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20 results on '"Sguazzotti, Maurizio"'

1. Clinical and Prognostic Implications of Right Ventricular Uptake on Bone Scintigraphy in Transthyretin Amyloid Cardiomyopathy

Author

Porcari, Aldostefano, Fontana, Marianna, Canepa, Marco, Biagini, Elena, Cappelli, Francesco, Gagliardi, Christian, Longhi, Simone, Pagura, Linda, Tini, Giacomo, Dore, Franca, Bonfiglioli, Rachele, Bauckneht, Matteo, Miceli, Alberto, Girardi, Francesca, Martini, Anna Lisa, Barbati, Giulia, Costanzo, Egidio Natalino, Caponetti, Angelo Giuseppe, Paccagnella, Andrea, Sguazzotti, Maurizio, La Malfa, Giovanni, Zampieri, Mattia, Sciagrà, Roberto, Perfetto, Federico, Rowczenio, Dorota, Gilbertson, Janet, Hutt, David F., Hawkins, Philip N., Rapezzi, Claudio, Merlo, Marco, Sinagra, Gianfranco, and Gillmore, Julian D.

Published
2024
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2. Incidence and predictors of sudden death in patients with cardiac amyloidosis.

Author

de Frutos, Fernando, Saturi, Giulia, Gonzalez-Lopez, Esther, Sguazzotti, Maurizio, Dominguez, Fernando, Ponziani, Alberto, Cabrera-Romero, Eva, Caponetti, Angelo Giuseppe, Lozano, Sara, Massa, Paolo, Peiro-Aventin, Belen, Accietto, Antonella, Mora-Ayestarán, Nerea, Giovannetti, Alessandro, Castro-Urda, Victor, Gagliardi, Christian, Cobo-Marcos, Marta, Rios-Tamayo, Rafael, Biagini, Elena, and Gomez-Bueno, Manuel

Subjects
CARDIAC amyloidosis, CARDIAC arrest, SUDDEN death, UNIVARIATE analysis, AMYLOIDOSIS
Abstract

Introduction: Although sudden death (SD) is a recognized complication of cardiac amyloidosis, there is scarce data about its incidence, mechanisms, and predictors. The aim of this study was to describe incidence of SD and to analyze possible risk factors. Methods: Consecutive patients with ATTR or AL cardiac amyloidosis evaluated at two European centers were identified. SD was defined as unexpected death in clinically stable patients. Cox proportional hazard regression was performed to assess risk factors in univariate analysis. Those statistically significant were then assessed through age-adjusted multivariate analysis. Results: Analysis included 784 patients, 569 with ATTR amyloidosis (mean age 74.1 ± 12.1 years) and 215 with AL amyloidosis (mean age 64.5 ± 10.8 years). After a median follow-up of 1.9 years, SD rate at 2 years was 1.8% in ATTR. Previous pacemaker implantation (PPM) was associated with increased risk after age-adjusted analysis (HR 4.97; 95%CI: 1.39–17.7; p = 0.01). SD rate in AL amyloidosis patients at 2 years was 8.0% after a median follow-up of 1.2 years. Betablockers and NYHA III-IV were independently associated with an increased risk after age-adjusted multivariate analysis (HR 7.06 95%CI (2.31–21.5) p = 0.001) and (HR 4.56 95%CI (1.51–13.8) p = 0.007) respectively. Conclusions: SD is more frequent in AL than in ATTR cardiac amyloidosis. SD is associated with different risk factors in both entities. [ABSTRACT FROM AUTHOR]

Published
2024
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3. Bone Scintigraphy in Cardiac Transthyretin-Related Amyloidosis: A Novel Time-Saving Tool for Semiquantitative Analysis, with Good Potential for Predicting Different Etiologies.

