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1. Clinical course of hypertrophic cardiomyopathy patients implanted with a transvenous or subcutaneous defibrillator

Author

Francia, P, Ziacchi, M, Adduci, C, Ammendola, E, Pieragnoli, P, Filippo, P, Rapacciuolo, A, Rella, V, Migliore, F, Viani, S, Musumeci, M, Biagini, E, Lovecchio, M, Baldini, R, Falasconi, G, Autore, C, Biffi, M, Cecchi, F, Francia P., Ziacchi M., Adduci C., Ammendola E., Pieragnoli P., Filippo P. D., Rapacciuolo A., Rella V., Migliore F., Viani S., Musumeci M. B., Biagini E., Lovecchio M., Baldini R., Falasconi G., Autore C., Biffi M., Cecchi F., Francia, P, Ziacchi, M, Adduci, C, Ammendola, E, Pieragnoli, P, Filippo, P, Rapacciuolo, A, Rella, V, Migliore, F, Viani, S, Musumeci, M, Biagini, E, Lovecchio, M, Baldini, R, Falasconi, G, Autore, C, Biffi, M, Cecchi, F, Francia P., Ziacchi M., Adduci C., Ammendola E., Pieragnoli P., Filippo P. D., Rapacciuolo A., Rella V., Migliore F., Viani S., Musumeci M. B., Biagini E., Lovecchio M., Baldini R., Falasconi G., Autore C., Biffi M., and Cecchi F.

Abstract

Aims The implantable cardioverter-defibrillator (ICD) is a life-saving therapy in patients with hypertrophic cardiomyopathy (HCM) at risk of sudden cardiac death. Implantable cardioverter-defibrillator complications are of concern. The subcutaneous ICD (S-ICD) does not use transvenous leads and is expected to reduce complications. However, it does not provide bradycardia and anti-tachycardia pacing (ATP). The aim of this study was to compare appropriate and inappropriate ICD interventions, complications, disease-related adverse events and mortality between HCM patients implanted with a S- or transvenous (TV)-ICD.Methods and results Consecutive HCM patients implanted with a S- (n = 216) or TV-ICD (n = 211) were enrolled. Propensity-adjusted cumulative Kaplan-Meier curves and multivariate Cox proportional hazard ratios were used to compare 5-year event-free survival and the risk of events. The S-ICD patients had lower 5-year risk of appropriate (HR: 0.32; 95%CI: 0.15-0.65; P = 0.002) and inappropriate (HR: 0.44; 95%CI: 0.20-0.95; P = 0.038) ICD interventions, driven by a high incidence of ATP therapy in the TV-ICD group. The S- and TV-ICD patients experienced similar 5-year rate of device-related complications, albeit the risk of major lead-related complications was lower in S-ICD patients (HR: 0.17; 95%CI: 0.038-0.79; P = 0.023). The TV- and S-ICD patients displayed similar risk of disease-related complications (HR: 0.64; 95%CI: 0.27-1.52; P = 0.309) and mortality (HR: 0.74; 95%CI: 0.29-1.87; P = 0.521).Conclusion Hypertrophic cardiomyopathy patients implanted with a S-ICD had lower 5-year risk of appropriate and inappropriate ICD therapies as well as of major lead-related complications as compared to those implanted with a TV-ICD. Long-term comparative follow-up studies will clarify whether the lower incidence of major lead-related complications will translate into a morbidity or survival benefit.Graphical Abstract

