Publications by authors named "Claudio Rapezzi"

300 Publications

Diagnosis and treatment of cardiac amyloidosis. A position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases.

Eur J Heart Fail 2021 Apr 7. Epub 2021 Apr 7.

2nd Department of Medicine, Department of Cardiovascular Medicine, First Faculty of Medicine, Charles University, General University Hospital, Prague, Czech Republic.

Cardiac amyloidosis is a serious and progressive infiltrative disease that is caused by the deposition of amyloid fibrils at the cardiac level. It can be due to rare genetic variants in the hereditary forms or as a consequence of acquired conditions. Thanks to advances in imaging techniques and the possibility of achieving a non-invasive diagnosis, we now know that cardiac amyloidosis is a more frequent disease than traditionally considered. In this position paper the Working Group on Myocardial and Pericardial Disease proposes an invasive and non-invasive definition of cardiac amyloidosis, addresses clinical scenarios and situations to suspect the condition and proposes a diagnostic algorithm to aid diagnosis. Furthermore, we also review how to monitor and treat cardiac amyloidosis, in an attempt to bridge the gap between the latest advances in the field and clinical practice.
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http://dx.doi.org/10.1002/ejhf.2140DOI Listing
April 2021

Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC Working Group on Myocardial and Pericardial Diseases.

Eur Heart J 2021 Apr 7. Epub 2021 Apr 7.

2nd Department of Medicine, Department of Cardiovascular Medicine, First Faculty of Medicine, Charles University, General University Hospital, Prague, Czech Republic.

Cardiac amyloidosis is a serious and progressive infiltrative disease that is caused by the deposition of amyloid fibrils at the cardiac level. It can be due to rare genetic variants in the hereditary forms or as a consequence of acquired conditions. Thanks to advances in imaging techniques and the possibility of achieving a non-invasive diagnosis, we now know that cardiac amyloidosis is a more frequent disease than traditionally considered. In this position paper the Working Group on Myocardial and Pericardial Disease proposes an invasive and non-invasive definition of cardiac amyloidosis, addresses clinical scenarios and situations to suspect the condition and proposes a diagnostic algorithm to aid diagnosis. Furthermore, we also review how to monitor and treat cardiac amyloidosis, in an attempt to bridge the gap between the latest advances in the field and clinical practice.
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http://dx.doi.org/10.1093/eurheartj/ehab072DOI Listing
April 2021

The impossible interviews-Sherlock Holmes interviews David Sackett: 'how much can we trust the guidelines?'

Eur Heart J 2021 Apr 2. Epub 2021 Apr 2.

Centro Cardiologico Universitario di Ferrara, Department of Translational Medicine, University of Ferrara, Azienda Ospedaliero Universitaria, via Aldo Moro 8, 44124 Cona (Ferrara), Italy.

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http://dx.doi.org/10.1093/eurheartj/ehab187DOI Listing
April 2021

Coronary and total thoracic calcium scores predict mortality and provides pathophysiologic insights in COVID-19 patients.

J Cardiovasc Comput Tomogr 2021 Mar 11. Epub 2021 Mar 11.

IRCCS San Raffaele Scientific Institute, Italy; Vita-Salute San Raffaele University, Italy.

Background: Coronavirus disease 2019 (COVID-19) has spread worldwide determining dramatic impacts on healthcare systems. Early identification of high-risk parameters is required in order to provide the best therapeutic approach. Coronary, thoracic aorta and aortic valve calcium can be measured from a non-gated chest computer tomography (CT) and are validated predictors of cardiovascular events and all-cause mortality. However, their prognostic role in acute systemic inflammatory diseases, such as COVID-19, has not been investigated.

Objectives: The aim was to evaluate the association of coronary artery calcium and total thoracic calcium on in-hospital mortality in COVID-19 patients.

Methods: 1093 consecutive patients from 16 Italian hospitals with a positive swab for COVID-19 and an admission chest CT for pneumonia severity assessment were included. At CT, coronary, aortic valve and thoracic aorta calcium were qualitatively and quantitatively evaluated separately and combined together (total thoracic calcium) by a central Core-lab blinded to patients' outcomes.

