Cardiac Amyloid Load

Kristen, Arnt V.; Brokbals, Eva; Siepen, Fabian aus dem; Bauer, Ralf; Hein, Selina; Aurich, Matthias; Riffel, Johannes; Behrens, Hans‐Michael; Krüger, Sandra; Schirmacher, Peter; Katus, Hugo A.; Röcken, Christoph

doi:10.1016/j.jacc.2016.04.035

Cited by 80 publications

(23 citation statements)

References 42 publications

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“…Mean arterial pressure is a predictor of mortality in the THAOS database among patients in the United States [36]. Low blood pressure has also been associated with greater mortality in patients with light-chain cardiac amyloidosis [16,37] as well as heart failure with reduced ejection fraction [38,39]. Decreases in arterial blood pressure may represent worsening amyloid with lower cardiac stroke volume or even the effects of progressive autonomic dysfunction in effected individuals [40].…”

Section: Discussionmentioning

confidence: 99%

Myocardial Contraction Fraction by M-Mode Echocardiography Is Superior to Ejection Fraction in Predicting Mortality in Transthyretin Amyloidosis

Rubin

Steidley

Carlsson

et al. 2018

Journal of Cardiac Failure

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Section: Discussionmentioning

confidence: 99%

Myocardial Contraction Fraction by M-Mode Echocardiography Is Superior to Ejection Fraction in Predicting Mortality in Transthyretin Amyloidosis

Rubin

Steidley

Carlsson

et al. 2018

Journal of Cardiac Failure

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“…transplantation, dialysis, and occasionally, organ transplantation [12,15]. Median survival of systemic AL amyloidosis with cardiac involvement is 15.7 months [16].…”

Section: G H -D O S E C H E M O T H E R a P Y A U T O L O G O U S mentioning

confidence: 99%

Local vs. systemic pulmonary amyloidosis—impact on diagnostics and clinical management

et al. 2018

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Immunoglobulin-derived light-chain (AL) amyloidosis of lungs and bronchi can appear as a systemic and a local form. While systemic AL amyloidosis may need haemato-oncological care, the localised form can be treated restrained. We re-evaluated 207 specimens of lungs and bronchi sent in for amyloid diagnostics. Amyloid was diagnosed by polarization microscopy using Congo red-stained tissue specimens and classified immunohistochemically. Histoanatomical amyloid distribution patterns were documented as well as additional histological findings. For 118 patients with AL amyloidosis, we retrieved clinical data. CT scan results were available from 59 patients. AL amyloidosis was the most common type (183 cases). ALλ was found in 141 and of ALκ in 27 cases. Fifteen cases were AL amyloid not otherwise specified. Twenty cases harboured transthyretin and three serum amyloid A derived amyloid. By correlation of histoanatomy, radiological and clinical data, amyloid was rarely in the initial differential diagnosis. Local AL amyloidosis often presented with a nodular pattern on CT scan and showed a significantly better disease-specific 10-year survival compared with systemic AL amyloidosis (96.0 vs. 51.9%). Localised and systemic pulmonary and bronchial AL amyloidosis are having a completely different prognosis. While CT scan might be indicative, histological and clinical assessment are mandatory to reach a proper diagnosis and guide patient care.

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“…Unfortunately, all of our patients had advanced disease with high amyloid burden and concomitant systolic dysfunction. When myocardial amyloid burden reaches a certain threshold, cardiac damage is known to become irreversible and chemotherapy does not appear to improve outcomes [ 17 ]. Two of our 3 patients had cerebral ischemic events despite sinus rhythm.…”

Section: Discussionmentioning

confidence: 99%

Cardiac Amyloidosis Detected on Imaging of Patients with Heart Failure

Roginić

Vinter²,

Trbušić³

et al. 2020

Am J Case Rep

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Cardiac Amyloid Load

Abstract: Early identification of subjects with AL amyloid is essential given that in late-stage disease with extensive amyloid load, our data suggested that outcomes are not affected by administration of chemotherapy.

Cited by 80 publications

References 42 publications

Myocardial Contraction Fraction by M-Mode Echocardiography Is Superior to Ejection Fraction in Predicting Mortality in Transthyretin Amyloidosis

Myocardial Contraction Fraction by M-Mode Echocardiography Is Superior to Ejection Fraction in Predicting Mortality in Transthyretin Amyloidosis

Local vs. systemic pulmonary amyloidosis—impact on diagnostics and clinical management

Cardiac Amyloidosis Detected on Imaging of Patients with Heart Failure

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