CMR-based T1-mapping offers superior diagnostic value compared to longitudinal strain-based assessment of relative apical sparing in cardiac amyloidosis

Korthals, Dennis; Chatzantonis, Grigorios; Bietenbeck, Michael; Meier, Claudia; Stalling, Philipp; Yılmaz, Ali

doi:10.1038/s41598-021-94650-2

Cited by 20 publications

(15 citation statements)

References 43 publications

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“…Similar to the pattern seen with other imaging parameters, higher T1 in basilar segments compared to apical segments is commonly seen and can help differentiate CA from other disease states where T1 may be increased ( 63 , 64 ). Regional areas of higher T1 correlates with lower strain in the same segment as well as extracellular volume (ECV) and LGE ( 63 , 65 ). Incremental increases in basal and mid T1 correlates with higher mortality as well ( 64 ).…”

Section: Magnetic Resonance Imagingsupporting

confidence: 63%

Multimodality Imaging in the Evaluation and Prognostication of Cardiac Amyloidosis

Scheel

Mukherjee

Hays

et al. 2022

Front. Cardiovasc. Med.

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Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy resulting from deposition of misfolded immunoglobulin light chains (AL-CA) or transthyretin (ATTR-CA) proteins in the myocardium. Survival varies between the different subtypes of amyloidosis and degree of cardiac involvement, but accurate diagnosis is essential to ensure initiation of therapeutic interventions that may slow or potentially prevent morbidity and mortality in these patients. As there are now effective treatment options for CA, identifying underlying disease pathogenesis is crucial and can be guided by multimodality imaging techniques such as echocardiography, magnetic resonance imaging, and nuclear scanning modalities. However, as use of cardiac imaging is becoming more widespread, understanding optimal applications and potential shortcomings is increasingly important. Additionally, certain imaging modalities can provide prognostic information and may affect treatment planning. In patients whom imaging remains non-diagnostic, tissue biopsy, specifically endomyocardial biopsy, continues to play an essential role and can facilitate accurate and timely diagnosis such that appropriate treatment can be started. In this review, we examine the multimodality imaging approach to the diagnosis of CA with particular emphasis on the prognostic utility and limitations of each imaging modality. We also discuss how imaging can guide the decision to pursue tissue biopsy for timely diagnosis of CA.

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Section: Magnetic Resonance Imagingsupporting

confidence: 63%

Multimodality Imaging in the Evaluation and Prognostication of Cardiac Amyloidosis

Scheel

Mukherjee

Hays

et al. 2022

Front. Cardiovasc. Med.

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“…Precisely, the association of diffuse subendocardial or transmural late gadolinium enhancement and an abnormal kinetics (myocardial nulling preceding or coinciding with blood pool), eventually coupled with an extracellular volume > 0.39%, is strongly supportive for the diagnosis of CA [94]. In support of this, a recent study published in Nature Scientific Reports suggests that CMR-based T1-mapping offers superior diagnostic value compared with longitudinal strain-based assessment of relative apical sparing in CA [95].…”

Section: Echocardiographic Characteristics Of a Patient With Amyloido...mentioning

confidence: 89%

Perspective Chapter: Lipoprotein (a), Cardiac Amyloidosis, and Aortic Stenosis - Underestimated Associations

Santangelo¹,

Bernardi²,

Faggiano³

et al. 2022

Aortic Stenosis - Recent Advances, New Perspectives and Applications

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This chapter aims to address two peculiar aspects of pathophysiology and clinical management of aortic valve stenosis, such as coexistence with cardiac amyloidosis and association with lipoprotein (a). Calcific aortic valve stenosis is the most common heart valve condition requiring surgical or transcatheter aortic valve replacement among adults in Western societies. Lipoprotein (a) has been shown to play an important role in the pathophysiological pathways leading to degenerative aortic stenosis, similar to that in the pathogenesis of atherosclerosis. Studies are needed to verify whether therapies that drastically reduce Lipoprotein (a) serum levels offer the possibility of a first medical treatment to arrest the progression of aortic stenosis. A large percentage of patients with aortic stenosis may have concomitant cardiac amyloidosis, commonly due to wild-type transthyretin. The challenge in this context is to differentiate aortic stenosis alone from aortic stenosis with cardiac amyloidosis, as cardiac amyloidosis shares several clinical, electrocardiographic, and echocardiographic features with the aortic stenosis phenotype. Recognition of transthyretin-related amyloidosis prior to any type of intervention is crucial for adequate risk stratification and to guide downstream management.

