Predictors of cardiac involvement and survival in patients with primary systemic light-chain amyloidosis: roles of the clinical, chemical, and 3-D speckle tracking echocardiography parameters

Zhu, Xiaoli; Hsi, David H.; Wang, Jing; Zuo, Li; Ta, Shengjun; Yang, Qianli; Xu, Lei; Zhao, Xueqin; Wang, Yan; Sun, Shan; Liu, Liwen

doi:10.1186/s12872-021-01856-3

Cited by 21 publications

(18 citation statements)

References 42 publications

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“…An apical sparing describes a pattern of opposing strain values within segments of the left ventricle. The presence of an apical sparing pattern in CA cohorts was noted by Löfbacka and colleagues (2021) , Pagourelias and colleagues (2017 ), Boldrini and colleagues (2020 ), Zhang and colleagues (2021 ), and Lei and colleagues (2021 ). In the study conducted by Roslan and colleagues (2018 ), strain imaging was available for only 7 out of 13 patients, all of which demonstrated apical sparing pattern.…”

Section: Resultsmentioning

confidence: 70%

“…3D TTE with speckle tracking focuses on mechanics of left ventricle–deriving global strain values from measurements of LV end-systolic and end-diastolic volumes, LV mass, and LVEF. Two of the studies ( Pradel et al, 2019 ; Lei et al 2021 ) evaluated the role of 3D echocardiography with speckle tracking. Both studies focused solely on the diagnosis of AL amyloidosis.…”

Section: Resultsmentioning

confidence: 99%

“…Both studies focused solely on the diagnosis of AL amyloidosis. In a 2021 retrospective cohort study, Lei and colleagues (2021 ) reviewed and performed strain analysis on 74 patients with confirmed AL amyloidosis. The primary focus of the study was to evaluate 3D speckle tracking echocardiography and conventional 2D echocardiogram in combination with additional markers to aid diagnosis of CA.…”

Section: Resultsmentioning

confidence: 99%

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The Role of Echocardiogram and Cardiac Rhythm Analysis for Early Detection of Cardiac Amyloidosis

Hubert¹,

Dains²

2022

JADPRO

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Purpose: The purpose of this integrative review was to establish the role of cardiac rhythm analysis (electrocardiogram; EKG) and echocardiogram in increasing clinical suspicion for and earlier diagnosis of cardiac amyloidosis. Methods: A literature review was conducted using PubMed and Scopus databases. Dates searched were from January 2017 to May 2021. Inclusion criteria included a diagnosis of cardiac amyloidosis, use of EKG, and echocardiogram participants 18 years and older. Articles were excluded if they were duplicates, had an irrelevant title, or were incomplete. Results: Results indicated neither EKG nor echocardiogram alone or in combination are sufficient for diagnosing cardiac amyloidosis. There is, however, a combination of findings that could potentially prove useful in “ruling in” cardiac amyloidosis and prompt further evaluation. Predominant findings in cardiac amyloidosis cohorts found on EKG showed low-voltage QRS complexes, a pseudo-infarct pattern in precordial leads, and an absence of left ventricular hypertrophy on EKG. There is no single echocardiogram finding specific to cardiac amyloidosis. Patients will generally present with thickened ventricular walls, and nearly all patients will display a preserved left ventricular ejection fraction until later stages of disease. Strain imaging, either via 2D or 3D transthoracic echocardiogram, is more useful in screening for or detecting cardiac amyloidosis and should be utilized in this instance. Findings in cardiac amyloidosis include decreased global longitudinal strain and relative apical sparing. Conclusion: Overall, EKG and echocardiogram are effective, feasible, and practical tools to increase clinical suspicion for cardiac amyloidosis for the purposes of early recognition and evaluation. These are useful only to “rule in” a diagnosis. Future studies are needed to validate these findings.

