How to Image Cardiac Amyloidosis

Dorbala, Sharmila; Cuddy, Sarah; Falk, Rodney H.

doi:10.1016/j.jcmg.2019.07.015

Cited by 200 publications

(173 citation statements)

References 70 publications

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“…Furthermore, cardiac imaging modalities are not free of limitations. The hallmark of CA on echocardiogram, such as increased left ventricular thickness, atrial enlargement, and preserved or reduced LV systolic function, can be present in other conditions with increased afterload, such as aortic stenosis, hypertrophic cardiomyopathy or hypertensive heart disease [ 8 ]. Likewise, the severe impairment of diastolic function, characterized by a restrictive pattern of LV filling (high E wave and low A-wave velocity, increased E/A ratio, and reduced deceleration time on diastolic mitral valve inflow), and high filling pressures on tissue Doppler imaging (mitral and tricuspid annular e′ velocities markedly reduced, with a high E/e′ ratio) is not specific for CA.…”

Section: Discussionmentioning

confidence: 99%

“…In amyloidosis, the abnormal myocardium signal can be measured using T1-/T2 mapping, LGE, and extracellular volume (ECV) imaging. Native T1 and ECV are markedly increased in CA [ 8 ]. Furthermore, global subendocardial, diffuse or transmural LV LGE and difficult nulling of the myocardium are specific features of CA, although they do not allow to differentiate between TTR and AL.…”

Section: Discussionmentioning

confidence: 99%

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Negative bone scintigraphy in wild-type transthyretin cardiac amyloidosis

Martini

Rizzo

Sarais

et al. 2020

BMC Cardiovasc Disord

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Background Amyloidosis is a rare systemic disease due to the extracellular tissue deposition of a fibrillar-shaped misfolded protein, called amyloid. Only two types of proteins commonly affect the heart leading to an infiltrative cardiomyopathy: immunoglobulin light chain and transthyretin (TTR) cardiac amyloidosis (CA). Despite the promising role of emerging imaging modalities, such as strain echocardiography, cardiac magnetic resonance and bone scintigraphy, its diagnosis is still often missed or delayed due to their inherent limitations and to a nonspecific clinical scenario with frequent concomitance of cardiac comorbidities. The gold standard for a definite diagnosis still remains endomyocardial biopsy, but in rare cases Congo Red staining could provide false negative results, as in our case, requiring immunoelectron microscopy. Case presentation A middle-aged male adult presented to the emergency department for relapse of heart failure. Echocardiography and cardiac magnetic resonance, along with the history of bilateral carpal tunnel syndrome, were suspicious for TTR-CA. The diagnosis, however, was hampered by concomitant cardiac comorbidities and conflicting results of imaging modalities. In fact bone scintigraphy was negative, as well as Congo Red Staining on myocardial tissue samples obtained by endomyocardial biopsy. Given the high clinical suspicion, immunoelectron microscopy was performed, showing TTR amyloid fibrils deposits, that confirmed the diagnosis. A genetic analysis excluded and hereditary form. The patient was then referred to a specialist center for specific treatment. Conclusions This is a rare case of a TTR-CA with a negative Bone Scintigraphy and Congo red staining, which demonstrated that CA is frequently misdiagnosed because of the low specific clinical manifestations and the results of imaging modalities that sometimes could be misleading, with subsequent delayed diagnosis and correct treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Negative bone scintigraphy in wild-type transthyretin cardiac amyloidosis

Martini

Rizzo

Sarais

et al. 2020

BMC Cardiovasc Disord

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“…This operator-dependent choice might be challenging in case of massive infiltration of the heart, for example in case of amyloidosis, and may result in erroneous choices resulting in non-diagnostic examinations ( Fig. 1) [4]. The introduction Fig.…”

Section: Cmr Approach To Restrictive Cardiomyopathiesmentioning

confidence: 99%

Cardiovascular magnetic resonance (CMR) in restrictive cardiomyopathies

et al. 2020

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The restrictive cardiomyopathies constitute a heterogeneous group of myocardial diseases with a different pathogenesis and overlapping clinical presentations. Diagnosing them frequently poses a challenge. Echocardiography, electrocardiograms and laboratory tests may show non-specific changes. In this context, cardiac magnetic resonance (CMR) may play a crucial role in defining the diagnosis and guiding treatments, by offering a robust myocardial characterization based on the inherent magnetic properties of abnormal tissues, thus limiting the use of endomyocardial biopsy. In this review article, we explore the role of CMR in the assessment of a wide range of myocardial diseases causing restrictive patterns, from iron overload to cardiac amyloidosis, endomyocardial fibrosis or radiation-induced heart disease. Here, we emphasize the incremental value of novel relaxometric techniques such as T1 and T2 mapping, which may recognize different storage diseases based on the intrinsic magnetic properties of the accumulating metabolites, with or without the use of gadolinium-based contrast agents. We illustrate the importance of these CMR techniques and their great support when contrast media administration is contraindicated. Finally, we describe the useful role of cardiac computed tomography for diagnosis and management of restrictive cardiomyopathies when CMR is contraindicated.

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“…Both AL-CA and ATTR-CA demonstrate the appearance of concentric hypertrophy, although a subgroup of patients with CA (particularly ATTR-CA) may present with asymmetric septal hypertrophy mimicking hypertrophic cardiomyopathy [6]. Importantly, the echocardiographic finding of LV hypertrophy in patients with CA is misleading, since the LV thickening is due to infiltrating amyloid fibrils rather than true myocyte hypertrophy, unless compensatory myocyte hypertrophy occurs [7, 8]. The mitral LV inflow pattern ranges from abnormal relaxation pattern in early disease (to be differentiated from a physiologic ageing manifestation) to the more specific restrictive filling pattern in severe, advanced disease.…”

Section: Ca Phenotype Of Heart Failure With a Preserved Ejection Fracmentioning

confidence: 99%

Cardiac Care of Patients with Cardiac Amyloidosis

Zadok

Kornowski

2020

Acta Haematol

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Cardiac amyloidosis, the majority of cases of which are due to immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR), affects different aspects of the heart and cardiovascular system. Amyloid-induced cardiomyopathy, clinically manifesting with heart failure and electrophysiological abnormalities, has distinct characteristics compared to non-amyloid cardiomyopathies. Accordingly, specific management strategies are required. This paper will review the cardiovascular manifestations of patients with cardiac amyloidosis and their suggested treatment strategies, emphasizing the importance of multidisciplinary care.

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How to Image Cardiac Amyloidosis

Cited by 200 publications

References 70 publications

Negative bone scintigraphy in wild-type transthyretin cardiac amyloidosis

Negative bone scintigraphy in wild-type transthyretin cardiac amyloidosis

Cardiovascular magnetic resonance (CMR) in restrictive cardiomyopathies

Cardiac Care of Patients with Cardiac Amyloidosis

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