Results: Non-survivors compared to survivors had higher coronary artery [Agatston (467.76 ​± ​570.92 vs 206.80 ​± ​424.13 ​mm, p ​< ​0.001); Volume (487.79 ​± ​565.34 vs 207.77 ​± ​406.81, p ​< ​0.001)], aortic valve [Volume (322.45 ​± ​390.90 vs 98.27 ​± ​250.74 mm, p ​< ​0.001; Agatston 337.38 ​± ​414.97 vs 111.70 ​± ​282.15, p ​< ​0.001)] and thoracic aorta [Volume (3786.71 ​± ​4225.57 vs 1487.63 ​± ​2973.19 mm, p ​< ​0.001); Agatston (4688.82 ​± ​5363.72 vs 1834.90 ​± ​3761.25, p ​< ​0.001)] calcium values. Coronary artery calcium (HR 1.308; 95% CI, 1.046-1.637, p ​= ​0.019) and total thoracic calcium (HR 1.975; 95% CI, 1.200-3.251, p ​= ​0.007) resulted to be independent predictors of in-hospital mortality.

Conclusion: Coronary, aortic valve and thoracic aortic calcium assessment on admission non-gated CT permits to stratify the COVID-19 patients in-hospital mortality risk.
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http://dx.doi.org/10.1016/j.jcct.2021.03.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7946543PMC
March 2021

Performance of Synthetic Extracellular Volume Fraction in Different Cardiac Phenotypes From a Prospective Cohort of Patients Referred for Cardiac Magnetic Resonance.

J Magn Reson Imaging 2021 Feb 15. Epub 2021 Feb 15.

GVM Care & Research, Maria Cecilia Hospital, Cotignola, Ravenna, Italy.

Background: A synthetic myocardial extracellular volume fraction (sECV) can be obtained without blood hematocrit (Hct) by using the linear relationship between Hct and the longitudinal relaxation time of blood. Concerns have been raised about the widespread clinical application of this approach.

Purpose: To assess the relationship between measured ECV (m-ECV) and sECV, using both a published model and a locally derived one.

Study Type: Single-center, prospective.

Field Strength/sequence: A 1.5 T/modified Look Locker (MOLLI) sequence.

Subjects: Fifty-two healthy subjects and 113 patients (76 with and 37 without a hypertrophic cardiac phenotype).

Assessment: Three ECV values were obtained for each patient: 1) measured ECV (m-ECV), using Hct from a venous blood sample; 2) Fent-synthetic ECV (F-sECV), using the equation proposed by Fent et al; and 3) Local-synthetic ECV (L-sECV), using the equation obtained from a local derivation cohort comprising 83 subjects.

Statistical Tests: Shapiro-Wilk test, analysis of variance, Kruskal Wallis test, Pearson correlation, Bland-Altman analysis, univariate and multivariable regression analysis.

Results: In the validation cohort (N = 82), Bland-Altmann analysis revealed an excellent agreement between m-ECV and L-sECV with a statistically insignificant bias (-0.1%, limits of agreement: -2.8% and 2.6%; P = 0.528), while in the overall population (N = 165), the mean bias between m-ECV and F-sECV was small but significant (1.2%, limits of agreement: -1.5% and 3.9%, P < 0.05). F-sECV bias was significantly higher for measured Hct (m-Hct) values <0.372 (2.3% vs. 1.0%, P < 0.05). Among the phenotype subgroups, amyloidotic patients showed a higher bias compared to others, both with F-sECV and L-sECV (2.3% vs. 1.1%, P < 0.05 and 1.1% vs. 0.2%, P < 0.05, respectively).

Data Conclusion: Although synthetic ECV performs well in an external cohort, the use of a local formula further improves the accuracy of ECV estimate over a broad spectrum of cardiac phenotypes. Local sECV performs better for a wider range of Hct values, compared to the published model. Amyloidosis is the only group associated with a lower accuracy.

Level Of Evidence: 5 TECHNICAL EFFICACY: Stage 2.
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http://dx.doi.org/10.1002/jmri.27556DOI Listing
February 2021

Standard ECG for differential diagnosis between Anderson-Fabry disease and hypertrophic cardiomyopathy.

Heart 2021 Feb 9. Epub 2021 Feb 9.

Cardiology Unit, IRCCS, Department of Experimental, Diagnostic and Specialty Medicine, Sant'Orsola Hospital, University of Bologna, Bologna, Emilia-Romagna, Italy

Objectives: To evaluate the role of the ECG in the differential diagnosis between Anderson-Fabry disease (AFD) and hypertrophic cardiomyopathy (HCM).

Methods: In this multicentre retrospective study, 111 AFD patients with left ventricular hypertrophy were compared with 111 patients with HCM, matched for sex, age and maximal wall thickness by propensity score. Independent ECG predictors of AFD were identified by multivariate analysis, and a multiparametric ECG score-based algorithm for differential diagnosis was developed.

