Non‐invasive predictors of survival in cardiac amyloidosis

Kristen, Arnt V.; Perz, Jolanta B.; Schönland, Stefan; Hegenbart, Ute; Schnabel, Philipp A.; Kristen, J. H.; Goldschmidt, Hartmut; Katus, Hugo A.; Dengler, Thomas J.

doi:10.1016/j.ejheart.2007.01.012

Cited by 112 publications

(101 citation statements)

References 29 publications

(31 reference statements)

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“…Left ventricular function is positively correlated with better prognosis in cardiac amyloidosis [158]. Though this study was very preliminary, without a control cohort, and the increase in left ventricular mass varied [159], the authors also found an increase in the mean mitral annular systolic velocity, a restorative effect, and a decrease in the total and low-density lipoprotein-associated cholesterol levels with no serious adverse events, suggesting that treatment with EGCG might be beneficial in these patients [157].…”

Section: Effects On Ttr Toxicity In Vivomentioning

confidence: 68%

Modulators of amyloid protein aggregation and toxicity: EGCG and CLR01

Attar

Rahimi

Bitan

2013

Translational Neuroscience

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Abnormal protein folding and self-assembly causes over 30 cureless human diseases for which no diseasemodifying therapies are available. The common side to all these diseases is formation of aberrant toxic protein oligomers and amyloid fibrils. Both types of assemblies are drug targets, yet each presents major challenges to drug design, discovery, and development. In this review, we focus on two small molecules that inhibit formation of toxic amyloid protein assemblies -the green-tea derivative (-)-epigallocatechin-3-gallate (EGCG), which was identified through a combination of epidemiologic data and a compound library screen, and the molecular tweezer CLR01, whose inhibitory activity was discovered in our group based on rational reasoning, and subsequently confirmed experimentally. Both compounds act in a manner that is not specific to one particular protein and thus are useful against a multitude of amyloidogenic proteins, yet they act via distinct putative mechanisms. CLR01 disrupts protein aggregation through specific binding to lysine residues, whereas the mechanisms underlying the activity of EGCG are only recently beginning to unveil. We discuss current in vitro and, where available, in vivo literature related to EGCG and CLR01's effects on amyloid β-protein, α-synuclein, transthyretin, islet amyloid polypeptide, and calcitonin. We also describe the toxicity, pharmacokinetics, and mechanism of action of each compound.

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Section: Effects On Ttr Toxicity In Vivomentioning

confidence: 68%

Modulators of amyloid protein aggregation and toxicity: EGCG and CLR01

Attar

Rahimi

Bitan

2013

Translational Neuroscience

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“…A low-voltage pattern on electrocardiography and LVEF allows identification of patients at high risk of death. 28 Dubrey et al demonstrated that late potentials were more frequent in patients with echocardiographic evidence of cardiac amyloidosis (31%) compared with patients with normal echocardiograms (9%, P<0.003), 29 and abnormal and signal-averaged electrocardiograms were both predictive of all-cause (P<0.0001) and sudden cardiac death (P<0.0001). Thus, 2D echocardiographic findings suggestive of advanced-stage cardiac amyloidosis may represent adverse prognostic factors.…”

Section: D Echocardiographic Assessment and Prognosismentioning

confidence: 99%

Echocardiographic Assessment of the Cardiac Amyloidoses

Koyama

Ikeda

2015

Circ J

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“…Cardiac amyloidosis is observed in approximately 50% of patients with systemic amyloidosis and functions as the most significant source of mortality in patients with AL amyloidosis. [7] In our case, we had diastolic cardiac insufficiency due to cardiac involvement and related symptoms. Systemic amyloidosis has been described in 5-15% of patients with MM.…”

Section: Discussionmentioning

confidence: 99%

The Diagnosis of Multiple Myeloma With Macroglossia and Edema Symptoms

Kara¹,

Tekinalp²,

Bilir³

et al. 2017

South Clin Ist Euras

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In this case, the importance of multiple myeloma in differential diagnosis of non-specific symptoms, such as macroglossia and significant pretibial edema, is highlighted. A 44-year-old male presented to the internal medicine outpatient clinic with complaints of ongoing and increasing enlargement of the tongue, difficulty in swallowing, and swelling of legs, all of which had been occurring for 6 months. In the CT scan, significant symmetrical enlargement of the tongue was detected. Making a pre-diagnosis of amyloidosis, a biopsy of the tongue was performed, but the results did not suggest amyloidosis. A rectal biopsy was therefore subsequently performed, and this time the results showed amyloid accumulation. Findings from the transthoracic echocardiography showed an ejection fraction of 48%, contrast hypertrophy in the left ventricle, Grade 3 diastolic dysfunction, 15-19 ml of pericardial fluid around the heart, and mild mitral failure. Taking into consideration the pathological and cardiologic findings, the patient was diagnosed with amyloidosis. Bearing in mind the possible relation between primary hematologic malignancy and amyloidosis, a bone marrow aspiration and a bone marrow biopsy were performed. From the results of the biopsy and aspiration of bone marrow, the patient was diagnosed with multiple myeloma and treatment plans were made accordingly. However, as the patient was in the process of undergoing treatment he died due to the development of cardiopulmonary failure.

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Non‐invasive predictors of survival in cardiac amyloidosis

Cited by 112 publications

References 29 publications

Modulators of amyloid protein aggregation and toxicity: EGCG and CLR01

Modulators of amyloid protein aggregation and toxicity: EGCG and CLR01

Echocardiographic Assessment of the Cardiac Amyloidoses

The Diagnosis of Multiple Myeloma With Macroglossia and Edema Symptoms

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