Author

Mattoni, Susanna, Morrone, Maria Francesca, Della Gala, Giuseppe, Prisco, Sonia Elisa, Sguazzotti, Maurizio, Saturi, Giulia, Longhi, Simone, Fanti, Stefano, Bonfiglioli, Rachele, and Strigari, Lidia

Subjects
PEARSON correlation (Statistics), CARDIAC amyloidosis, RADIONUCLIDE imaging, WORKFLOW software, RECEIVER operating characteristic curves
Abstract

(1) Background: The visual and semiquantitative analysis of Technetium-99metastable-3,3-diphospono-1,2-propanodicarboxylic acid (99mTc-DPD) bone scintigraphy is promising for diagnosing cardiac amyloidosis but time-consuming. We validated a faster method, the geometric mean (GM) method with a semi-automated workflow, for heart–whole body (WB) ratio (H/WBr), heart retention (Hr), and WB retention (WBr) calculations compared to the classic method (CM) established in the literature. The capability of semiquantitative scintigraphy indexes to differentiate the etiology in transthyretin-related cardiac amyloidosis (cATTR) patients was investigated. (2) Methods: H/WBr, Hr, and WBr were calculated by extracting counts for WB, kidneys, bladder, and heart on early and late planar image scans and applying background, scan-time, and decay corrections, using CM and GM both on a referring workstation and on a semi-automated workflow in external software. The comparison between CM and GM was assessed with Pearson's correlation, Lin's Concordance Correlation Coefficient (CCC), and Bland–Altman analysis. H/WBr, Hr, and WBr and several clinical variables were used to implement LASSO, Random Forest (RF), and Neural Network (NN) models to predict mutated and wild-type ATTR etiologies. ROC curves and AUC were calculated. (3) Results: Hr, WBr, and H/WBr using CM and GM were highly correlated. Bland–Altman analysis between CM and GM showed biases of 0.12% [CI:0.04%;0.19%] for H/WBr, 0.07% [CI: 0.01%; 0.13%] for Hr, and -0.50% [CI: −1.22%; 0.22%] for WBr. LASSO and NN models had good performance in predicting etiologies with AUC values of 87.3% and 73.6%, respectively. The RF model showed a poorer AUC of 55.8%. (4) Conclusions: The GM in the assisted workflow was validated against the CM. LASSO and NN approaches allowed a good prediction performance to be obtained for patient etiology. [ABSTRACT FROM AUTHOR]

Published
2024
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4. Characterization and natural history of different phenotypes in hereditary transthyretin amyloidosis: 40-year experience at a single Italian referral center

Author

Caponetti, Angelo Giuseppe, primary, Sguazzotti, Maurizio, additional, Accietto, Antonella, additional, Saturi, Giulia, additional, Ponziani, Alberto, additional, Giovannetti, Alessandro, additional, Massa, Paolo, additional, Ruotolo, Irene, additional, Sena, Giuseppe, additional, Zaccaro, Andrea, additional, Parisi, Vanda, additional, Bonfiglioli, Rachele, additional, Guaraldi, Pietro, additional, Gagliardi, Christian, additional, Cortelli, Pietro, additional, Galie, Nazzareno, additional, Biagini, Elena, additional, and Longhi, Simone, additional

Published
2024
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5. Real‐world candidacy to mavacamten in a contemporary hypertrophic obstructive cardiomyopathy population