Published
2023

2. Diagnostic pathways to wild-type transthyretin amyloid cardiomyopathy: a multicentre network study

Author

Tini, G, Milani, P, Zampieri, M, Caponetti, A, Fabris, F, Foli, A, Argiro, A, Mazzoni, C, Gagliardi, C, Longhi, S, Saturi, G, Vergaro, G, Aimo, A, Russo, D, Varra, G, Serenelli, M, Fabbri, G, De Michieli, L, Palmiero, G, Ciliberti, G, Carigi, S, Sessarego, E, Mandoli, G, Ricci Lucchi, G, Rella, V, Monti, E, Gardini, E, Bartolotti, M, Crotti, L, Merli, E, Mussinelli, R, Vianello, P, Cameli, M, Marzo, F, Guerra, F, Limongelli, G, Cipriani, A, Perlini, S, Obici, L, Perfetto, F, Autore, C, Porto, I, Rapezzi, C, Sinagra, G, Merlo, M, Musumeci, B, Emdin, M, Biagini, E, Cappelli, F, Palladini, G, Canepa, M, Tini G., Milani P., Zampieri M., Caponetti A. G., Fabris F., Foli A., Argiro A., Mazzoni C., Gagliardi C., Longhi S., Saturi G., Vergaro G., Aimo A., Russo D., Varra G. G., Serenelli M., Fabbri G., De Michieli L., Palmiero G., Ciliberti G., Carigi S., Sessarego E., Mandoli G. E., Ricci Lucchi G., Rella V., Monti E., Gardini E., Bartolotti M., Crotti L., Merli E., Mussinelli R., Vianello P. F., Cameli M., Marzo F., Guerra F., Limongelli G., Cipriani A., Perlini S., Obici L., Perfetto F., Autore C., Porto I., Rapezzi C., Sinagra G., Merlo M., Musumeci B., Emdin M., Biagini E., Cappelli F., Palladini G., Canepa M., Tini, G, Milani, P, Zampieri, M, Caponetti, A, Fabris, F, Foli, A, Argiro, A, Mazzoni, C, Gagliardi, C, Longhi, S, Saturi, G, Vergaro, G, Aimo, A, Russo, D, Varra, G, Serenelli, M, Fabbri, G, De Michieli, L, Palmiero, G, Ciliberti, G, Carigi, S, Sessarego, E, Mandoli, G, Ricci Lucchi, G, Rella, V, Monti, E, Gardini, E, Bartolotti, M, Crotti, L, Merli, E, Mussinelli, R, Vianello, P, Cameli, M, Marzo, F, Guerra, F, Limongelli, G, Cipriani, A, Perlini, S, Obici, L, Perfetto, F, Autore, C, Porto, I, Rapezzi, C, Sinagra, G, Merlo, M, Musumeci, B, Emdin, M, Biagini, E, Cappelli, F, Palladini, G, Canepa, M, Tini G., Milani P., Zampieri M., Caponetti A. G., Fabris F., Foli A., Argiro A., Mazzoni C., Gagliardi C., Longhi S., Saturi G., Vergaro G., Aimo A., Russo D., Varra G. G., Serenelli M., Fabbri G., De Michieli L., Palmiero G., Ciliberti G., Carigi S., Sessarego E., Mandoli G. E., Ricci Lucchi G., Rella V., Monti E., Gardini E., Bartolotti M., Crotti L., Merli E., Mussinelli R., Vianello P. F., Cameli M., Marzo F., Guerra F., Limongelli G., Cipriani A., Perlini S., Obici L., Perfetto F., Autore C., Porto I., Rapezzi C., Sinagra G., Merlo M., Musumeci B., Emdin M., Biagini E., Cappelli F., Palladini G., and Canepa M.

Abstract

Aim: Epidemiology of wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) remains poorly defined. A better characterization of pathways leading to ATTRwt-CA diagnosis is of key importance, and potentially informative of disease course and prognosis. The aim of this study was to describe the characteristics of contemporary pathways leading to ATTRwt-CA diagnosis, and their potential association with survival. Methods and results: This was a retrospective study of patients diagnosed with ATTRwt-CA at 17 Italian referral centres for CA. Patients were categorized into different ‘pathways’ according to the medical reason that triggered the diagnosis of ATTRwt-CA (hypertrophic cardiomyopathy [HCM] pathway, heart failure [HF] pathway, incidental imaging or incidental clinical pathway). Prognosis was investigated with all-cause mortality as endpoint. Overall, 1281 ATTRwt-CA patients were included in the study. The diagnostic pathway leading to ATTRwt-CA diagnosis was HCM in 7% of patients, HF in 51%, incidental imaging in 23%, incidental clinical in 19%. Patients in the HF pathway, as compared to the others, were older and had a greater prevalence of New York Heart Association (NYHA) class III–IV and chronic kidney disease. Survival was significantly worse in the HF versus other pathways, but similar among the three others. In multivariate model, older age at diagnosis, NYHA class III–IV and some comorbidities but not the HF pathway were independently associated with worse survival. Conclusions: Half of contemporary ATTRwt-CA diagnoses occur in a HF setting. These patients had worse clinical profile and outcome than those diagnosed either due to suspected HCM or incidentally, although prognosis remained primarily related to age, NYHA functional class and comorbidities rather than the diagnostic pathway itself.

