A national survey on prevalence of possible echocardiographic red flags of amyloid cardiomyopathy in consecutive patients undergoing routine echocardiography: study design and patients characterization — the first insight from the AC-TIVE Study

Merlo, Marco; Porcari, Aldostefano; Pagura, Linda; Cameli, Matteo; Vergaro, Giuseppe; Musumeci, Beatrice; Biagini, Elena; Canepa, Marco; Crotti, Lia; Imazio, Massimo; Forleo, Cinzia; Cappelli, Francesco; Favale, Stefano; Bella, Gianluca Di; Dore, Franca; Lombardi, Carlo; Pavasini, Rita; Rella, Valeria; Palmiero, Giuseppe; Caiazza, Martina; Albanese, Miriam; Guaricci, Andrea Igoren; Branzi, Giovanna; Caponetti, Angelo Giuseppe; Saturi, Giulia; Malfa, Giovanni La; Merlo, Andrea; Andreis, Alessandro; Bruno, Francesco; Longo, Francesca; Sfriso, Enrico; Ienno, Luca Di; Carli, Giuseppe De; Giacomin, Elisa; Spini, Valentina; Milidoni, Antonino; Limongelli, Giuseppe; Autore, Camillo; Olivotto, Iacopo; Badano, Luigi P.; Parati, Gianfranco; Perlini, Stefano; Metra, Marco; Emdin, Michele; Rapezzi, Claudio; Sinagra, Gianfranco

doi:10.1093/eurjpc/zwab127

Cited by 31 publications

(33 citation statements)

References 12 publications

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“…It has been identified in 13% of patients >60 years old with HFpEF and it was prevalent in 14-16% of older patients with severe calcified aortic stenosis undergoing transcatheter aortic valve replacement (166,(168)(169)(170)(171). The clinical suspicion of cardiac amyloidosis should arise when the patient presents electrocardiographic anomalies (such as low QRS voltage) and bilateral carpal tunnel syndrome, signs and symptoms of HF, septal and posterior wall thickness >14 mm (i.e., Interventricular septum diameter (IVSd) and Posterior wall diameter (PWd) >14 mm with reduced GLS and apical sparing as optional criteria) at echocardiography, and age >65 years (172). The diagnosis of AL amyloidosis can be confirmed by abnormal hematological tests, cardiac imaging, and endomyocardial or extra-cardiac biopsy (Figures 1, 3).…”

Section: Cardiac Amyloidosismentioning

confidence: 99%

Advances in Multimodality Cardiovascular Imaging in the Diagnosis of Heart Failure With Preserved Ejection Fraction

Torto

Guaricci²,

Pomarico³

et al. 2022

Front. Cardiovasc. Med.

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Heart failure with preserved ejection fraction (HFpEF) is a syndrome defined by the presence of heart failure symptoms and increased levels of circulating natriuretic peptide (NP) in patients with preserved left ventricular ejection fraction and various degrees of diastolic dysfunction (DD). HFpEF is a complex condition that encompasses a wide range of different etiologies. Cardiovascular imaging plays a pivotal role in diagnosing HFpEF, in identifying specific underlying etiologies, in prognostic stratification, and in therapeutic individualization. Echocardiography is the first line imaging modality with its wide availability; it has high spatial and temporal resolution and can reliably assess systolic and diastolic function. Cardiovascular magnetic resonance (CMR) is the gold standard for cardiac morphology and function assessment, and has superior contrast resolution to look in depth into tissue changes and help to identify specific HFpEF etiologies. Differently, the most important role of nuclear imaging [i.e., planar scintigraphy and/or single photon emission CT (SPECT)] consists in the screening and diagnosis of cardiac transthyretin amyloidosis (ATTR) in patients with HFpEF. Cardiac CT can accurately evaluate coronary artery disease both from an anatomical and functional point of view, but tissue characterization methods have also been developed. The aim of this review is to critically summarize the current uses and future perspectives of echocardiography, nuclear imaging, CT, and CMR in patients with HFpEF.

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Section: Cardiac Amyloidosismentioning

confidence: 99%

Advances in Multimodality Cardiovascular Imaging in the Diagnosis of Heart Failure With Preserved Ejection Fraction

Torto

Guaricci²,

Pomarico³

et al. 2022

Front. Cardiovasc. Med.

