Unveiling outcomes in coexisting severe aortic stenosis and transthyretin cardiac amyloidosis

Rosenblum, Hannah; Masri, Ahmad; Narotsky, David L.; Goldsmith, Jeff; Hamid, Nadira; Hahn, Rebecca T.; Kodali, Susheel; Vahl, Torsten; Nazif, Tamim; Khalique, Omar; Bokhari, Sabahat; Soman, Prem; Cavalcante, João L.; Maurer, Mathew S.; Castaño, Adam

doi:10.1002/ejhf.1974

Cited by 88 publications

(107 citation statements)

References 34 publications

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“…The first anecdotic reports have questioned the usefulness of aortic valve replacement, even when performed by TAVI. More recent prospective studies in relatively large cohorts of patients with severe AS in the elderly with or without ATTR-AC undergoing TAVI have shown similar short and medium term results regarding overall survival (with a higher risk of hospitalization for HF amongst patients with ATTR-AC) [33,38]. Waiting for further randomized studies, these results suggest that TAVI should not be denied only on the basis of the coexistence of ATTR-AC.…”

Section: Atrial Fibrillation and Anticoagulationmentioning

confidence: 84%

“…Recent studies have shown that ATTR-AC frequently coexists with severe degenerative aortic stenosis (AS) (prevalence ranging from 8 to 16%) due to the shared epidemiologic substrate and the possible effect of increased myocardial strain of aortic stenosis acting on an underlying amyloidogenetic substrate [38]. The first anecdotic reports have questioned the usefulness of aortic valve replacement, even when performed by TAVI.…”

Section: Atrial Fibrillation and Anticoagulationmentioning

confidence: 99%

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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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Section: Atrial Fibrillation and Anticoagulationmentioning

confidence: 84%

Section: Atrial Fibrillation and Anticoagulationmentioning

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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“…Recently, several studies have evaluated the prognostic implications of the coexistence of AC-TTR and severe AS in TAVR candidates, showing how amyloidosis would not significantly impact the survival of patients with severe AS, and especially how TAVR intervention would improve outcome in patients in which the two pathologies coexist. 1 , 2 …”

mentioning

confidence: 99%

Transthyretin amyloidosis in aortic stenosis: clinical and therapeutic implications

Fabbri

Serenelli

Cantone

et al. 2021

European Heart Journal Supplements

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About one in seven elderly patients with severe calcific aortic stenosis (AS) also have ATTR amyloid cardiomyopathy (AC-TTR). The reasons for this close association are not fully known, but the two entities are not only related by common epidemiology. For example, it is possible to hypothesize that an amyloidotic infiltration of the aortic valve, even partial, can act as a trigger for the development of endothelial damage and subsequent calcification. Another hypothesis is the increased myocardial strain induced by AS may locally favour the process of amyloidogenesis and tissue infiltration. In a patient with AS, the coexistence of AC-TTR can be suspected by careful analysis of the echocardiogram and the ECG, especially if a clinical history of carpal tunnel syndrome coexists. Bone tracer scintigraphy allows a diagnosis of certainty. Recently, several studies have evaluated the prognostic implications of the coexistence of the two entities in candidates for percutaneous aortic valve replacement, showing how amyloidosis would not significantly impact the results of the procedure, but would only be associated with a greater risk of distant heart failure. In patients with AS associated with AC-TTR, valve replacement should not be ruled out in the presence of the usual clinical-haemodynamic indications.

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“…ATTR-CA is diagnosed by cardiac tracer uptake on bone scan and unremarkable serum and urine free light chain assessment. This method was applied in several studies on older AS patients undergoing transcatheter AVR (TAVR) and a prevalence of AS-CA ranging from 8.4% to 16.0% was reported [2,[6][7][8]. AL, the other predominant amyloid protein found in the heart, is rarely detected in severe AS scheduled for TAVR [6]; however, given the immediate therapeutic implications of AL-CA, this subtype always has to be ruled out by urine and serum free light chain assessment if AS-CA is suspected [5].…”

mentioning

confidence: 99%

“…Until recently, prognostic implications as well as effectiveness of AVR in dual AS-CA were unknown. Even futility of AVR was discussed; however, very recently several studies showed equal survival rates of AS-CA and AS alone following TAVR [2,6,7,9], disproving the conception of treatment futility in AS-CA.…”

mentioning

confidence: 99%