Characteristics and natural history of early-stage cardiac transthyretin amyloidosis

Law, Steven; Bézard, Mélanie; Petrie, Aviva; Chacko, Liza; Cohen, Oliver; Ravichandran, Sriram; Ogunbiyi, M Olabisi; Kharoubi, Mounira; Ganesananthan, Sashiananthan; Ganesananthan, Sharmananthan; Gilbertson, Janet A.; Rowczenio, Dorota; Wechalekar, Ashutosh; Martinez‐Naharro, Ana; Lachmann, HJ; Whelan, Carol; Hutt, David F.; Hawkins, Philip N.; Damy, Thibaud; Fontana, Marianna; Gillmore, Julian D.

doi:10.1093/eurheartj/ehac259

Cited by 39 publications

(25 citation statements)

References 24 publications

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“…However, RV free‐wall longitudinal strain (particularly in its basal and mid‐segments) is significantly more impaired in the AL subtype, followed by ATTRwt to ATTRv patients, allowing differentiation 17 . A RV apical ratio >0.8 and LV apical ratio >0.96 showed high accuracy in the differentiation between AL and ATTR 13 . On the other hand, LA reservoir and pump function on strain imaging are lower in ATTRwt compared to AL and ATTRv 29 …”

Section: Echocardiographymentioning

confidence: 87%

“…17 A RV apical ratio >0.8 and LV apical ratio >0.96 showed high accuracy in the differentiation between AL and ATTR. 13 On the other hand, LA reservoir and pump function on strain imaging are lower in ATTRwt compared to AL and ATTRv. 29 Many of these parameters can also be useful for prognostic assessment.…”

Section: Advanced Echocardiographymentioning

confidence: 99%

“…10 When both markers are increased it indicate more severe cardiac involvement, but also poorer outcome, and current staging systems and prognostic classification models for risk stratification use both serum levels. [10][11][12][13] NTproBNP is also more sensitive than conventional echocardiographic parameters in detecting clinical improvement or worsening during amyloid cardiomyopathy follow-up, being particularly useful for monitoring treatment response, both in ATTR CA and AL CA. 9,13 2 | ECHOCARDIOGRAPHY Transthoracic echocardiography (TTE) has widespread availability and portability (including bedside), low cost, and it does not require ionizing radiation.…”

Section: Electrocardiogram and Laboratory Testingmentioning

confidence: 99%

“…[10][11][12][13] NTproBNP is also more sensitive than conventional echocardiographic parameters in detecting clinical improvement or worsening during amyloid cardiomyopathy follow-up, being particularly useful for monitoring treatment response, both in ATTR CA and AL CA. 9,13 2 | ECHOCARDIOGRAPHY Transthoracic echocardiography (TTE) has widespread availability and portability (including bedside), low cost, and it does not require ionizing radiation. It is, for those reasons, the fastest and safest imaging technique.…”

Section: Electrocardiogram and Laboratory Testingmentioning

confidence: 99%

“…Cardiac troponin elevations are caused by myocardial injury related with toxicity of amyloid fibrils and increases in NTproBNP are linked to increased cardiac wall stress associated with amyloid infiltration 10 . When both markers are increased it indicate more severe cardiac involvement, but also poorer outcome, and current staging systems and prognostic classification models for risk stratification use both serum levels 10–13 . NTproBNP is also more sensitive than conventional echocardiographic parameters in detecting clinical improvement or worsening during amyloid cardiomyopathy follow‐up, being particularly useful for monitoring treatment response, both in ATTR CA and AL CA 9,13 …”

Section: Introductionmentioning

confidence: 99%

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Multimodality imaging in cardiac amyloidosis: State‐of‐the‐art review

Timóteo

Rosa

Bras

et al. 2022

J of Clinical Ultrasound

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Amyloidosis is a systemic disease, characterized by deposition of amyloid fibrils in various organs, including the heart. For the diagnosis of cardiac amyloidosis (CA) it is required a high level of clinical suspicion and in the presence of clinical, laboratorial, and electrocardiographic red flags, a comprehensive multimodality imaging evaluation is warranted, including echocardiography, magnetic resonance, scintigraphy, and computed tomography, that will confirm diagnosis and define the CA subtype, which is of the utmost importance to plan a treatment strategy. We will review the use of multimodality imaging in the evaluation of CA, including the latest applications, and a practical flow‐chart will sum‐up this evidence.