Results: Short PR interval, prolonged QRS duration, right bundle branch block (RBBB), R in augmented vector left (aVL) ≥1.1 mV and inferior ST depression independently predicted AFD diagnosis. A point-by-point ECG score was then derived with the following diagnostic performances: c-statistic 0.80 (95% CI 0.74 to 0.86) for discrimination, the Hosmel-Lemeshow χ 6.14 (p=0.189) for calibration, sensitivity 69%, specificity 84%, positive predictive value 82% and negative predictive value 72%. After bootstrap resampling, the mean optimism was 0.025, and the internal validated c-statistic for the score was 0.78.

Conclusions: Standard ECG can help to differentiate AFD from HCM while investigating unexplained left ventricular hypertrophy. Short PR interval, prolonged QRS duration, RBBB, R in aVL ≥1.1 mV and inferior ST depression independently predicted AFD. Their systematic evaluation and the integration in a multiparametric ECG score can support AFD diagnosis.
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http://dx.doi.org/10.1136/heartjnl-2020-318271DOI Listing
February 2021

Use of biomarkers to diagnose and manage cardiac amyloidosis.

Eur J Heart Fail 2021 Feb 21;23(2):217-230. Epub 2021 Feb 21.

Institute of Life Sciences, Scuola Superiore Sant'Anna, Pisa, Italy.

Amyloidoses are characterized by the tissue accumulation of misfolded proteins into insoluble fibrils. The two most common types of systemic amyloidosis result from the deposition of immunoglobulin light chains (AL) and wild-type or variant transthyretin (ATTRwt/ATTRv). Cardiac involvement is the main determinant of outcome in both AL and ATTR, and cardiac amyloidosis (CA) is increasingly recognized as a cause of heart failure. In CA, circulating biomarkers are important diagnostic tools, allow to refine risk stratification at baseline and during follow-up, help to tailor the therapeutic strategy and monitor the response to treatment. Among amyloid precursors, free light chains are established biomarkers in AL amyloidosis, while the plasma transthyretin assay is currently being investigated as a tool for supporting the diagnosis of ATTRv amyloidosis, predicting outcome and monitor response to novel tetramer stabilizers or small interfering RNA drugs in ATTR CA. Natriuretic peptides (NPs) and troponins are consistently elevated in patients with AL and ATTR CA. Plasma NPs, troponins and free light chains hold prognostic significance in AL amyloidosis, and are evaluated for therapy decision-making and follow-up, while the value of NPs and troponins in ATTR is less well established. Biomarkers can be usefully integrated with clinical and imaging variables at all levels of the clinical algorithm of systemic amyloidosis, from screening to diagnosis and prognosis, and treatment tailoring.
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http://dx.doi.org/10.1002/ejhf.2113DOI Listing
February 2021

Atrial Flutter in Patient With Critical COVID-19: Beneficial Effects of Rhythm Control on Respiratory Distress.

JACC Case Rep 2021 Jan 6;3(1):162-164. Epub 2021 Jan 6.

Cardiological Center, University of Ferrara, S. Anna Hospital, Ferrara, Italy.

We report the case of a patient critically ill with coronavirus disease-2019 (COVID-19) in which atrial flutter with high ventricular response rate occurred, contributing to worsening of the respiratory distress. After failure of noninvasive rate and rhythm control strategies, successful transcatheter ablation was performed and the respiratory distress of the patient improved. ().
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http://dx.doi.org/10.1016/j.jaccas.2020.11.015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7836416PMC
January 2021

Myocarditis in COVID-19 patients: current problems.

Intern Emerg Med 2021 Jan 23. Epub 2021 Jan 23.

Cardiology Center, University of Ferrara, Viale Aldo Moro 8, 44024, Cona, Ferrara, Italy.

Myocarditis has been reported as a possible clinical presentation or complication in patients with coronavirus disease (COVID)-19 due to SARS-CoV-2. Despite the alarm that this possibility generated among physicians, there is paucity of information about mechanisms, prevalence, prognosis, diagnosis and therapy of myocarditis in the context of COVID-19. This brief review has the goal to revise and summarize current knowledge on myocarditis in COVID-19 patients and underline problems especially related to diagnosis and treatment.
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http://dx.doi.org/10.1007/s11739-021-02635-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7823176PMC
January 2021

[EXPLORER-HCM: mavacamten for the treatment of symptomatic obstructive hypertrophic cardiomyopathy].

Authors:
Claudio Rapezzi

G Ital Cardiol (Rome) 2021 Jan;22(1):30-32

Università degli Studi di Ferrara e Maria Cecilia Hospital, GVM Care & Research, Cotignola (RA).

