. Methodological quality was assessed using a validated instrument. Of the 2947 studies identified, 27 met the criteria for inclusion. Sensitivity and specificity of CMR in diagnosing cardiac amyloidosis was 85.7% and 92.0% against EMB reference and 78.9% and 93.9% with any organ histology reference. Corresponding sensitivity and specificity of nuclear scintigraphy was 88.4% and 87.2% against EMB reference and 82.0% and 98.8% with histology from any organ. CMR was unable to reliably differentiate ATTR from AL amyloidosis (sensitivity 28.1–99.0% and specificity 11.0–60.0%). Sensitivity and specificity of nuclear scintigraphy in the differentiation of ATTR from AL amyloidosis ranged from 90.9% to 91.5% and from 88.6% to 97.1%. Pooled negative likelihood ratio and positive likelihood ratio for scintigraphy in this setting were 0.1 and 8, with EMB reference standard. Study quality assessed by QUADAS‐2 was generally poor with evidence of bias.ConclusionsCardiac magnetic resonance is a useful test for diagnosing cardiac amyloidosis but is not reliable in further classifying the disease. Nuclear scintigraphy offers strong diagnostic performance in both the detection of cardiac amyloidosis and differentiating ATTR from AL amyloidosis. Our findings support the use of both imaging modalities in a non‐invasive diagnostic algorithm that also tests for the presence of monoclonal protein.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Diagnostic performance of imaging investigations in detecting and differentiating cardiac amyloidosis: a systematic review and meta‐analysis

et al. 2019

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“…The gold standard for diagnosing cardiac amyloidosis is endomyocardial biopsy, but noninvasive cardiac imaging can replace the need for cardiac biopsy. Cardiac MRI late gadolinium enhancement was shown to be highly sensitive (80–100%) with a negative predictive value of 85–100%, while the specificity and positive predictive values are 80–94 and 81–92%, respectively [17, 18]. Cardiac MRI is unable to reliably differentiate between the various subtypes of cardiac amyloidosis.…”

Section: Diagnosis and Typingmentioning

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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“…In contrast to echocardiography, cardiac magnetic resonance imaging (CMR) is not dependent on good acoustic windows, has higher spatial resolution, and provides tissue characterization. Studies have shown that certain late gadolinium enhancement (LGE) patterns on CMR are highly sensitive and specific for CA and may even precede morphologic increases in LV wall thickness [26]. Gadolinium contrast agents preferentially distribute in the extracellular space.…”

Section: Cardiac Magnetic Resonance Imagingmentioning

confidence: 99%

Cardiac Amyloidosis: Diagnosis and Treatment Strategies

et al. 2017

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Cardiac amyloidosis in the United States is most often due to myocardial infiltration by immunoglobulin protein, such as in AL amyloidosis, or by the protein transthyretin, such as in hereditary and senile amyloidosis. Cardiac amyloidosis often portends a poor prognosis especially in patients with systemic AL amyloidosis. Despite better understanding of the pathophysiology of amyloid, many patients are still diagnosed late in the disease course. This review investigates the current understanding and new research on the diagnosis and treatment strategies in patients with cardiac amyloidosis. Myocardial amyloid infiltration distribution occurs in a variety of patterns. Structural and functional changes on echocardiography can suggest presence of amyloid, but CMR and nuclear imaging provide important complementary information on amyloid burden and the amyloid subtype, respectively. While for AL amyloid, treatment success largely depends on early diagnosis, for ATTR amyloid, new investigational agents that reduce production of transthyretin protein may have significant impact on clinical outcomes. Advancements in the non-invasive diagnostic detection and improvements in early disease recognition will undoubtedly facilitate a larger proportion of patients to receive early therapy when it is most effective.

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Clinical and prognostic utility of cardiovascular magnetic resonance imaging in myeloma patients with suspected cardiac amyloidosis

Abstract: CMR is a clinically useful tool for diagnosis and prognostication in myeloma patients with suspected CA.

Cited by 45 publications

References 27 publications

Diagnostic performance of imaging investigations in detecting and differentiating cardiac amyloidosis: a systematic review and meta‐analysis

Diagnostic performance of imaging investigations in detecting and differentiating cardiac amyloidosis: a systematic review and meta‐analysis

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

Cardiac Amyloidosis: Diagnosis and Treatment Strategies

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