Cardiac Amyloidosis: An Updated Review With Emphasis on Diagnosis and Future Directions

Bhogal, Sukhdeep; Ladia, Vatsal; Sitwala, Puja; Cook, E R; Bajaj, Kailash; Ramu, Vijay; Lavie, Carl J.; Paul, Timir K.

doi:10.1016/j.cpcardiol.2017.04.003

Cited by 88 publications

(126 citation statements)

References 83 publications

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“…Patients present with dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspnea, abdominal distension, and lower extremity edema. 38 While the heart is a target of several types of amyloid, more than 95% of cases are due to AL and ATTR. 5,37,39,40 It has been estimated that 20% to 35% of those with a lightchain paraprotein develop amyloid, while between 50% and 90% of those with AL amyloid have cardiac involvement, 41,42 making this the most common form found in the heart.…”

Section: Heartmentioning

confidence: 99%

An Organ System–Based Approach to Differential Diagnosis of Amyloid Type in Surgical Pathology

Giannini

Nast

2019

Archives of Pathology &Amp; Laboratory Medicine

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Context.— Amyloidosis is an uncommon but important entity. A protein-based classification of amyloidosis defines the underlying disease process, directing clinical management and providing prognostic information. However, in routine surgical pathology there often is no attempt to classify amyloid other than staining to determine light chain–associated amyloidosis. Systemic and localized amyloidosis vary with respect to frequency of organ involvement by different amyloid types, and most amyloid proteins have commercial antibodies available for identification. Objective.— To provide a guide for the likelihood of amyloid type by organ system. Data Sources.— Literature review based on PubMed searches containing the word amyloid, specifically addressing the prevalence and significance of amyloid proteins in each organ system other than the brain, and the authors' practice experience. Conclusions.— In patients with amyloidosis, determination of the responsible protein is critical for appropriate patient care. In large subspecialty practices and reference laboratories with experience in using and analyzing relevant immunohistochemistry, most amyloid proteins can be identified with an organ-specific algorithm. Referring to an organ-based algorithm may be helpful in providing clinicians with a more specific differential diagnosis regarding amyloid type to help guide clinical evaluation and treatment. When the protein cannot be characterized, mass spectrometry can be performed to definitively classify the amyloid type.

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Section: Heartmentioning

confidence: 99%

An Organ System–Based Approach to Differential Diagnosis of Amyloid Type in Surgical Pathology

Giannini

Nast

2019

Archives of Pathology &Amp; Laboratory Medicine

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“…We found it was easier to control the speed of injection in these patients, because with reduced systolic function, it's less likely to fill LV cavity with high concentration of contrast agents that may cause far field attenuation and interfere the tracking of basal segments. Another possible reason for that is the etiology of these patients, which cause deposition of fibril proteins in the interstitium of myocardium like cardiac amyloidosis 34 . It's characterized by thickening of ventricular wall and valves and classic granular sparking in myocardium.…”

Section: Correlation Between Lvef and Glsmentioning

confidence: 99%

Comparison of Left Ventricular Systolic Function Quantification Using Contrast-Enhanced and Non-Contrast Echocardiographic Images

Liang

Wang

Zhang

et al. 2020

Preprint

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Background: Left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS) are two important index for the quantification of left ventricular systolic function. With the help of ultrasound contrast agents, we can improve the definition of endocardial borders and allow the quantification of LVEF in patients with poor image quality. However, the feasibility of GLS measurements in contrast-enhanced images is still controversial. Our study aimed to explore the feasibility of GLS measured by velocity vector imaging (VVI) in contrast-enhanced images, compare the difference of measurements in contrast-enhanced and non-contrast images, and analyze the relation between LVEF and GLS in both conditions.Methods: A total of 133 patients with cancer, who were registered for transthoracic echocardiography as well as contrast-enhanced echocardiography were studied. LVEF was measured using the biplane modified Simpson’s rule and GLS was measured with offline VVI analysis of the three standard apical views in non-contrast and contrast-enhanced images respectively. Linear regression was performed to derive correlation coefficients between LVEF and GLS both in non-contrast and contrast-enhanced images.Results: GLS measurements in non-contrast images were discarded in 2/133 patients (1.5%), while in contrast-enhanced images were obtained in all patients. LVEF (64.12B7.47% vs. 66.25a8.61%, respectively; P < 0.01) and GLS (-20.99c4.67% vs. -23.40k4.58%, respectively; P < 0.01) were both significantly higher in the presence of contrast agents. A linear regression between LVEF and GLS in non-contrast images (r=0.627, P<0.001) was observed, as well as in contrast-enhanced images (r=0.649，P<0.001).Conclusion: GLS measured by VVI in contrast-enhanced echocardiography is a feasible and reliable index for the quantification of left ventricular systolic function, even in patients with poor image quality. Compared with the measurements in non-contrast images, both LVEF and GLS measurements are higher in the presence of contrast agents.

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“…En algunos casos, se observa el realce parcheado o moteado, que puede ser localizado o transmural (17,18) . Este examen alcanza una sensibilidad de 80 % y una especificidad de 94 % (4) . En este paciente no se pudo realizar este estudio.…”

Section: A B C Discusiónunclassified

“…Para determinar el tipo de amiloide se realizará estudios de inmunohistoquímica. En este paciente la biopsia rectal brindó el diagnóstico (4) .…”

Section: A B C Discusiónunclassified

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Amiloidosis cardiaca: reporte de un caso

et al. 2018

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Cardiac amyloidosis is a rare clinical entity and, therefore, almost unknown. It shows as a restrictive cardiomyopathy, since it is an infiltrative disease caused by the extracellular deposition of amyloids in the cardiac wall. Although it may be systemic, the heart condition determines its poor prognosis. This is the case of a 38-year-old patient who was admitted for dyspnea and severe fatigue. He was diagnosed with IgA multiple myeloma, lambda light chains, clinical stage IIIB, and initiated treatment with cyclophosphamide, thalidomide and dexamethasone. The patient stopped the treatment, and returned after one year and 2 months with an advanced disease and presence of amyloidosis. The patient progressed torpidly and eventually died. A review of the medical literature is performed concerning amyloidosis types, physiopathology, diagnostic methods and treatment.

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Cardiac Amyloidosis: An Updated Review With Emphasis on Diagnosis and Future Directions

Cited by 88 publications

References 83 publications

An Organ System–Based Approach to Differential Diagnosis of Amyloid Type in Surgical Pathology

An Organ System–Based Approach to Differential Diagnosis of Amyloid Type in Surgical Pathology

Comparison of Left Ventricular Systolic Function Quantification Using Contrast-Enhanced and Non-Contrast Echocardiographic Images

Amiloidosis cardiaca: reporte de un caso

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