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Section: Resultsmentioning

confidence: 70%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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The Role of Echocardiogram and Cardiac Rhythm Analysis for Early Detection of Cardiac Amyloidosis

Hubert¹,

Dains²

2022

JADPRO

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“…The major determinant of outcome in amyloidosis is the extent of cardiac involvement 28 . Echocardiographic wall thickening, diastolic relaxation abnormalities, right ventricular dysfunction with valvular thickening, and abnormal echocardiographic strain have all been shown to be associated with prognosis.…”

Section: Prognosismentioning

confidence: 99%

Immunoglobulin light chain amyloidosis: 2022 update on diagnosis, prognosis, and treatment

Gertz

2022

American J Hematol

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Disease overview Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include heart failure with preserved ejection fraction, nephrotic syndrome, hepatic dysfunction, peripheral/autonomic neuropathy, and “atypical smoldering multiple myeloma or monoclonal gammopathy of undetermined significance (MGUS).” Diagnosis Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple‐green birefringence is required for the diagnosis of AL amyloidosis. Invasive organ biopsy is not required in 85% of patients. Verification that amyloid is composed of immunoglobulin light chains is mandatory. The gold standard is laser capture mass spectroscopy. Prognosis N‐terminal pro–brain natriuretic peptide (NT‐proBNP or BNP), serum troponin T (or I), and difference between involved and uninvolved immunoglobulin free light chain values are used to classify patients into four groups of similar size; median survivals are 73, 35, 15, and 5 months. Therapy All patients with a systemic amyloid syndrome require therapy to prevent deposition of amyloid in other organs and prevent progressive organ failure. Current first‐line therapy with the best outcome is daratumumab, bortezomib, cyclophosphamide, and dexamethasone. The goal of therapy is a complete response (CR). In patients failing to achieve this depth of response options for consolidation include pomalidomide, stem cell transplantation, venetoclax, and bendamustine. Future challenges Delayed diagnosis remains a major obstacle to initiating effective therapy prior to the development of end‐stage organ failure. Trials of antibodies to catabolize deposited fibrils are underway.

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“…A large number of cardiovascular parameters have been investigated for prognostic relevance with regards to AL amyloidosis and are summarized in Table 1 . 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 For those thought to have isolated renal amyloidosis, a PR interval over 160 msec and a QTc interval over 417 msec were both independently associated with all-cause mortality ( P values of .005 and .004, respectively) 49 and could therefore serve as a simple way to screen for patients who warrant a more intensive look at their cardiac function prior to initiation of therapy.…”

Section: Pretreatment Variablesmentioning

confidence: 99%

Light Chain Amyloidosis: Epidemiology, Staging, and Prognostication

Baker

2022

Methodist DeBakey Cardiovascular Journal

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Amyloidosis is a disorder of protein misfolding and metabolism in which insoluble fibrils are deposited in various tissues, causing organ dysfunction and eventually death. Out of the 60-plus heterogeneous amyloidogenic proteins that have been identified, approximately 30 are associated with human disease. The unifying feature of these proteins is their tendency to form beta-pleated sheets aligned in an antiparallel fashion. These sheets then form rigid, nonbranching fibrils that resist proteolysis, causing mechanical disruption and local oxidative stress in affected organs such as the heart, liver, kidneys, nervous system, and gastrointestinal tract. Here we review the epidemiology of light chain amyloidosis, the staging, and the concomitant prognostication that is critical in determining the appropriate treatment.

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Predictors of cardiac involvement and survival in patients with primary systemic light-chain amyloidosis: roles of the clinical, chemical, and 3-D speckle tracking echocardiography parameters

Cited by 21 publications

References 42 publications

The Role of Echocardiogram and Cardiac Rhythm Analysis for Early Detection of Cardiac Amyloidosis

The Role of Echocardiogram and Cardiac Rhythm Analysis for Early Detection of Cardiac Amyloidosis

Immunoglobulin light chain amyloidosis: 2022 update on diagnosis, prognosis, and treatment

Light Chain Amyloidosis: Epidemiology, Staging, and Prognostication

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