Atrial amyloidosis: mechanisms and clinical manifestations

Vergaro, Giuseppe; Aimo, Alberto; Rapezzi, Claudio; Castiglione, Vincenzo; Fabiani, Iacopo; Pucci, Angela; Buda, Gabriele; Passino, Claudio; Lupón, Josep; Bayés‐Genís, Antoni; Emdin, Michele; Braunwald, Eugene

doi:10.1002/ejhf.2650

Cited by 36 publications

(20 citation statements)

References 59 publications

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“…AANP amyloid deposits in the atrium, often referred to as isolated atrial amyloidosis (IAA) [ 8 , 9 ], have been reported in surgically resected appendages and autopsy studies [ 4 , 10 – 16 ]; however, very few histologically reports of ATTR amyloid in the atrium are published [ 4 , 10 – 12 ]. Further, most previous studies were limited to evaluation of the atrium and did not evaluate the ventricles or other cardiac structures.…”

Section: Discussionmentioning

confidence: 99%

Transthyretin derived amyloid deposits in the atrium and the aortic valve: insights from multimodality evaluations and mid-term follow up

Okada

Kakuta

Tadokoro

et al. 2023

BMC Cardiovasc Disord

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Background Recent studies have reported atrial involvement and coexistence of aortic stenosis in transthyretin (ATTR) cardiac amyloidosis (CA). However, pathological reports of extraventricular ATTR amyloid deposits in atrial structures or heart valves are limited, and the clinical implications of ATTR amyloid deposits outside the ventricles are not fully elucidated. Case presentation We report 3 cases of extraventricular ATTR amyloid deposits confirmed in surgically resected aortic valves and left atrial structures, all of which were unlikely to have significant ATTR amyloidosis infiltrating the ventricles as determined by multimodality evaluation including 99mtechnetium-pyrophosphate scintigraphy, cardiac magnetic resonance, endomyocardial biopsy and their mid-term clinical course up to 5 years. These findings suggested that these were extraventricular ATTR amyloid deposits localized in the aortic valve and the left atrium. Conclusions While long-term observation is required to fully clarify whether these extraventricular ATTR amyloid deposits are truly localized outside the ventricles or are early stages of ATTR-CA infiltrating the ventricles, our 3 cases with multimodality evaluations and mid-term follow up suggest the existence of extraventricular ATTR amyloid deposits localized in the aortic valve and left atrial structures.

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Section: Discussionmentioning

confidence: 99%

Transthyretin derived amyloid deposits in the atrium and the aortic valve: insights from multimodality evaluations and mid-term follow up

Okada

Kakuta

Tadokoro

et al. 2023

BMC Cardiovasc Disord

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“…As a consequence of atrial dysfunction and enlargement, patients with ATTR-CA have a higher risk of left atrial thrombosis even when in SR. This may also be related to the amyloid deposition into the atrial wall, and the cardiotoxic damage of atrial cardiomyocytes by amyloid precursors ( 77 ). Among patients with CA and AF/atrial flutter, anticoagulation is indicated regardless of CHA2DS2-VASc score as in these patients it is not associated with the probability of LAA thrombosis.…”

Section: Attr Symptomatic Treatmentmentioning

confidence: 99%

Treating amyloid transthyretin cardiomyopathy: lessons learned from clinical trials

Tomasoni

Bonfioli

Aimo

et al. 2023

Front. Cardiovasc. Med.

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An increasing awareness of the disease, new diagnostic tools and novel therapeutic opportunities have dramatically changed the management of patients with amyloid transthyretin cardiomyopathy (ATTR-CM). Supportive therapies have shown limited benefits, mostly related to diuretics for the relief from signs and symptoms of congestion in patients presenting heart failure (HF). On the other hand, huge advances in specific (disease-modifying) treatments occurred in the last years. Therapies targeting the amyloidogenic cascade include several pharmacological agents that inhibit hepatic synthesis of TTR, stabilize the tetramer, or disrupt fibrils. Tafamidis, a TTR stabilizer that demonstrated to prolong survival and improve quality of life in the ATTR-ACT trial, is currently the only approved drug for patients with ATTR-CM. The small interfering RNA (siRNA) patisiran and the antisense oligonucleotide (ASO) inotersen have been approved for the treatment of patients with hereditary ATTR polyneuropathy regardless of the presence of cardiac involvement, with patisiran also showing preliminary benefits on the cardiac phenotype. Ongoing phase III clinical trials are investigating another siRNA, vutrisiran, and a novel ASO formulation, eplontersen, in patients with ATTR-CM. CRISPR–Cas9 represents a promising strategy of genome editing to obtain a highly effective blockade of TTR gene expression.

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“…Isolated right or left atrial CA is related to atrial natriuretic peptide overproduction and has been found in about 50% of patients by Sukhacheva et al [ 28 , 29 ].…”

Section: Pathophysiology and Clinical Features Of The Right Involveme...mentioning

confidence: 99%

Imaging findings of right cardiac amyloidosis: impact on prognosis and clinical course

Tana

Palmiero³

et al. 2023

J Ultrasound

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Cardiac involvement from amyloidosis is of growing interest in the overall literature. Despite cardiac amyloidosis (CA) has been considered for a long time a rare disease, the diagnostic awareness is increasing mainly thanks to the improvement of diagnostic softwares and of imaging techniques such as cardiac magnetic resonance (CMR). Some authors have observed an increase of prevalence rate of CA; moreover it’s often underestimated because clinical manifestations are aspecific. The interstitial infiltration of the left ventricle has been extensively studied, while the involvement of the right ventricle (RV) has been less investigated. Involvement of the RV, even in the absence of pulmonary hypertension or clearly left ventricle infiltration, plays an important role as prognostic factor and is useful to achieve an early diagnosis. Therefore, the use of fast and low-cost diagnostic methods such as ultrasound strain of the right ventricle could be used to recognize cardiac amyloidosis early. Herein the importance of evaluating the right ventricular involvement, which can predict the most severe course of the disease also without overt clinical manifestations. The role of imaging, in particular of echocardiography, CMR, and scintigraphy is here reported.

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Atrial amyloidosis: mechanisms and clinical manifestations

Cited by 36 publications

References 59 publications

Transthyretin derived amyloid deposits in the atrium and the aortic valve: insights from multimodality evaluations and mid-term follow up

Transthyretin derived amyloid deposits in the atrium and the aortic valve: insights from multimodality evaluations and mid-term follow up

Treating amyloid transthyretin cardiomyopathy: lessons learned from clinical trials

Imaging findings of right cardiac amyloidosis: impact on prognosis and clinical course

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