Author

Bertero, Edoardo, primary, Chiti, Chiara, additional, Schiavo, Maria Alessandra, additional, Tini, Giacomo, additional, Costa, Paolo, additional, Todiere, Giancarlo, additional, Mabritto, Barbara, additional, Dei, Lorenzo‐Lupo, additional, Giannattasio, Alessia, additional, Mariani, Davide, additional, Lofiego, Carla, additional, Santolamazza, Caterina, additional, Monda, Emanuele, additional, Quarta, Giovanni, additional, Barbisan, Davide, additional, Mandoli, Giulia Elena, additional, Mapelli, Massimo, additional, Sguazzotti, Maurizio, additional, Negri, Francesco, additional, De Vecchi, Simona, additional, Ciabatti, Michele, additional, Tomasoni, Daniela, additional, Mazzanti, Andrea, additional, Marzo, Francesca, additional, de Gregorio, Cesare, additional, Raineri, Claudia, additional, Vianello, Pier Filippo, additional, Marchi, Alberto, additional, Biagioni, Giulia, additional, Insinna, Eleonora, additional, Parisi, Vanda, additional, Ditaranto, Raffaello, additional, Barison, Andrea, additional, Giammaresi, Andrea, additional, De Ferrari, Gaetano Maria, additional, Priori, Silvia, additional, Metra, Marco, additional, Pieroni, Maurizio, additional, Patti, Giuseppe, additional, Imazio, Massimo, additional, Perugini, Enrica, additional, Agostoni, Piergiuseppe, additional, Cameli, Matteo, additional, Merlo, Marco, additional, Sinagra, Gianfranco, additional, Senni, Michele, additional, Limongelli, Giuseppe, additional, Ammirati, Enrico, additional, Vagnarelli, Fabio, additional, Crotti, Lia, additional, Badano, Luigi, additional, Calore, Chiara, additional, Gabrielli, Domenico, additional, Re, Federica, additional, Musumeci, Giuseppe, additional, Emdin, Michele, additional, Barbato, Emanuele, additional, Musumeci, Beatrice, additional, Autore, Camillo, additional, Biagini, Elena, additional, Porto, Italo, additional, Olivotto, Iacopo, additional, and Canepa, Marco, additional

Published
2023
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6. Predictors and outcomes of pacemaker implantation in patients with cardiac amyloidosis

Author

Saturi, Giulia, primary, De Frutos, Fernando, additional, Sguazzotti, Maurizio, additional, Gonzalez-Lopez, Esther, additional, Nardi, Elena, additional, Domínguez, Fernando, additional, Ponziani, Alberto, additional, Cabrera, Eva, additional, Caponetti, Angelo Giuseppe, additional, Lozano, Sara, additional, Massa, Paolo, additional, Cobo-Marcos, Marta, additional, Accietto, Antonella, additional, Castro-Urda, Victor, additional, Giovannetti, Alessandro, additional, Toquero, Jorge, additional, Gagliardi, Christian, additional, Gómez-Bueno, Manuel, additional, Rios-Tamayo, Rafael, additional, Biagini, Elena, additional, Segovia, Javier, additional, Galiè, Nazzareno, additional, García-Pavía, Pablo, additional, and Longhi, Simone, additional

Published
2023
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7. Predictors and outcomes of pacemaker implantation in patients with cardiac amyloidosis.

Author

Saturi, Giulia, De Frutos, Fernando, Sguazzotti, Maurizio, Gonzalez-Lopez, Esther, Nardi, Elena, Domínguez, Fernando, Ponziani, Alberto, Cabrera, Eva, Giuseppe Caponetti, Angelo, Lozano, Sara, Massa, Paolo, Cobo-Marcos, Marta, Accietto, Antonella, Castro-Urda, Victor, Giovannetti, Alessandro, Toquero, Jorge, Gagliardi, Christian, Gómez-Bueno, Manuel, Rios-Tamayo, Rafael, and Biagini, Elena

Subjects
CARDIAC amyloidosis, CARDIAC pacemakers, CARDIAC patients, GLOBAL longitudinal strain, HEART conduction system, PLASMA cell diseases
Published
2024
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8. Real‐world candidacy to mavacamten in a contemporary hypertrophic obstructive cardiomyopathy population.

Author

Bertero, Edoardo, Chiti, Chiara, Schiavo, Maria Alessandra, Tini, Giacomo, Costa, Paolo, Todiere, Giancarlo, Mabritto, Barbara, Dei, Lorenzo‐Lupo, Giannattasio, Alessia, Mariani, Davide, Lofiego, Carla, Santolamazza, Caterina, Monda, Emanuele, Quarta, Giovanni, Barbisan, Davide, Mandoli, Giulia Elena, Mapelli, Massimo, Sguazzotti, Maurizio, Negri, Francesco, and De Vecchi, Simona

Subjects
HYPERTROPHIC cardiomyopathy, VENTRICULAR outflow obstruction, VENTRICULAR ejection fraction
Abstract