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http://dx.doi.org/10.1714/3502.34878DOI Listing
January 2021

Tafamidis is entering the clinical arena for the treatment of transthyretin-related cardiomyopathy: certainties and unmet needs.

Eur J Heart Fail 2021 Feb 26;23(2):286-289. Epub 2021 Jan 26.

Institute of Life Sciences, Scuola Superiore Sant'Anna, Pisa, Italy.

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http://dx.doi.org/10.1002/ejhf.2104DOI Listing
February 2021

Efficacy of Tafamidis in Patients With Hereditary and Wild-Type Transthyretin Amyloid Cardiomyopathy: Further Analyses From ATTR-ACT.

JACC Heart Fail 2021 Feb 9;9(2):115-123. Epub 2020 Dec 9.

Columbia University College of Physicians and Surgeons, New York, New York, USA.

Objectives: Tafamidis is an effective treatment for transthyretin amyloid cardiomyopathy (ATTR-CM), this study aimed to determine whether there is a differential effect between variant transthyretin amyloidosis (ATTRv) and wild-type transthyretin (ATTRwt).

Background: ATTR-CM is a progressive, fatal disorder resulting from mutations in the ATTRv or the deposition of denatured ATTRwt.

Methods: In pre-specified analyses from ATTR-ACT (Tafamidis in Transthyretin Cardiomyopathy Clinical Trial), baseline characteristics, all-cause mortality, and change from baseline to month 30 in 6-min walk test distance and Kansas City Cardiomyopathy Questionnaire Overall Summary score were compared in patients with ATTRwt and ATTRv.

Results: There were 335 patients with ATTRwt (201 tafamidis, 134 placebo) and 106 with ATTRv (63 tafamidis, 43 placebo) enrolled in ATTR-ACT. Patients with ATTRwt (vs. ATTRv) had less advanced disease at baseline and a lower rate of disease progression over the study. The reduction in all-cause mortality with tafamidis compared with placebo was not different between ATTRwt (hazard ratio: 0.706 [95% confidence interval (CI): 0.474 to 1.052]; p = 0.0875) and ATTRv (hazard ratio: 0.690 [95% CI: 0.408 to 1.167]; p = 0.1667). Tafamidis was associated with a similar reduction (vs. placebo) in the decline in 6-min walk test distance in ATTRwt (mean ± SE difference from placebo, 77.14 ± 10.78; p < 0.0001) and ATTRv (79.61 ± 29.83 m; p = 0.008); and Kansas City Cardiomyopathy Questionnaire Overall Summary score in ATTRwt (12.72 ± 2.10; p < 0.0001) and ATTRv (18.18 ± 7.75; p = 0.019).

Conclusions: Pre-specified analyses from ATTR-ACT confirm the poor prognosis of untreated ATTRv-related cardiomyopathy compared with ATTRwt, but show the reduction in mortality and functional decline with tafamidis treatment is similar in both disease subtypes. (Safety and Efficacy of Tafamidis in Patients With Transthyretin Cardiomyopathy [ATTR-ACT]; NCT01994889).
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http://dx.doi.org/10.1016/j.jchf.2020.09.011DOI Listing
February 2021

Diastolic dysfunction, frailty and prognosis in elderly patients with acute coronary syndromes.

Int J Cardiol 2021 Mar 30;327:31-35. Epub 2020 Nov 30.

UO Cardiologia, Azienda Ospedaliero-Universitaria di Ferrara, Ferrara, Italy.

Background To investigate the relationship between 1-year outcome and diastolic dysfunction (DD) and frailty and/or physical performance (PP) in older adults admitted to hospital for acute coronary syndrome (ACS). Methods and results Older (age ≥ 70 years) hospitalized for ACS and receiving coronary artery angiography ± percutaneous coronary intervention were included. Before discharge a complete transthoracic echocardiogram (TTE) was performed with the assessment of DD, following the 2016 American Society of Echocardiography/European Association of Cardiovascular Imaging algorithm. Seven different scales of frailty and PP were assessed. The relationship between DD and tests of frailty and PP was investigated, as well as the association with the 1-year occurrence of all-cause death or re-hospitalization. Overall, 329 patients were included in the analysis. Patients were stratified in two groups: DD grade 0-1 versus 2-3. Those with undetermined degree of DD have been excluded by the analysis (n = 106). Mean age of the groups was 77 ± 5 vs 79 ± 6 years, respectively. Scales of frailty and/or PP were significantly poor in patients with DD grade 2-3 compared to the others. After multivariate Cox regression (considering age, female sex, haemoglobin, albumin, clinical presentation, LVEF and SPPB) DD (degree 2-3 vs. 0-1) emerged as an independent predictor of the composite endpoint (HR 1.69, 95%CI 1.04-2.75, p = 0.033). This was mainly driven by 1-year re-hospitalization (HR 2.01, 95%CI 1.22-3.27, p < 0.001). Conclusions In older ACS patients the assessment of DD is related to parameters of frailty and PP and it is an independent predictor of 1-year outcome.
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http://dx.doi.org/10.1016/j.ijcard.2020.11.056DOI Listing
March 2021

How far should guidelines be followed?