Published
2023

3. From the phenotype to precision medicine: An update on the cardiomyopathies diagnostic workflow

Author

Autore, C, Bariani, R, Bauce, B, Biagini, E, Canepa, M, Castelletti, S, Crotti, L, Limongelli, G, Merlo, M, Monda, E, Pio Loco Detto Gava, C, Parisi, V, Tini, G, Imazio, M, Autore C., Bariani R., Bauce B., Biagini E., Canepa M., Castelletti S., Crotti L., Limongelli G., Merlo M., Monda E., Pio Loco Detto Gava C., Parisi V., Tini G., Imazio M., Autore, C, Bariani, R, Bauce, B, Biagini, E, Canepa, M, Castelletti, S, Crotti, L, Limongelli, G, Merlo, M, Monda, E, Pio Loco Detto Gava, C, Parisi, V, Tini, G, Imazio, M, Autore C., Bariani R., Bauce B., Biagini E., Canepa M., Castelletti S., Crotti L., Limongelli G., Merlo M., Monda E., Pio Loco Detto Gava C., Parisi V., Tini G., and Imazio M.

Abstract

Cardiomyopathies are disease of the cardiac muscle largely due to genetic alterations of proteins with 'structural' or 'functional' roles within the cardiomyocyte, going from the regulation of contraction-relaxation, metabolic and energetic processes to ionic fluxes. Modifications occurring to these proteins are responsible, in the vast majority of cases, for the phenotypic manifestations of the disease, including hypertrophic, dilated, arrhythmogenic and restrictive cardiomyopathies. Secondary nonhereditary causes to be excluded include infections, toxicity from drugs or alcohol or medications, hormonal imbalance and so on. Obtaining a phenotypic definition and an etiological diagnosis is becoming increasingly relevant and feasible, thanks to the availability of new tailored treatments and the diagnostic advancements made particularly in the field of genetics. This is, for example, the case for transthyretin cardiac amyloidosis, Fabry disease or dilated cardiomyopathies due to laminopathies. For these diseases, specific medications have been developed, and a more tailored arrhythmic risk stratification guides the implantation of a defibrillator. In addition, new medications directly targeting the altered protein responsible for the phenotype are becoming available (including the myosin inhibitors mavacantem and aficamten, monoclonal antibodies against Ras-MAPK, genetic therapies for sarcoglycanopathies), thus making a precision medicine approach less unrealistic even in the field of cardiomyopathies. For these reasons, a contemporary approach to cardiomyopathies must consider diagnostic algorithms founded on the clinical suspicion of the disease and developed towards a more precise phenotypic definition and etiological diagnosis, based on a multidisciplinary methodology putting together specialists from different disciplines, facilities for advanced imaging testing and genetic and anatomopathological competencies.

Published
2023

4. ECG/echo indexes in the diagnostic approach to amyloid cardiomyopathy: A head-to-head comparison from the AC-TIVE study