Aims: In the EXPLORER‐HCM trial, mavacamten reduced left ventricular outflow tract obstruction (LVOTO) and improved functional capacity of symptomatic hypertrophic obstructive cardiomyopathy (HOCM) patients. We sought to define the potential use of mavacamten by comparing real‐world HOCM patients with those enrolled in EXPLORER‐HCM and assessing their eligibility to treatment. Methods and results: We collected information on HOCM patients followed up at 25 Italian HCM outpatient clinics and with significant LVOTO (i.e. gradient ≥30 mmHg at rest or ≥50 mmHg after Valsalva manoeuvre or exercise) despite pharmacological or non‐pharmacological therapy. Pharmacological or non‐pharmacological therapy resolved LVOTO in 1044 (61.2%) of the 1706 HOCM patients under active follow‐up, whereas 662 patients (38.8%) had persistent LVOTO. Compared to the EXPLORER‐HCM trial population, these real‐world HOCM patients were older (62.1 ± 14.3 vs. 58.5 ± 12.2 years, p = 0.02), had a lower body mass index (26.8 ± 5.3 vs. 29.7 ± 4.9 kg/m2, p < 0.0001) and a more frequent history of atrial fibrillation (21.5% vs. 9.8%, p = 0.027). At echocardiography, they had lower left ventricular ejection fraction (LVEF, 66 ± 7% vs. 74 ± 6%, p < 0.0001), higher left ventricular outflow tract gradients at rest (60 ± 27 vs. 52 ± 29 mmHg, p = 0.003), and larger left atrial volume index (49 ± 16 vs. 40 ± 12 ml/m2, p < 0.0001). Overall, 324 (48.9%) would have been eligible for enrolment in the EXPLORER‐HCM trial and 339 (51.2%) for treatment with mavacamten according to European guidelines. Conclusions: Real‐world HOCM patients differ from the EXPLORER‐HCM population for their older age, lower LVEF and larger atrial volume, potentially reflecting a more advanced stage of the disease. About half of real‐world HOCM patients were found eligible to mavacamten. [ABSTRACT FROM AUTHOR]

Published
2024
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9. Tc-99m labelled bone scintigraphy in suspected cardiac amyloidosis

Author

Rauf, Muhammad Umaid, primary, Hawkins, Philip N, additional, Cappelli, Francesco, additional, Perfetto, Federico, additional, Zampieri, Mattia, additional, Argiro, Alessia, additional, Petrie, Aviva, additional, Law, Steven, additional, Porcari, Aldostefano, additional, Razvi, Yousuf, additional, Bomsztyk, Joshua, additional, Ravichandran, Sriram, additional, Ioannou, Adam, additional, Patel, Rishi, additional, Starr, Neasa, additional, Hutt, David F, additional, Mahmood, Shameem, additional, Wisniowski, Brendan, additional, Martinez–Naharro, Ana, additional, Venneri, Lucia, additional, Whelan, Carol, additional, Roczenio, Dorota, additional, Gilbertson, Janet, additional, Lachmann, Helen J, additional, Wechalekar, Ashutosh D, additional, Rapezzi, Claudio, additional, Serenelli, Matteo, additional, Massa, Paolo, additional, Caponetti, Angelo Giuseppe, additional, Ponziani, Alberto, additional, Accietto, Antonella, additional, Giovannetti, Alessandro, additional, Saturi, Giulia, additional, Sguazzotti, Maurizio, additional, Gagliardi, Christian, additional, Biagini, Elena, additional, Longhi, Simone, additional, Fontana, Marianna, additional, and Gillmore, Julian D, additional

Published
2023
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10. Screening approaches to cardiac amyloidosis in different clinical settings: Current practice and future perspectives

Author

Caponetti, Angelo Giuseppe, primary, Accietto, Antonella, additional, Saturi, Giulia, additional, Ponziani, Alberto, additional, Sguazzotti, Maurizio, additional, Massa, Paolo, additional, Giovannetti, Alessandro, additional, Ditaranto, Raffaello, additional, Parisi, Vanda, additional, Leone, Ornella, additional, Guaraldi, Pietro, additional, Cortelli, Pietro, additional, Gagliardi, Christian, additional, Longhi, Simone, additional, Galiè, Nazzareno, additional, and Biagini, Elena, additional