Eur Heart J Suppl 2020 Nov 18;22(Suppl L):L121-L123. Epub 2020 Nov 18.

Barts Heart Centre and Institute of Cardiovascular Science, University College London, London, UK.

Clinical guidelines irreparably characterize contemporary medicine. Referring to guidelines has become routine in both medical literature and daily clinical activity, with the risk of becoming the only-or at least the main-inspiring element of the physician's behaviour. This would lead to the mortification of clinical reasoning, a term that is synonymous with an individualized approach, focused on the single patient, and not on a population.
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http://dx.doi.org/10.1093/eurheartj/suaa150DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7673602PMC
November 2020

[ANMCO/SIC Consensus document on the management of myocarditis].

G Ital Cardiol (Rome) 2020 Dec;21(12):969-989

S.C. Cardiologia, Dipartimento Cardiotoracovascolare, Azienda Sanitaria Universitaria Giuliano Isontina-ASUGI, Università di Trieste.

Myocarditis is an inflammatory heart disease that can occur acutely, as in acute myocarditis, or persistently, as in chronic myocarditis or chronic inflammatory cardiomyopathy. Different agents can induce myocarditis, with viruses being the most common triggers. Generally, acute myocarditis affects relatively young people and men more than women. Myocarditis has a broad spectrum of clinical presentations and evolution trajectories, although most cases resolve spontaneously. Patients with reduced left ventricular ejection fraction, heart failure symptoms, advanced atrioventricular block, sustained ventricular arrhythmias or cardiogenic shock (the latter known as fulminant myocarditis) are at increased risk for death and heart transplantation. The presentation of chronic inflammatory cardiomyopathy may be more subtle, with progressive symptoms of heart failure or appearance of rhythm disturbance, not rarely preceded by an infective episode. Autoimmune disorder or systemic inflammatory conditions can be another significant predisposing substrate of myocarditis, especially in women. Emerging causes of myocarditis are drug-related like the new anticancer therapies, the immune checkpoint inhibitors. In this Italian Association of Hospital Cardiologists (ANMCO) and Italian Society of Cardiology (SIC) expert consensus document on myocarditis, we propose diagnostic strategies for identifying possible causes of the disease and factors associated with increased risk. Finally, we propose potential treatments and when referring patients to tertiary centers, especially for high-risk patients. Even if endomyocardial biopsy is the invasive diagnostic tool for making definitive diagnosis and differentiation of histological subtypes (i.e., lymphocytic vs eosinophilic vs giant cell myocarditis), it is not always readily available in all centers. Thus, we propose when this exam is mandatory or when it can be postponed or substituted by cardiac magnetic resonance imaging. This document reflects the Italian perspective on managing patients with myocarditis and their follow-up, considering also current US and European scientific position statements.
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http://dx.doi.org/10.1714/3472.34551DOI Listing
December 2020

[Diagnosis and treatment of cardiomyopathies: a pradigm shift and an evolutionary step forward in Cardiology].

G Ital Cardiol (Rome) 2020 Dec;21(12):923-925

Centro Cardiologico Universitario, Università degli Studi, Ferrara.

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http://dx.doi.org/10.1714/3472.34546DOI Listing
December 2020

Aortic stenosis, transcatheter aortic valve replacement and transthyretin cardiac amyloidosis: are we progressively unraveling the tangle?

Eur J Heart Fail 2021 Feb 25;23(2):259-263. Epub 2020 Nov 25.

Cardiovascular Center, University of Ferrara, Ferrara, Italy.

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http://dx.doi.org/10.1002/ejhf.2057DOI Listing
February 2021

'Corona' versus 'coronary'.

Eur Heart J 2021 Feb;42(6):555-557

Maria Cecilia Hospital, Cotignola (RA), Italy.

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http://dx.doi.org/10.1093/eurheartj/ehaa897DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7717209PMC
February 2021

Adapting to survive.