Author

Pagura, L, Porcari, A, Cameli, M, Biagini, E, Canepa, M, Crotti, L, Imazio, M, Forleo, C, Pavasini, R, Limongelli, G, Perlini, S, Metra, M, Boriani, G, Emdin, M, Sinagra, G, Merlo, M, Longo, F, Rossi, M, Varrà, G, Saro, R, Dore, F, Girardi, F, Vergaro, G, Musumeci, B, Autore, C, Cappelli, F, Perfetto, F, Olivotto, I, Favale, S, Carella, M, Guaricci, A, Ciccone, M, Di Bella, G, Tomasoni, D, Rella, V, Branzi, G, Badano, L, Parati, G, Palmiero, G, Caiazza, M, Caponetti, A, Saturi, G, Labate, M, Andreis, A, Paneva, E, De Ferrari, G, Di Ienno, L, De Carli, G, Giacomin, E, Arzilli, C, Pagura L., Porcari A., Cameli M., Biagini E., Canepa M., Crotti L., Imazio M., Forleo C., Pavasini R., Limongelli G., Perlini S., Metra M., Boriani G., Emdin M., Sinagra G., Merlo M., Longo F., Rossi M., Varrà G. G., Saro R., Dore F., Girardi F., Vergaro G., Musumeci B., Autore C., Cappelli F., Perfetto F., Olivotto I., Favale S., Carella M. C., Guaricci A. I., Ciccone M. M., Di Bella G., Tomasoni D., Rella V., Branzi G., Badano L., Parati G., Palmiero G., Caiazza M., Caponetti A. G., Saturi G., Labate M. E., Andreis A., Paneva E., De Ferrari G. M., Di Ienno L., De Carli G., Giacomin E., Arzilli C., Pagura, L, Porcari, A, Cameli, M, Biagini, E, Canepa, M, Crotti, L, Imazio, M, Forleo, C, Pavasini, R, Limongelli, G, Perlini, S, Metra, M, Boriani, G, Emdin, M, Sinagra, G, Merlo, M, Longo, F, Rossi, M, Varrà, G, Saro, R, Dore, F, Girardi, F, Vergaro, G, Musumeci, B, Autore, C, Cappelli, F, Perfetto, F, Olivotto, I, Favale, S, Carella, M, Guaricci, A, Ciccone, M, Di Bella, G, Tomasoni, D, Rella, V, Branzi, G, Badano, L, Parati, G, Palmiero, G, Caiazza, M, Caponetti, A, Saturi, G, Labate, M, Andreis, A, Paneva, E, De Ferrari, G, Di Ienno, L, De Carli, G, Giacomin, E, Arzilli, C, Pagura L., Porcari A., Cameli M., Biagini E., Canepa M., Crotti L., Imazio M., Forleo C., Pavasini R., Limongelli G., Perlini S., Metra M., Boriani G., Emdin M., Sinagra G., Merlo M., Longo F., Rossi M., Varrà G. G., Saro R., Dore F., Girardi F., Vergaro G., Musumeci B., Autore C., Cappelli F., Perfetto F., Olivotto I., Favale S., Carella M. C., Guaricci A. I., Ciccone M. M., Di Bella G., Tomasoni D., Rella V., Branzi G., Badano L., Parati G., Palmiero G., Caiazza M., Caponetti A. G., Saturi G., Labate M. E., Andreis A., Paneva E., De Ferrari G. M., Di Ienno L., De Carli G., Giacomin E., and Arzilli C.

Abstract

Background and aims: The discordance between QRS voltages on electrocardiogram (ECG) and left ventricle (LV) wall thickness (LVWT) on echocardiogram (echo) is a recognized red flag (RF) of amyloid cardiomyopathy (AC) and can be measured by specific indexes. No head-to-head comparison of different ECG/echo indexes among subjects with echocardiographic suspicion of AC has yet been undertaken. The study aimed at evaluating the performance and the incremental diagnostic value of different ECG/echo indexes in this subset of patients. Methods: Electrocardiograms of subjects with LV hypertrophy, preserved ejection fraction and ≥ 1 echocardiographic RF of AC participating in the AC-TIVE study, an Italian prospective multicenter study, were independently analyzed by two cardiologists. Low QRS voltages and 8 different ECG/echo indexes were evaluated. Cohort specific cut-offs were computed. Results: Among 170 patients, 55 (32 %) were diagnosed with AC. Combination of low QRS voltages with interventricular septum ≥ 1,6 cm was the most specific (specificity 100 %, positive predictive value 100 %) ECG/echo index, while the ratio between the sum of all QRS voltages and LVWT <7,8 was the most sensitive and accurate (sensitivity 94 %, negative predictive value 97 %, accuracy 82 %). When the latter index was added to a model using easily-accessible clinical variables, the diagnostic accuracy for AC greatly increased (AUC from 0,84 to 0,95; p = 0,007). Conclusions: Among patients with non-dilated hypertrophic ventricles with normal ejection fraction and echocardiographic RF of AC, easily-measurable ECG/echo indexes, mainly when added to few clinical variables, can help the physician orient second level investigations. External validation of the results is warranted.

