Diagnostic score for the detection of cardiac amyloidosis in patients with left ventricular hypertrophy and impact on prognosis

Cariou, Eve; Smires, Youssef Bennani; Victor, G.; Robin, Guillaume; Ribes, David; Pascal, P.; Petermann, Antoine; Fournier, Pauline; Faguer, Stanislas; Roncalli, Jérôme; Rousseau, Hervé; Chauveau, Dominique; Carrié, Didier; Berry, Isabelle; Galinier, Michel; Lairez, Olivier

doi:10.1080/13506129.2017.1333956

Cited by 29 publications

(27 citation statements)

References 31 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%

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%

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 aim is identifying pre-symptomatic patients with initial, possibly reversible, organ involvement. Thus, diagnosis scores have been proposed 40,41 integrating simple clinical, biological, electrocardiographic, and echocardiographic features to optimize delay in CA diagnosis thanks to simple and accessible parameters.…”

Section: Specialist Care Networkmentioning

confidence: 99%

Gateway and journey of patients with cardiac amyloidosis

et al. 2020

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Aims Advances have been made over the last decade in the management of cardiac amyloidosis (CA), but a delayed diagnosis is still common. The aim of this study was to describe the journey to CA diagnosis from initial clinical and to analyse time to diagnosis. Methods and results Between January 2001 and May 2019, 270 consecutive patients with CA diagnosed at Toulouse University Hospital were retrospectively included in this cross-sectional study: 111 (41%) light chain amyloidosis, 122 (45%) wild-type transthyretin amyloidosis, and 37 (14%) hereditary transthyretin amyloidosis. CA onset occurred mostly with dyspnoea (50%) or systematic follow-up (10%). The cardiologist was the first line specialist in 68% of patients, followed by the nephrologist (9%) and neurologist (8%). Patients encountered a median (minimum-maximum) number of two (1-7) physician specialists and performed a median (minimum-maximum) number of three (1-8) tests before diagnosis. Median delay between symptom onset and CA diagnosis was 8 [IQR 5-14], 10 [IQR 3-34], and 18 [IQR 4-49] months, respectively, in light chain amyloidosis, wild-type transthyretin amyloidosis, and hereditary transthyretin amyloidosis subgroups (P = .060). Having performed electromyography or spirometry was associated with a longer delay in diagnosis in the overall population: odds ratio = 1.13; 95% confidence interval 1.02 to 1.24; and odds ratio = 1.13; 1.03 to 1.24, respectively, probably due to non-specific initial symptoms. Conclusions CA is a protean disease with various first line specialists causing a diagnostic wandering despite increasing medical community awareness. It requires a multidisciplinary specialist care networks to educate and manage symptoms and therapies.

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“… 10 Echocardiography in combination with cardiovascular magnetic resonance (CMR) imaging can be used to diagnose and prognosticate CA. 11 12 Additionally, technetium 99m-pyrophosphate (Tc99m-PYP) is a single-photon emission CT (SPECT) agent that is highly sensitive and specific for the diagnosis of ATTR amyloidosis. 13 In this review, we discuss echocardiographic, cardiac MRI and nuclear cardiology.…”

Section: Introductionmentioning

confidence: 99%

Role of cardiovascular imaging for the diagnosis and prognosis of cardiac amyloidosis

et al. 2018

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Cardiac amyloidosis (CA) describes the pathological process of amyloid protein deposition in the extracellular space of the myocardium. Unfortunately, the diagnosis of CA is often made late and when the disease process is advanced. However, advances in cardiovascular imaging have allowed for better prognostication and establishing diagnostic pathways with high sensitivity and specificity. This review discusses the role of echocardiography, cardiac MRI and nuclear cardiology in current clinical practice for diagnosis and prognosis of CA.

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Diagnostic score for the detection of cardiac amyloidosis in patients with left ventricular hypertrophy and impact on prognosis

Abstract: An integrated evaluation of 6 diagnostic factors including arterial blood pressure, ECG and echocardiographic parameters to build a diagnostic score is a simple and easily method to discriminate the 3 main CA in patients with LVH.

Cited by 29 publications

References 31 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

Gateway and journey of patients with cardiac amyloidosis

Role of cardiovascular imaging for the diagnosis and prognosis of cardiac amyloidosis

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