Eur Heart J 2020 11;41(41):3981-3983

Maria Cecilia Hospital, GVM Care&Research, Cotignola, Italy.

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http://dx.doi.org/10.1093/eurheartj/ehaa675DOI Listing
November 2020

Transcatheter Mitral Valve Repair in Cardiogenic Shock and Mitral Regurgitation: A Patient-Level, Multicenter Analysis.

JACC Cardiovasc Interv 2021 Jan 14;14(1):1-11. Epub 2020 Oct 14.

CAPITAL Research Group, University of Ottawa Heart Institute, Ottawa, Ontario, Canada; Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, Ontario, Canada; Division of Cardiology, University of Ottawa Heart Institute, Ottawa, Ontario, Canada. Electronic address:

Objectives: The aim of this study was to evaluate the outcome of transcatheter mitral valve repair (TMVr) in patients with cardiogenic shock and significant mitral regurgitation (MR).

Background: Patients in cardiogenic shock with severe MR have a poor prognosis in the setting of conventional medical therapy. Because of its favorable safety profile, TMVr is being increasingly used as an acute therapy in this population, though its efficacy remains unknown.

Methods: A multicenter, collaborative, patient-level analysis was conducted. Patients with cardiogenic shock and moderate to severe (3+) or severe (4+) MR who were not surgical candidates were treated with TMVr. The primary outcome was in-hospital mortality. Secondary outcomes included 90-day mortality, heart failure (HF) hospitalization, and the combined event rate of 90-day mortality and HF hospitalization following dichotomization by TMVr device success.

Results: Between January 2011 and February 2019, 141 patients across 14 institutions met the inclusion criteria. In-hospital mortality occurred in 22 patients (15.6%), at 90 days in 38 patients (29.5%), and at one year in 55 patients (42.6%). Median length of hospital stay following TMVr was 10 days (interquartile range: 6 to 20 days). HF hospitalization occurred in 26 patients (18.4%) at a median of 73 days (interquartile range: 26 to 546 days). When stratified by TMVr procedural results, successful TMVr reduced rates of in-hospital mortality (hazard ratio [HR]: 0.36; 95% confidence interval [CI]: 0.13 to 0.98; p = 0.04), 90-day mortality (HR: 0.36; 95% CI: 0.16 to 0.78; p = 0.01), and the composite of 90-day mortality and HF hospitalization (HR: 0.41; 95% CI: 0.19 to 0.90; p = 0.03).

Conclusions: TMVr may improve short- and intermediate-term mortality in high-risk patients with cardiogenic shock and moderate to severe MR. Randomized studies are needed to definitively establish MR as a therapeutic target in patients with cardiogenic shock.
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http://dx.doi.org/10.1016/j.jcin.2020.08.037DOI Listing
January 2021

Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery.

Eur J Intern Med 2020 12 5;82:7-15. Epub 2020 Oct 5.

Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Italy. Electronic address:

Transthyretin amyloid cardiomyopathy (ATTR-AC) is an under-recognized and underdiagnosed disease. Although traditionally considered a rare condition, the epidemiology of the disease is rapidly changing due to the possibility of non-invasive diagnosis through cardiac scintigraphy with bone tracers and novel disease-modifying treatments providing survival advantages. Nevertheless, many questions and grey areas have to be addressed, such as the natural history of ATTR-AC, the role and implications of genotype-phenotype interactions, the best clinical management, prognostic stratification and the most appropriate treatments, including those already recommended for patients with heart failure. Clinicians have to cope with old beliefs and evolving concepts in ATTR-AC. A wide horizon of possibilities for physicians of many specialties is unfolding and awaits discovery.
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http://dx.doi.org/10.1016/j.ejim.2020.09.025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7534738PMC
December 2020

Arterial thrombo-embolic events in cardiac amyloidosis: a look beyond atrial fibrillation.

Amyloid 2021 Mar 28;28(1):12-18. Epub 2020 Sep 28.

Cardiology, Clinical and Molecular Medicine Department, Sapienza University of Rome, Rome, Italy.

Background: Intracardiac thrombosis is reported to occur frequently in cardiac amyloidosis (CA). However, data regarding arterial thrombo-embolic events (AEs) in CA are limited. We aimed at assessing prevalence, clinical characteristics and predictors of AEs in a large multicentric CA cohort.