Published
2023

5. Unmasking the prevalence of amyloid cardiomyopathy in the real world: results from Phase 2 of the AC-TIVE study, an Italian nationwide survey

Author

Merlo, M, Pagura, L, Porcari, A, Cameli, M, Vergaro, G, Musumeci, B, Biagini, E, Canepa, M, Crotti, L, Imazio, M, Forleo, C, Cappelli, F, Perfetto, F, Favale, S, Di Bella, G, Dore, F, Girardi, F, Tomasoni, D, Pavasini, R, Rella, V, Palmiero, G, Caiazza, M, Carella, M, Igoren Guaricci, A, Branzi, G, Caponetti, A, Saturi, G, La Malfa, G, Merlo, A, Andreis, A, Bruno, F, Longo, F, Rossi, M, Varra, G, Saro, R, Di Ienno, L, De Carli, G, Giacomin, E, Arzilli, C, Limongelli, G, Autore, C, Olivotto, I, Badano, L, Parati, G, Perlini, S, Metra, M, Emdin, M, Rapezzi, C, Sinagra, G, Merlo M., Pagura L., Porcari A., Cameli M., Vergaro G., Musumeci B., Biagini E., Canepa M., Crotti L., Imazio M., Forleo C., Cappelli F., Perfetto F., Favale S., Di Bella G., Dore F., Girardi F., Tomasoni D., Pavasini R., Rella V., Palmiero G., Caiazza M., Carella M. C., Igoren Guaricci A., Branzi G., Caponetti A. G., Saturi G., La Malfa G., Merlo A. C., Andreis A., Bruno F., Longo F., Rossi M., Varra G. G., Saro R., Di Ienno L., De Carli G., Giacomin E., Arzilli C., Limongelli G., Autore C., Olivotto I., Badano L., Parati G., Perlini S., Metra M., Emdin M., Rapezzi C., Sinagra G., Merlo, M, Pagura, L, Porcari, A, Cameli, M, Vergaro, G, Musumeci, B, Biagini, E, Canepa, M, Crotti, L, Imazio, M, Forleo, C, Cappelli, F, Perfetto, F, Favale, S, Di Bella, G, Dore, F, Girardi, F, Tomasoni, D, Pavasini, R, Rella, V, Palmiero, G, Caiazza, M, Carella, M, Igoren Guaricci, A, Branzi, G, Caponetti, A, Saturi, G, La Malfa, G, Merlo, A, Andreis, A, Bruno, F, Longo, F, Rossi, M, Varra, G, Saro, R, Di Ienno, L, De Carli, G, Giacomin, E, Arzilli, C, Limongelli, G, Autore, C, Olivotto, I, Badano, L, Parati, G, Perlini, S, Metra, M, Emdin, M, Rapezzi, C, Sinagra, G, Merlo M., Pagura L., Porcari A., Cameli M., Vergaro G., Musumeci B., Biagini E., Canepa M., Crotti L., Imazio M., Forleo C., Cappelli F., Perfetto F., Favale S., Di Bella G., Dore F., Girardi F., Tomasoni D., Pavasini R., Rella V., Palmiero G., Caiazza M., Carella M. C., Igoren Guaricci A., Branzi G., Caponetti A. G., Saturi G., La Malfa G., Merlo A. C., Andreis A., Bruno F., Longo F., Rossi M., Varra G. G., Saro R., Di Ienno L., De Carli G., Giacomin E., Arzilli C., Limongelli G., Autore C., Olivotto I., Badano L., Parati G., Perlini S., Metra M., Emdin M., Rapezzi C., and Sinagra G.

Abstract

Aim: To investigate the prevalence of amyloid cardiomyopathy (AC) and the diagnostic accuracy of echocardiographic red flags of AC among consecutive adult patients undergoing transthoracic echocardiogram for reason other than AC in 13 Italian institutions. Methods and results: This is an Italian prospective multicentre study, involving a clinical and instrumental work-up to assess AC prevalence among patients ≥55 years old with an echocardiogram suggestive of AC (i.e. at least one echocardiographic red flag of AC in hypertrophic, non-dilated left ventricles with preserved ejection fraction). The study was registered at ClinicalTrials.gov (NCT04738266). Overall, 381 patients with an echocardiogram suggestive of AC were identified among a cohort of 5315 screened subjects, and 217 patients completed the investigations. A final diagnosis of AC was made in 62 patients with an estimated prevalence of 29% (95% confidence interval 23%–35%). Transthyretin-related AC (ATTR-AC) was diagnosed in 51 and light chain-related AC (AL-AC) in 11 patients. Either apical sparing or a combination of ≥2 other echocardiographic red flags, excluding interatrial septum thickness, provided a diagnostic accuracy >70%. Conclusion: In a cohort of consecutive adults with echocardiographic findings suggestive of AC and preserved left ventricular ejection fraction, the prevalence of AC (either ATTR or AL) was 29%. Easily available echocardiographic red flags, when combined together, demonstrated good diagnostic accuracy.