Published
2023
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11. 172 CLINICAL AND PROGNOSTIC IMPLICATIONS OF RV UPTAKE WITH RADIONUCLIDE SCINTIGRAPHY IN TRANSTHYRETIN CARDIAC AMYLOIDOSIS

Author

Porcari, Aldostefano, primary, Fontana, Marianna, additional, Canepa, Marco, additional, Biagini, Elena, additional, Cappelli, Francesco, additional, Gagliardi, Christian, additional, Longhi, Simone, additional, Pagura, Linda, additional, Tini, Giacomo, additional, Dore, Franca, additional, Bonfiglioli, Rachele, additional, Bauckneht, Matteo, additional, Miceli, Alberto, additional, Girardi, Francesca, additional, Martini, Anna Lisa, additional, Barbati, Giulia, additional, Costanzo, Egidio Natalino, additional, Caponetti, Angelo Giuseppe, additional, Paccagnella, Andrea, additional, Sguazzotti, Maurizio, additional, La Malfa, Giovanni, additional, Zampieri, Mattia, additional, Sciagrà, Roberto, additional, Perfetto, Federico, additional, Hutt, David, additional, Rapezzi1, Claudio, additional, Merlo, Marco, additional, Sinagra, Gianfranco, additional, and Gillmore, Julian D, additional

Published
2022
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13. 826 NEW THERAPEUTIC PERSPECTIVES IN CARDIAC AMYLOIDOSIS

Author

Giovannetti, Alessandro, primary, Accietto, Antonella, additional, Caponetti, Angelo Giuseppe, additional, Saturi, Giulia, additional, Ponziani, Alberto, additional, Massa, Paolo, additional, Sguazzotti, Maurizio, additional, Gagliardi, Christian, additional, Galiè, Nazzareno, additional, Biagini, Elena, additional, and Longhi, Simone, additional

Published
2022
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14. 172 Two mutations, one patient: which phenotype?

Author

Giovannetti, Alessandro, primary, Accietto, Antonella, additional, Caponetti, Angelo Giuseppe, additional, Saturi, Giulia, additional, Ponziani, Alberto, additional, Massa, Paolo, additional, Sguazzotti, Maurizio, additional, Longhi, Simone, additional, Galiè, Nazzareno, additional, and Gagliardi, Christian, additional

Published
2021
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15. 334 Hereditary transthyretin amyloidosis: main features and profiles of different clinical phenotypes

Author

Massa, Paolo, primary, Caponetti, Angelo Giuseppe, additional, Saturi, Giulia, additional, Ponziani, Albero, additional, Sguazzotti, Maurizio, additional, Accietto, Antonella, additional, Dal Passo, Beatrice, additional, Longhi, Simone, additional, Bonfiglioli, Rachele, additional, Mattana, Francesco, additional, Guaraldi, Pietro, additional, Cortelli, Pietro, additional, Galié, Nazzareno, additional, Biagini, Elena, additional, and Gagliardi, Christian, additional

Published
2021
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16. 199 Lower aortic valve calcium scores by computed tomography scan. A potential new red flag of concomitant cardiac amyloidosis in patients with severe aortic stenosis

Author

Ponziani, Alberto, primary, Saturi, Giulia, additional, Santona, Laura, additional, Sguazzotti, Maurizio, additional, Caponetti, Angelo Giuseppe, additional, Massa, Paolo, additional, Gagliardi, Christian, additional, Giovannetti, Alessandro, additional, Lovato, Luigi, additional, Attinà, Domenico, additional, Bonfiglioli, Rachele, additional, Saia, Francesco, additional, Galiè, Nazzareno, additional, Biagini, Elena, additional, and Longhi, Simone, additional

Published
2021
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17. 351 Prevalence and prognostic significance of RV uptake (biventricular uptake) at planar scintigraphy in patients with ATTR cardiac amyloidosis

