Cardiac amyloidosis: An underdiagnosed/underappreciated disease

Manolis, Antonis S; Manolis, Antonis A.; Manolis, Theodora A.; Melita, Helen

doi:10.1016/j.ejim.2019.07.022

Cited by 42 publications

(73 citation statements)

References 135 publications

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“…Transthyretin-related amyloidoses (ATTR) may be either hereditary (caused by autosomal dominant mutations in the TTR gene) or acquired (due to misaggregation of wild-type transthyretin). ATTR amyloidosis is an increasingly common cause of HFpEF and must be excluded in patients suspected of HF [ 63 , 64 ]. The amyloid is deposited in the myocardium and/or peripheral nervous system [ 65 ].…”

Section: Hfpef and Amyloidosismentioning

confidence: 99%

Heart Failure with Preserved Ejection Fraction—a Concise Review

et al. 2020

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Purpose of Review Heart failure with preserved ejection fraction (HFpEF) is a relatively new disease entity used in medical terminology; however, both the number of patients and its clinical significance are growing. HFpEF used to be seen as a mild condition; however, the symptoms and quality of life of the patients are comparable to those with reduced ejection fraction. The disease is much more complex than previously thought. In this article, information surrounding the etiology, diagnosis, prognosis, and possible therapeutic options of HFpEF are reviewed and summarized. Recent Findings It has recently been proposed that heart failure (HF) is rather a heterogeneous syndrome with a spectrum of overlapping and distinct characteristics. HFpEF itself can be distilled into different phenotypes based on the underlying biology. The etiological factors of HFpEF are unclear; however, systemic low-grade inflammation and microvascular damage as a consequence of comorbidities associated with endothelial dysfunction, oxidative stress, myocardial remodeling, and fibrosis are considered to play a crucial role in the pathogenesis of a disease. The H 2 FPEF score and the HFpEF nomogram are recently validated highly sensitive tools employed for risk assessment of subclinical heart failure. Summary Despite numerous studies, there is still no evidence-based pharmacotherapy for HFpEF and the mortality and morbidity associated with HFpEF remain high. A better understanding of the etiological factors, the impact of comorbidities, the phenotypes of the disease, and implementation of machine learning algorithms may play a key role in the development of future therapeutic strategies.

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Section: Hfpef and Amyloidosismentioning

confidence: 99%

Heart Failure with Preserved Ejection Fraction—a Concise Review

et al. 2020

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“…In recent years, specific clinical presentations of ATTR-AC have been identified [14] , [15] , [16] . In particular, elderly patients with HF and preserved EF admitted to cardiology or internal medicine departments may harbour amyloid in their hearts.…”

Section: Changing Perspectives On Attr-acmentioning

confidence: 99%

Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery

Porcari

Merlo

Rapezzi

et al. 2020

European Journal of Internal Medicine

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Highlights Transthyretin amyloid cardiomyopathy (ATTR-AC) is an under-diagnosed disease. Many grey areas are emerging in clinical management and prognostic stratification. Candidates’ selection for novel drugs is crucial, but response criteria are lacking. The impact of evidence-based therapies for HF in ATTR-AC should be investigated. Multidisciplinary team is the way to deliver the best clinical management.

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“…Стабилизация транстиретина (тафамидис, дифлюнизал, зеленый чай, ТТR-стабилизатор AG10) 3. Расщепление амилоидных фибрилл (доксициклин/таурдезоксихолевая кисота (ТUDCA), моноклональнын антитела) [48].…”

Section: патогенетическое лечение аттR-амилоидозаunclassified

Cardiac Amyloidosis: Internist and Cardiologist Insight

Резник

Нгуен

Степанова

et al. 2020

Arhivʺ vnutrennej mediciny

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Cardiac amyloidosis (amyloid cardiomyopathy) is a disease damage to the heart caused by extracellular amyloid deposition. In some cases, there may be local damage to the structures of the heart, for example, the atria; more often, heart damage is part of a systemic (generalized) pathology. Depending on the amyloid precursor protein, 36 types of amyloidosis are described, among which hereditary and acquired forms are distinguished. Cardiac amyloidosis is diagnosed 1) in the case of the amyloid infiltration in the myocardial bioptates or 2) in the case of non-cardiac amyloid deposition and the left ventricular wall thickening >12 mm without arterial hypertension and other reasons. The heart is most often affected in AL-, ATTR-, AA-, AANF-types of amyloidosis. Cardiac amyloidosis should be considered in patients with a heart failure with an unclear etiology, especially with preserved left ventricular ejection fraction, refractory to treatment, with proteinuria and CKD 4-5, in patients with idiopathic atrial fibrillation and conduction disturbances, in patients with left ventricular wall thickening of unclear etiology, low ECG voltage, unexplained arterial hypotension and pulmonary hypertension. Screening for cardiac amyloidosis should include non-invasive methods such as electrophoresis and immunofixation of blood and urine proteins, the free light lambda and kappa chains of immunoglobulins, 99Tc-DPD scintigraphy, genetic testing (if hereditary variants of amyloidosis are suspected), as well as a histological examination of biopsy samples stained with Congo red and polarizing microscopy.

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Cardiac amyloidosis: An underdiagnosed/underappreciated disease

Cited by 42 publications

References 135 publications

Heart Failure with Preserved Ejection Fraction—a Concise Review

Heart Failure with Preserved Ejection Fraction—a Concise Review

Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery

Cardiac Amyloidosis: Internist and Cardiologist Insight

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