Published
2022

6. Scar architecture determinants of ventricular arrhythmias in patients with hypertrophic cardiomyopathy: a cardiac magnetic resonance study

Author

Francia, P, primary, Sclafani, M, additional, Franco, P, additional, Falasconi, G, additional, Soto-Iglesias, D, additional, Cigliese, B, additional, Schiavo, M A, additional, Parisi, V, additional, Biagini, E, additional, Musumeci, M B, additional, Autore, C, additional, Alderete, J, additional, Marti-Almor, J, additional, Penela, D, additional, and Berruezo, A, additional

Published
2023
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7. P123 TRANSCATHETER ABLATION OF SUPRAVENTRICULAR ARRHYTHMIAS IN PATIENTS SUFFERING FROM HYPERTROPHIC CARDIOMYOPATHY: A PROPENSITY SCORE–BASED ANALYSIS

Author

Pierri, A, primary, Albani, S, additional, Buongiorno, A, additional, Ricotti, A, additional, Grossi, S, additional, De Rosa, C, additional, Mabritto, B, additional, Bongioanni, S, additional, Luceri, S, additional, Negri, F, additional, Grilli, G, additional, Barbisan, D, additional, Burelli, M, additional, Biondi, F, additional, Cireddu, M, additional, Berg, J, additional, Musumeci, M, additional, Di Donna, P, additional, Vianello, P, additional, Del Franco, A, additional, Scaglione, M, additional, Barbati, G, additional, Berchialla, P, additional, Russo, V, additional, Imazio, M, additional, Porto, I, additional, Canepa, M, additional, Peretto, G, additional, Francia, P, additional, Autore, C, additional, Castagno, D, additional, Gaita, F, additional, Olivotto, I, additional, Merlo, M, additional, Sinagra, G, additional, and Musumeci, G, additional

Published
2023
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9. Current patterns of beta-blocker prescription in cardiac amyloidosis: an Italian nationwide survey

Author

Tini, G, Cappelli, F, Biagini, E, Musumeci, B, Merlo, M, Crotti, L, Cameli, M, Di Bella, G, Cipriani, A, Marzo, F, Guerra, F, Forleo, C, Gagliardi, C, Zampieri, M, Carigi, S, Vianello, P, Mandoli, G, Ciliberti, G, Lichelli, L, Mariani, D, Porcari, A, Russo, D, Licordari, R, Ponziani, A, Porto, I, Perfetto, F, Autore, C, Rapezzi, C, Sinagra, G, Canepa, M, Tini G., Cappelli F., Biagini E., Musumeci B., Merlo M., Crotti L., Cameli M., Di Bella G., Cipriani A., Marzo F., Guerra F., Forleo C., Gagliardi C., Zampieri M., Carigi S., Vianello P. F., Mandoli G. E., Ciliberti G., Lichelli L., Mariani D., Porcari A., Russo D., Licordari R., Ponziani A., Porto I., Perfetto F., Autore C., Rapezzi C., Sinagra G., Canepa M., Tini, G, Cappelli, F, Biagini, E, Musumeci, B, Merlo, M, Crotti, L, Cameli, M, Di Bella, G, Cipriani, A, Marzo, F, Guerra, F, Forleo, C, Gagliardi, C, Zampieri, M, Carigi, S, Vianello, P, Mandoli, G, Ciliberti, G, Lichelli, L, Mariani, D, Porcari, A, Russo, D, Licordari, R, Ponziani, A, Porto, I, Perfetto, F, Autore, C, Rapezzi, C, Sinagra, G, Canepa, M, Tini G., Cappelli F., Biagini E., Musumeci B., Merlo M., Crotti L., Cameli M., Di Bella G., Cipriani A., Marzo F., Guerra F., Forleo C., Gagliardi C., Zampieri M., Carigi S., Vianello P. F., Mandoli G. E., Ciliberti G., Lichelli L., Mariani D., Porcari A., Russo D., Licordari R., Ponziani A., Porto I., Perfetto F., Autore C., Rapezzi C., Sinagra G., and Canepa M.