Methods And Results: Four-hundred-six consecutive CA patients (134 AL, 73 ATTRm and 199 ATTRwt) from 5 Italian referral centres were retrospectively evaluated and followed-up for a median time of 19 months. Thirty-one patients (7.6%) suffered from an AE, of whom 10 (32.2%) were in sinus rhythm and had no history of AF. There were no significant differences in terms of age, gender and type of CA between patients with or without AEs. Fourteen (7.6%) of 185 patients on anticoagulation had an AE despite therapy. Anticoagulation therapy did not appear to fully protect from the risk of events (HR 1.23, 95%CI 0.52-2.92,  = .64). The only predictor of AEs, in particular among CA patients in sinus rhythm, was a CHA2DS2-VASC score ≥ 3 (HR 2.84, 95%CI 1.02-7.92,  = .05 in overall population; HR 10.13, 95%CI 1.12-91.19,  = .04 in patients in sinus rhythm).

Conclusions: In our large, multicentric, real-world cohort, prevalence and incidence rate of AEs was high. A consistent proportion of events occurred despite anticoagulation therapy or in patients in sinus rhythm. A higher CHA2DS2-VASc score might identify patients at risk of AEs also among those in sinus rhythm.
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http://dx.doi.org/10.1080/13506129.2020.1798922DOI Listing
March 2021

Avoiding misdiagnosis: expert consensus recommendations for the suspicion and diagnosis of transthyretin amyloidosis for the general practitioner.

BMC Fam Pract 2020 09 23;21(1):198. Epub 2020 Sep 23.

Amyloidosis Research and Treatment Center Foundation, IRCCS Policlinico San Matteo, San Matteo, Italy.

Background: Transthyretin amyloidosis (also known as ATTR amyloidosis) is a systemic, life-threatening disease characterized by transthyretin (TTR) fibril deposition in organs and tissue. A definitive diagnosis of ATTR amyloidosis is often a challenge, in large part because of its heterogeneous presentation. Although ATTR amyloidosis was previously considered untreatable, disease-modifying therapies for the treatment of this disease have recently become available. This article aims to raise awareness of the initial symptoms of ATTR amyloidosis among general practitioners to facilitate identification of a patient with suspicious signs and symptoms.

Methods: These consensus recommendations for the suspicion and diagnosis of ATTR amyloidosis were developed through a series of development and review cycles by an international working group comprising key amyloidosis specialists. This working group met to discuss the barriers to early and accurate diagnosis of ATTR amyloidosis and develop a consensus recommendation through a thorough search of the literature performed using PubMed Central.

Results: The cardiac and peripheral nervous systems are most frequently involved in ATTR amyloidosis; however, many patients often also experience gastrointestinal and other systemic manifestations. Given the multisystemic nature of symptoms, ATTR amyloidosis is often misdiagnosed as a more common disorder, leading to significant delays in the initiation of treatment. Although histologic evaluation has been the gold standard to confirm ATTR amyloidosis, a range of tools are available that can facilitate early and accurate diagnosis. Of importance, genetic testing should be considered early in the evaluation of a patient with unexplained peripheral neuropathy.

Conclusions: A diagnostic algorithm based on initial red flag symptoms and manifestations of cardiac or neurologic involvement will facilitate identification by the general practitioner of a patient with clinically suspicious symptoms, enabling subsequent referral of the patient to a multidisciplinary specialized medical center.
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http://dx.doi.org/10.1186/s12875-020-01252-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7513485PMC
September 2020

Electrocardiographic features of 431 consecutive, critically ill COVID-19 patients: an insight into the mechanisms of cardiac involvement.

Europace 2020 12;22(12):1848-1854

Cardiological Center, University of Ferrara, Italy.

Aims: Our aim was to describe the electrocardiographic features of critical COVID-19 patients.

Methods And Results: We carried out a multicentric, cross-sectional, retrospective analysis of 431 consecutive COVID-19 patients hospitalized between 10 March and 14 April 2020 who died or were treated with invasive mechanical ventilation. This project is registered on ClinicalTrials.gov (identifier: NCT04367129). Standard ECG was recorded at hospital admission. ECG was abnormal in 93% of the patients. Atrial fibrillation/flutter was detected in 22% of the patients. ECG signs suggesting acute right ventricular pressure overload (RVPO) were detected in 30% of the patients. In particular, 43 (10%) patients had the S1Q3T3 pattern, 38 (9%) had incomplete right bundle branch block (RBBB), and 49 (11%) had complete RBBB. ECG signs of acute RVPO were not statistically different between patients with (n = 104) or without (n=327) invasive mechanical ventilation during ECG recording (36% vs. 28%, P = 0.10). Non-specific repolarization abnormalities and low QRS voltage in peripheral leads were present in 176 (41%) and 23 (5%), respectively. In four patients showing ST-segment elevation, acute myocardial infarction was confirmed with coronary angiography. No ST-T abnormalities suggestive of acute myocarditis were detected. In the subgroup of 110 patients where high-sensitivity troponin I was available, ECG features were not statistically different when stratified for above or below the 5 times upper reference limit value.