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“…An extensive literature search regarding the diagnostic value of non-invasive imaging modalities to diagnose cardiac AA amyloidosis did not result in any landmark studies nor in convincing case reports. Obviously, echocardiography is a widely available tool that has improved significantly the diagnosis of CA after the introduction of speckle-tracking-based strain analysis, however, is still lacking high sensitivity as well as specificity (21)(22)(23). In contrast, bone scintigraphy and targeted PET studies offer higher sensitivity and specificity regarding the detection of CA, and even allow to differentiate between AL and ATTR amyloidosis if additional monoclonal protein studies are available.…”

Section: Discussionmentioning

confidence: 99%

“…Our CMR protocol comprised a cine steady-state free precession pulse sequence for ventricular function and a two-dimensional (2D) inversion recovery fast spoiled gradient-echo sequence 10 to 15 min after administration of a gadolinium-based contrast agent (Gadobutrol 0.10 mmol/kg) for detection of myocardial pathology. Moreover, a modified Look-Locker inversion recovery (MOLLI) T1-mapping sequence was applied in basal, mid and apical short-axes prior to contrast agent administration and ~20 min thereafter to determine native T1 and ECV values as described previously ( 21 ). Motion corrected native and post-contrast T1 maps were generated from the pre- and post-contrast T1 sequences.…”

Section: Methodsmentioning

confidence: 99%

“…Additionally, for ECV calculation, a region of interest was drawn in the blood pool (avoiding the papillary muscles) in all analyzed T1 maps. Motion corrected and segmented ECV maps were generated from the native and post-contrast segmented T1 maps, using the patient's hematocrit level as described by us elsewhere ( 21 ). “Global” T1, T2 and ECV values were calculated by averaging all 16 segments from three short-axis slices.…”

Section: Methodsmentioning

confidence: 99%

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Diagnosis of Cardiac Involvement in Amyloid A Amyloidosis by Cardiovascular Magnetic Resonance Imaging

Chamling

Drakos

Bietenbeck

et al. 2021

Front. Cardiovasc. Med.

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Background: Diagnosis of cardiac involvement in amyloid A (AA) amyloidosis is challenging since AA amyloidosis is a rare disease and cardiac involvement even less frequent. The diagnostic yield of currently available non-invasive imaging methods is not well-studied and rather limited, and invasive endomyocardial biopsy (EMB) is rarely performed due to the potential risk of this procedure. Cardiovascular magnetic resonance (CMR)-based myocardial tissue characterization by late-gadolinium-enhancement (LGE) imaging and novel-mapping approaches may increase the diagnostic yield in AA amyloidosis.Methods: Two patients with AA amyloidosis in whom cardiac involvement was suspected based on CMR findings and subsequently proven by biopsy work-up are presented. CMR studies were performed on a 1.5-T system and comprised a cine steady-state free precession pulse sequence for ventricular function and a late-gadolinium-enhancement (LGE) sequence for detection of myocardial pathology. Moreover, a modified Look-Locker inversion recovery (MOLLI) T1-mapping sequence was applied in basal, mid and apical short-axes prior to contrast agent administration and ~20 min thereafter to determine native T1 and ECV values.Results: Both patients showed slightly dilated left ventricles (LV) with mild to moderate LV hypertrophy and preserved systolic function. Only a very subtle pattern of LGE was observed in both patients with AA amyloidosis. However, markedly elevated native T1 (max. 1,108 and 1,112 ms, respectively) and extracellular volume fraction (ECV) values (max. 39 and 48%, respectively) were measured in the myocardium suggesting the presence of cardiac involvement - with subsequent EMB-based proof of AA amyloidosis.Conclusion: We recommend a multi-parametric CMR approach in patients with AA amyloidosis comprising both LGE-based contrast-imaging and T1-mapping-based ECV measurement of the myocardium for non-invasive work-up of suspected cardiac involvement. The respective CMR findings may be used as gatekeeper for additional invasive procedures (such as EMB) and as a non-invasive monitoring tool regarding assessment and modification of ongoing treatments.

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CMR-based T1-mapping offers superior diagnostic value compared to longitudinal strain-based assessment of relative apical sparing in cardiac amyloidosis

Cited by 20 publications

References 43 publications

Multimodality Imaging in the Evaluation and Prognostication of Cardiac Amyloidosis

Multimodality Imaging in the Evaluation and Prognostication of Cardiac Amyloidosis

Perspective Chapter: Lipoprotein (a), Cardiac Amyloidosis, and Aortic Stenosis - Underestimated Associations

Diagnosis of Cardiac Involvement in Amyloid A Amyloidosis by Cardiovascular Magnetic Resonance Imaging

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