Abstract

Aims: The use of beta-blocker therapy in cardiac amyloidosis (CA) is debated. We aimed at describing patterns of beta-blocker prescription through a nationwide survey. Methods and results: From 11 referral centres, we retrospectively collected data of CA patients with a first evaluation after 2016 (n = 642). Clinical characteristics at first and last evaluation were collected, with a focus on medical therapy. For patients in whom beta-blocker therapy was started, stopped, or continued between first and last evaluation, the main reason for beta-blocker management was requested. Median age of study population was 77 years; 81% were men. Arterial hypertension was found in 58% of patients, atrial fibrillation (AF) in 57%, and coronary artery disease in 16%. Left ventricular ejection fraction was preserved in 62% of cases, and 74% of patients had advanced diastolic dysfunction. Out of the 250 CA patients on beta-blockers at last evaluation, 215 (33%) were already taking this therapy at first evaluation, while 35 (5%) were started it, in both cases primarily because of high-rate AF. One-hundred-nineteen patients (19%) who were on beta-blocker at first evaluation had this therapy withdrawn, mainly because of intolerance in the presence of heart failure with advanced diastolic dysfunction. The remaining 273 patients (43%) had never received beta-blocker therapy. Beta-blockers usage was similar between CA aetiologies. Patients taking vs. not taking beta-blockers differed only for a greater prevalence of arterial hypertension, coronary artery disease, AF, and non-restrictive filling pattern (P < 0.01 for all) in the former group. Conclusions: Beta-blockers prescription is not infrequent in CA. Such therapy may be tolerated in the presence of co-morbidities for which beta-blockers are routinely used and in the absence of advanced diastolic dysfunction.

Published
2021

12. Multimodality imaging in the diagnostic work-up of endocarditis and cardiac implantable electronic device (CIED) infection

Author

Galea, N, Bandera, F, Lauri, C, Autore, C, Laghi, A, Erba, P, Galea N., Bandera F., Lauri C., Autore C., Laghi A., Erba P. A., Galea, N, Bandera, F, Lauri, C, Autore, C, Laghi, A, Erba, P, Galea N., Bandera F., Lauri C., Autore C., Laghi A., and Erba P. A.

Abstract

Infective endocarditis (IE) is a serious cardiac condition, which includes a wide range of clinical presentations, with varying degrees of severity. The diagnosis is multifactorial and a proper characterization of disease requires the identification of the primary site of infection (usually the cardiac valve) and the search of secondary systemic complications. Early depiction of local complications or distant embolization has a great impact on patient management and prognosis, as it may induce to aggressive antibiotic treatment or, in more advanced cases, cardiac surgery. In this setting, the multimodality imaging has assumed a pivotal role in the clinical decision making and it requires the physician to be aware of the advantages and disadvantages of each imaging technique. Echocardiography is the first imaging test, but it has several limitations. Therefore, the integration with other imaging modalities (computed tomography, magnetic resonance imaging, nuclear imaging) becomes often necessary. Different strategies should be applied depending on whether the infection is suspected or already ascertained, whether located in native or prosthetic valves, in the left or right chambers, or if it involves an implanted cardiac device. In addition, detection of extracardiac IE-related lesions is crucial for a correct management and treatment. The aim of this review is to illustrate strengths and weaknesses of the various methods in the most common clinical scenarios.