Conclusions: The ECG is abnormal in almost all critically ill COVID-19 patients and shows a large spectrum of abnormalities, with signs of acute RVPO in 30% of the patients. Rapid and simple identification of these cases with ECG at hospital admission can facilitate classification of the patients and provide pathophysiological insights.
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http://dx.doi.org/10.1093/europace/euaa258DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7543398PMC
December 2020

Current evidence on the diagnostic and prognostic role of native T1 mapping in heart diseases.

Trends Cardiovasc Med 2020 Aug 7. Epub 2020 Aug 7.

Department of Cardiovascular Imaging, Centro Cardiologico Monzino, IRCCS, Milan, Italy.

Tissue characterization represents a prerogative of cardiac magnetic resonance. Beside late gadolinium enhancement, native T1 mapping (nT1m) reveals tissue composition. It could represent a useful tool for example when contrast medium can't be administrated. The present review summarises current evidence about nT1m in main heart diseases.
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http://dx.doi.org/10.1016/j.tcm.2020.08.001DOI Listing
August 2020

A Pathogenic Galactosidase A Mutation Coexisting With an MYBPC3 Mutation in a Female Patient With Hypertrophic Cardiomyopathy.

Can J Cardiol 2020 Sep 14;36(9):1554.e1-1554.e3. Epub 2020 Apr 14.

Azienda Ospedaliero Universitaria - Policlinico di St. Orsola, Cardiology Unit, Cardio-Thoracic-Vascular Department, Bologna, Italy. Electronic address:

The coexistence of GLA (Pro259Ser, c.775C>T) and MYBPC3 (c.1351+2T>C) mutations was found in a female patient with hypertrophic cardiomyopathy. Histology documented abundant vacuolisation with osmiophilic lamellar bodies and positive Gb3 immunohistochemistry. In the presence of a hypertrophic cardiomyopathy phenotype, the systematic search for unusual findings is mandatory to rule out a phenocopy.
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http://dx.doi.org/10.1016/j.cjca.2020.04.008DOI Listing
September 2020

ATTRv amyloidosis Italian Registry: clinical and epidemiological data.

Amyloid 2020 Dec 22;27(4):259-265. Epub 2020 Jul 22.

Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.

Introduction: ATTRv amyloidosis is worldwide spread with endemic foci in Portugal and Sweden, Japan, Brazil, Maiorca, and Cyprus. A national Registry was developed to characterise the epidemiology and genotype-phenotype correlation of ATTRv amyloidosis in Italy and to allow a better planning of diagnostic and therapeutic services.

Methods: Fifteen Italian referral centres for amyloidosis spread all over the country have contributed to the Registry.

Results: Four-hundred-forty-seven subjects were enrolled, 187 asymptomatic carriers and 260 affected patients. Thirty-one different mutations were recorded. The seven most represented genetic variants were significantly different in terms of age at onset, clinical features and geographical distribution. National prevalence is 4.33/million with higher values in Southern Italy. Overall symptoms of polyneuropathy were present at disease onset in about half of the patients, symptoms of cardiomyopathy in a quarter of patients, the rest referring carpal tunnel syndrome, dysautonomia or lumbar spinal stenosis. 52.6% of patients were in FAP stage 1, 20.4% in stage 2 and 13.5% in stage 3, while 13.5% patients had no neuropathy, presenting only cardiological symptoms.

Conclusions: We presented an epidemiological study based on collaboration among referral centres for ATTRv amyloidosis spread in all the Italian territory, using web-based Registry. It provided a detailed map of the regional distribution of the disease. The increased awareness of the disease among general practitioners and medical specialists has contributed to reduce the diagnostic delay and the rate of misdiagnosis. The Registry will allow to collect also future information about clinical and instrumental follow-up.
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http://dx.doi.org/10.1080/13506129.2020.1794807DOI Listing
December 2020

Safety and efficacy of levosimendan in patients with cardiac amyloidosis.

Eur J Intern Med 2020 10 3;80:114-116. Epub 2020 Jul 3.

Institute of Life Sciences, Scuola Superiore Sant'Anna, Pisa, Italy; Fondazione Toscana Gabriele Monasterio, Pisa, Italy.

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http://dx.doi.org/10.1016/j.ejim.2020.06.037DOI Listing
October 2020