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“…However, these studies had a mainly histological focus; none of them were conducted on unselected populations, nor did they provide a correlation between histological and clinical data. The epidemiology of the disease is currently unknown, especially regarding ATTR, but recent investigations using cardiac scintigraphy with bone tracers suggest that CA is a relatively frequent condition (5)(6)(7).…”

Section: Introductionmentioning

confidence: 99%

Incidence and Characterization of Concealed Cardiac Amyloidosis Among Unselected Elderly Patients Undergoing Post-mortem Examination

Porcari

Bussani

Merlo

et al. 2021

Front. Cardiovasc. Med.

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Background: The prevalence of cardiac amyloidosis (CA) is unknown.Aims and Methods: We sought to (a) determine the prevalence of CA in unselected patients ≥75 years undergoing autopsy, (b) characterize cardiological profiles of CA and non-CA patients by providing clinical-histological correlations, and (c) compare their cardiological profiles. After dedicated staining, the localization (interstitial or vascular) and the distribution (non-diffuse or diffuse) of amyloid deposition were analyzed. Cardiological data at last evaluation were retrospectively assessed for the presence of CA red-flags.Results: CA (50% light chains, 50% transthyretin) was found in 43% (n = 24/56) of the autopsied hearts. Atria were involved in 96% of cases. Amyloid localized both at the perivascular and interstitial levels (95.5 and 85%, respectively) with a slightly predominant non-diffuse distribution (58% of cases). Compared to the other patients, CA patients had a more frequent history of heart failure (HF) (79 vs. 47%, p = 0.014), advanced NYHA functional class (III-IV 25 vs. 6%, p = 0.047), atrial fibrillation (68 vs. 36%, p = 0.019), discrepancy between QRS voltage and left ventricular (LV) thickness (70 vs. 12%, p < 0.001), thicker LV walls (15 vs. 11 mm, p < 0.001), enlarged left atrium (49 vs. 42 mm, p = 0.019) and restrictive filling pattern (56 vs. 19%, p = 0.020). The presence of right ventricular amyloidosis seemed to identify hearts with a higher amyloid burden. Among the CA patients, >30% had ≥3 echocardiographic red-flags of disease.Conclusion: CA can be found in 43% of autopsied hearts from patients ≥75 years old, especially in patients with HF, LV hypertrophy and atrial fibrillation.

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“…Several echocardiographic red flags may raise the suspicion of CA. A prospective multicenter Italian study is currently investigating the prevalence of echocardiographic red flags of CA in consecutive patients ≥55 years who performed routine transthoracic echocardiography [29].…”

Section: Cardiac Amyloidosismentioning

confidence: 99%

“…The suspicion of cardiac amyloidosis may be triggered by some relevant clinical scenarios, such as low-flow low-gradient aortic stenosis, HFpEF, or when increased LV wall thickness (>12 mm) is associated with extracardiac red flags [10,29,101]. Cardiac scintigraphy with Tc99m-bone-avid radiotracers (PYP, HMDP or DPD) is highly specific in detecting transthyretin cardiac amyloidosis (ATTR-CA) [102,103].…”

Section: Cardiac Amyloidosismentioning

confidence: 99%

Multimodality Imaging in Cardiomyopathies with Hypertrophic Phenotypes

Monda

Palmiero

Lioncino

et al. 2022

JCM

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Multimodality imaging is a comprehensive strategy to investigate left ventricular hypertrophy (LVH), providing morphologic, functional, and often clinical information to clinicians. Hypertrophic cardiomyopathy (HCM) is defined by an increased LV wall thickness not only explainable by abnormal loading conditions. In the context of HCM, multimodality imaging, by different imaging techniques, such as echocardiography, cardiac magnetic resonance, cardiac computer tomography, and cardiac nuclear imaging, provides essential information for diagnosis, sudden cardiac death stratification, and management. Furthermore, it is essential to uncover the specific cause of HCM, such as Fabry disease and cardiac amyloidosis, which can benefit of specific treatments. This review aims to elucidate the current role of multimodality imaging in adult patients with HCM.

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A national survey on prevalence of possible echocardiographic red flags of amyloid cardiomyopathy in consecutive patients undergoing routine echocardiography: study design and patients characterization — the first insight from the AC-TIVE Study

Cited by 31 publications

References 12 publications

Advances in Multimodality Cardiovascular Imaging in the Diagnosis of Heart Failure With Preserved Ejection Fraction

Advances in Multimodality Cardiovascular Imaging in the Diagnosis of Heart Failure With Preserved Ejection Fraction

Incidence and Characterization of Concealed Cardiac Amyloidosis Among Unselected Elderly Patients Undergoing Post-mortem Examination

Multimodality Imaging in Cardiomyopathies with Hypertrophic Phenotypes

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