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Section: Echocardiographymentioning

confidence: 87%

Section: Advanced Echocardiographymentioning

confidence: 99%

Section: Electrocardiogram and Laboratory Testingmentioning

confidence: 99%

Section: Electrocardiogram and Laboratory Testingmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Multimodality imaging in cardiac amyloidosis: State‐of‐the‐art review

Timóteo

Rosa

Bras

et al. 2022

J of Clinical Ultrasound

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The short and long‐term characteristics and outcomes of patients with grade 1 myocardial uptake on cardiac scintigraphy

et al. 2023

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Aims This study aimed to characterize the final diagnosis and prognosis of patients with grade 1 myocardial scintigraphy uptake, which is an unequivocal result for the diagnosis of transthyretin cardiac amyloidosis (ATTR‐CA) requiring further invasive investigation with tissue biopsy. Methods and results We retrospectively compared the clinical and imaging parameters of patients suspected for ATTR‐CA (based on clinical and echocardiographic parameters) with grade 1 vs. grades 2/3 technetium pyrophosphate uptake on cardiac scintigraphy. Prospectively, grade 1 patients underwent re‐evaluation for ATTR‐CA at long term. Of the 132 ATTR‐CA suspected patients, 89 (67%) were diagnosed as grade 1 and 43 (33%) as grades 2/3 uptake. Grade 1 vs. grades 2/3 patients were younger and female predominant with lower biomarker levels and left ventricular mass. Based on available imaging and pathology findings, only 6 out of the 89 patients with grade 1 uptake (7%) were finally diagnosed with light‐chain cardiac amyloidosis, whereas no patient was diagnosed with ATTR‐CA. At 2 [interquartile range (IQR) 0.75, 3.25] years of follow‐up, the survival of patients with grade 1 vs. grades 2/3 uptake was significantly better [hazard ratio 0.271 (95% confidence interval 0.130 to 0.563, P = 0.0005)]. Prospectively, 30 patients with grade 1 uptake were re‐evaluated at a median follow‐up of 3.2 (IQR 2.2, 3.9) years. Their New York Heart Association class, biomarker levels, and echocardiography findings remained stable. No patient (0/25) demonstrated grades 2/3 uptake at repeated long‐term scintigraphy. Conclusions Patients with suspected ATTR‐CA and a grade 1 scintigraphy uptake demonstrate a stable clinical, laboratory, imaging, and scintigraphy phenotype along with a benign survival profile at long‐term follow‐up. Larger studies should define the optimal evaluation strategy in this population.

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Early diagnosis, disease stage and prognosis in wild‐type transthyretin amyloid cardiomyopathy: The DIAMOND study

Tini,

Musumeci,

Milani

et al. 2024

ESC Heart Failure

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AimsDisease staging and prognostic scoring in wild‐type transthyretin‐related cardiac amyloidosis (ATTRwt‐CA) can be captured by two systems (NAC and Columbia scores). However, uncertainty remains as epidemiology of the disease is evolving rapidly. We evaluated features associated with staging systems across ATTRwt‐CA patients from different diagnostic pathways, and their association with prognosis.MethodsWe performed an analysis on DIAMOND patients with available data to evaluate NAC and Columbia score. DIAMOND was a retrospective study from 17 Italian referral centres for CA, enrolling 1281 patients diagnosed between 2016 and 2021, and aimed at describing characteristics of pathways leading to ATTRwt‐CA diagnosis. Of the original cohort, 811 patients were included in this analysis. Each patient had NAC and Columbia score calculated. Patients were grouped according to NAC and Columbia scoring classes. We described characteristics of patients according to staging classes and diagnostic pathways at diagnosis. Prevalence of early diagnoses, defined as NAC Ia, NYHA class I, no use of diuretics, no history of heart failure (HF) hospitalizations nor of atrial fibrillation prior to diagnosis, was investigated. Finally, prognostic variables were tested alone and grouped as NAC or Columbia scores in Cox univariate and multivariate regression analyses. Prognosis was investigated as all‐cause mortality, in the whole population and dividing patients in HF versus other diagnostic pathways.ResultsOnly 1% of the study population had an early ATTRwt‐CA diagnosis. Distribution of prognostic variables and of NAC and Columbia classes was heterogeneous across diagnostic pathways. The prevalence of NAC III and Columbia III was higher in the HF diagnostic pathway, but all NAC and Columbia classes were present in all pathways. Both NAC and Columbia scores were associated with all‐cause mortality at univariate Cox regression analysis in the whole population, in patients from the HF diagnostic pathway and in those from other pathways. At multivariate analysis, Columbia score remained significantly associated with the outcome, together with age at diagnosis, left ventricular ejection fraction and maximal wall thickness.ConclusionsIn this contemporary nationwide cohort, an ATTRwt‐CA early diagnosis was very rare. Disease staging with NAC and Columbia scoring systems determined classes of patients with heterogeneous features. Both scores were significantly associated with mortality, but other variables also had prognostic significance.

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Characteristics and natural history of early-stage cardiac transthyretin amyloidosis

Cited by 39 publications

References 24 publications

Multimodality imaging in cardiac amyloidosis: State‐of‐the‐art review

Multimodality imaging in cardiac amyloidosis: State‐of‐the‐art review

The short and long‐term characteristics and outcomes of patients with grade 1 myocardial uptake on cardiac scintigraphy

Early diagnosis, disease stage and prognosis in wild‐type transthyretin amyloid cardiomyopathy: The DIAMOND study

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