Author

Porcari, Aldostefano, primary, Pagura, Linda, additional, Canepa, Marco, additional, Biagini, Elena, additional, Cappelli, Francesco, additional, Gagliardi, Christian, additional, Longhi, Simone, additional, Tini, Giacomo, additional, Dore, Franca, additional, Bonfiglioli, Rachele, additional, Bauckneht, Matteo, additional, Miceli, Alberto, additional, Girardi, Francesca, additional, Martini, Anna Lisa, additional, Caponetti, Angelo Giuseppe, additional, Paccagnella, Andrea, additional, Sguazzotti, Maurizio, additional, Malfa, Giovanni La, additional, Zampieri, Mattia, additional, Alessia, Argiro, additional, Porto, Italo, additional, Perfetto, Federico, additional, Rapezzi, Claudio, additional, Merlo, Marco, additional, and Sinagra, Gianfranco, additional

Published
2021
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18. Role of Gas6 and TAM Receptors in the Identification of Cardiopulmonary Involvement in Systemic Sclerosis and Scleroderma Spectrum Disorders

Author

Bellan, Mattia, primary, Dimagli, Arnaldo, additional, Piccinino, Cristina, additional, Giubertoni, Ailia, additional, Ianniello, Aurora, additional, Grimoldi, Federico, additional, Sguazzotti, Maurizio, additional, Nerviani, Alessandra, additional, Barini, Michela, additional, Carriero, Alessandro, additional, Smirne, Carlo, additional, Burlone, Michela Emma, additional, Rigamonti, Cristina, additional, Minisini, Rosalba, additional, Salmi, Livia, additional, Barbaglia, Matteo Nazzareno, additional, Castello, Luigi Mario, additional, Sola, Daniele, additional, Marino, Paolo, additional, Avanzi, Gian Carlo, additional, Pirisi, Mario, additional, and Sainaghi, Pier Paolo, additional

Published
2020
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19. Red Cell Distribution Width and Platelet Count as Biomarkers of Pulmonary Arterial Hypertension in Patients with Connective Tissue Disorders

Author

Bellan, Mattia, primary, Giubertoni, Ailia, additional, Piccinino, Cristina, additional, Dimagli, Arnaldo, additional, Grimoldi, Federico, additional, Sguazzotti, Maurizio, additional, Burlone, Michela Emma, additional, Smirne, Carlo, additional, Sola, Daniele, additional, Marino, Paolo, additional, Pirisi, Mario, additional, and Sainaghi, Pier Paolo, additional

Published
2019
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20. [Ten questions about transthyretin amyloidosis].

Author

Giovannetti A, Accietto A, Massa P, Leone O, Guaraldi P, Saturi G, Caponetti AG, Sguazzotti M, Ponziani A, Gagliardi C, Galiè N, Cortelli P, Longhi S, and Biagini E

Subjects
Humans, Prealbumin genetics, Prealbumin metabolism, Amyloid Neuropathies, Familial diagnosis, Amyloid Neuropathies, Familial therapy, Cardiomyopathies diagnosis, Cardiomyopathies etiology, Cardiomyopathies therapy, Heart Failure complications
Abstract

Systemic amyloidosis is a hereditary or acquired disease characterized by deposition of amyloid insoluble fibrils into body organs and tissues, causing structural abnormalities and organ dysfunction, i.e. heart failure. This disease is classified according to the precursor protein involved; immunoglobulin light chains, transthyretin and apolipoprotein A1 underlie the cardiac involvement. Amyloid cardiomyopathy is characterized by symmetric biventricular hypertrophy, preserved systolic function, and pronounced diastolic dysfunction. Although transthyretin-related cardiac amyloidosis has always been considered a rare disease, in the last few years it has been found to be one of the most common causes of hypertrophic cardiomyopathy, thanks to better diagnostic algorithms and considerable improvements in cardiac imaging. Achieving an early diagnosis is a challenge for the modern cardiologist since new disease-modifying therapies have been developed in recent years. This article aims to answer to the main questions about transthyretin-related cardiac amyloidosis: when to suspect it, how to diagnose it and how to treat it.

Published
2022
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