Diagnosis and supportive therapeutic management of cardiac light chain amyloidosis—a cardiologist’s perspective

Binder, Christina; Duca, Franz

doi:10.1007/s12254-021-00678-5

Cited by 5 publications

(3 citation statements)

References 62 publications

(58 reference statements)

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“…The cardiac workup is described in detail in the manuscript of Duca and Binder in this issue [22]. The cardiac work up is of major importance particularly since cardiac impairment directly influences treatment tolerability and survival.…”

Section: Cardiac Work Upmentioning

confidence: 99%

Immunoglobulin light chain amyloidosis

Agis

Krauth

2021

memo

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SummaryImmunoglobulin light chain (AL) amyloidosis is a rare and underdiagnosed life-threatening systemic disease, primarily caused by insoluble depositions of misfolded monoclonal light chains. The monoclonal light chain paraprotein originates from a small clonal B‑cell or a clonal plasma cell population. If left undetected the paraprotein can induce a number of complications based on organ damage. The most dangerous and life-threatening organ dysfunction emerges from cardiac involvement. Thus, patients overall survival depends on early detection. Establishing the correct diagnosis and clear characterization of the amyloid-forming protein, staging, risk assessment and treatment are crucial and depend on a highly experienced interdisciplinary, multiprofessional team.

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Section: Cardiac Work Upmentioning

confidence: 99%

Immunoglobulin light chain amyloidosis

Agis

Krauth

2021

memo

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“…Scattered evidence on valve disease derives from echocardiographic studies. Patients with CA have been reported to have thickened mitral or aortic valves in up to 31% of cases [10][11][12]; mitral regurgitation is often mild to moderate [13], but hemodynamically signi cant mitral or tricuspid regurgitation can be found in 50% of patients in more advanced stages [11]. In this study we performed the rst systematic assessment of the echocardiographic features of valvular CA.…”

Section: Introductionmentioning

confidence: 99%

Valve disease in cardiac amyloidosis: an echocardiographic score

Aimo

Fabiani

Maccarana

et al. 2022

Preprint

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Background Cardiac amyloidosis (CA) may affect all cardiac structures, including the valves. Methods From 423 patients undergoing a diagnostic workup for CA we selected 2 samples of 20 patients with amyloid transthyretin (ATTR-) or light-chain (AL-) CA, and age- and sex-matched controls. We chose 31 echocardiographic items related to the mitral, aortic and tricuspid valves, giving a value of 1 to each abnormal item. Results Patients with ATTR-CA displayed more often a shortened/hidden and restricted posterior mitral valve leaflet (PMVL), thickened mitral chordae tendineae and aortic stenosis than those with AL-CA, and less frequent PMVL calcification than matched controls. Score values were 15.8 (13.6–17.4) in ATTR-CA, 11.0 (9.3–14.9) in AL-CA, 12.8 (11.1–14.4) in ATTR-CA controls, and 11.0 (9.1–13.0) in AL-CA controls (p = 0.004 for ATTR- vs. AL-CA, 0.009 for ATTR-CA vs. their controls, and 0.461 for AL-CA vs. controls). Area under the curve values to diagnose ATTR-CA were 0.782 in patients with ATTR-CA or matched controls, and 0.773 in patients with LV hypertrophy. Conclusions Patients with ATTR-CA have a prominent impairment of mitral valve structure and function, and higher score values. The valve score is quite effective in identifying patients with ATTR-CA among patients with CA or unexplained hypertrophy.

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“…Scattered evidence on valve disease derives from echocardiographic studies. Patients with CA have been reported to have thickened mitral or aortic valves in up to 31% of cases [ 10 – 12 ]; mitral regurgitation is often mild to moderate [ 13 ], but hemodynamically significant mitral or tricuspid regurgitation can be found in 50% of patients in more advanced stages [ 11 ]. In this study we performed the first systematic assessment of the echocardiographic features of valvular CA.…”

Section: Introductionmentioning

confidence: 99%

Valve disease in cardiac amyloidosis: an echocardiographic score

Aimo

Fabiani

Maccarana

et al. 2023

Int J Cardiovasc Imaging

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Cardiac amyloidosis (CA) may affect all cardiac structures, including the valves. From 423 patients undergoing a diagnostic workup for CA we selected 2 samples of 20 patients with amyloid transthyretin (ATTR-) or light-chain (AL-) CA, and age- and sex-matched controls. We chose 31 echocardiographic items related to the mitral, aortic and tricuspid valves, giving a value of 1 to each abnormal item. Patients with ATTR–CA displayed more often a shortened/hidden and restricted posterior mitral valve leaflet (PMVL), thickened mitral chordae tendineae and aortic stenosis than those with AL–CA, and less frequent PMVL calcification than matched controls. Score values were 15.8 (13.6–17.4) in ATTR–CA, 11.0 (9.3–14.9) in AL–CA, 12.8 (11.1–14.4) in ATTR–CA controls, and 11.0 (9.1–13.0) in AL–CA controls (p = 0.004 for ATTR- vs. AL–CA, 0.009 for ATTR–CA vs. their controls, and 0.461 for AL–CA vs. controls). Area under the curve values to diagnose ATTR–CA were 0.782 in patients with ATTR–CA or matched controls, and 0.773 in patients with LV hypertrophy. Patients with ATTR–CA have a prominent impairment of mitral valve structure and function, and higher score values. The valve score may help identify patients with ATTR–CA among patients with CA or unexplained hypertrophy.

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Diagnosis and supportive therapeutic management of cardiac light chain amyloidosis—a cardiologist’s perspective

Cited by 5 publications

References 62 publications

Immunoglobulin light chain amyloidosis

Immunoglobulin light chain amyloidosis

Valve disease in cardiac amyloidosis: an echocardiographic score

Valve disease in cardiac amyloidosis: an echocardiographic score

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