Published
2020

14. 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

Author

Merlo, M, Porcari, A, Pagura, L, Cameli, M, Vergaro, G, Musumeci, B, Biagini, E, Canepa, M, Crotti, L, Imazio, M, Forleo, C, Cappelli, F, Favale, S, Di Bella, G, Dore, F, Lombardi, C, Pavasini, R, Rella, V, Palmiero, G, Caiazza, M, Albanese, M, Guaricci, A, Branzi, G, Caponetti, A, Saturi, G, La Malfa, G, Merlo, A, Andreis, A, Bruno, F, Longo, F, Sfriso, E, Di Ienno, L, De Carli, G, Giacomin, E, Spini, V, Milidoni, A, Limongelli, G, Autore, C, Olivotto, I, Badano, L, Parati, G, Perlini, S, Metra, M, Emdin, M, Rapezzi, C, Sinagra, G, 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, Di Bella, Gianluca, Dore, Franca, Lombardi, Carlo Mario, Pavasini, Rita, Rella, Valeria, Palmiero, Giuseppe, Caiazza, Martina, Albanese, Miriam, Guaricci, Andrea Igoren, Branzi, Giovanna, Caponetti, Angelo Giuseppe, Saturi, Giulia, La Malfa, Giovanni, Merlo, Andrea Carlo, Andreis, Alessandro, Bruno, Francesco, Longo, Francesca, Sfriso, Enrico, Di Ienno, Luca, De Carli, Giuseppe, Giacomin, Elisa, Spini, Valentina, Milidoni, Antonino, Limongelli, Giuseppe, Autore, Camillo, Olivotto, Iacopo, Badano, Luigi, Parati, Gianfranco, Perlini, Stefano, Metra, Marco, Emdin, Michele, Rapezzi, Claudio, Sinagra, Gianfranco, Merlo, M, Porcari, A, Pagura, L, Cameli, M, Vergaro, G, Musumeci, B, Biagini, E, Canepa, M, Crotti, L, Imazio, M, Forleo, C, Cappelli, F, Favale, S, Di Bella, G, Dore, F, Lombardi, C, Pavasini, R, Rella, V, Palmiero, G, Caiazza, M, Albanese, M, Guaricci, A, Branzi, G, Caponetti, A, Saturi, G, La Malfa, G, Merlo, A, Andreis, A, Bruno, F, Longo, F, Sfriso, E, Di Ienno, L, De Carli, G, Giacomin, E, Spini, V, Milidoni, A, Limongelli, G, Autore, C, Olivotto, I, Badano, L, Parati, G, Perlini, S, Metra, M, Emdin, M, Rapezzi, C, Sinagra, G, 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, Di Bella, Gianluca, Dore, Franca, Lombardi, Carlo Mario, Pavasini, Rita, Rella, Valeria, Palmiero, Giuseppe, Caiazza, Martina, Albanese, Miriam, Guaricci, Andrea Igoren, Branzi, Giovanna, Caponetti, Angelo Giuseppe, Saturi, Giulia, La Malfa, Giovanni, Merlo, Andrea Carlo, Andreis, Alessandro, Bruno, Francesco, Longo, Francesca, Sfriso, Enrico, Di Ienno, Luca, De Carli, Giuseppe, Giacomin, Elisa, Spini, Valentina, Milidoni, Antonino, Limongelli, Giuseppe, Autore, Camillo, Olivotto, Iacopo, Badano, Luigi, Parati, Gianfranco, Perlini, Stefano, Metra, Marco, Emdin, Michele, Rapezzi, Claudio, and Sinagra, Gianfranco

Published
2022

15. C64 UNMASKING THE PREVALENCE OF AMYLOID CARDIOMYOPATHY IN THE REAL WORLD: RESULTS FROM PHASE 2 OF AC–TIVE STUDY, AN ITALIAN NATIONWIDE SURVEY

Author

Merlo, M, primary, Pagura, L, additional, Porcari, A, additional, Cameli, M, additional, Vergaro, G, additional, Musumeci, B, additional, Biagini, E, additional, Canepa, M, additional, Crotti, L, additional, Imazio, M, additional, Forleo, C, additional, Cappelli, F, additional, Favale, S, additional, Di Bella, G, additional, Dore, F, additional, Girardi, F, additional, Tomasoni, D, additional, Pavasini, R, additional, Rella, V, additional, Palmiero, G, additional, Caiazza, M, additional, Albanese, M, additional, Igoren Guarrucci, A, additional, Branzi, G, additional, Caponetti, A, additional, Saturi, G, additional, La Malfa, G, additional, Merlo, A, additional, Andreis, A, additional, Bruno, F, additional, Longo, F, additional, Rossi, M, additional, Varra‘, G, additional, Saro, R, additional, Di Ienno, L, additional, De Carli, G, additional, Giacomin, E, additional, Spini, V, additional, Limongelli, G, additional, Autore, C, additional, Olivotto, I, additional, Badano, L, additional, Parati, G, additional, Perlini, S, additional, Metra, M, additional, Emdin, M, additional, Rapezzi, C, additional, and Sinagra, G, additional

Published
2022
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16. P295 EXERCISE OXYGEN KINETIC IN HYPERTROPHIC CARDIOMYOPATHY: RESULTS FROM A MULTICENTER CARDIOPULMONARY EXERCISE TESTING STUDY

Author

Mapelli, M, primary, Romani, S, additional, Magrì, D, additional, Merlo, M, additional, Cittar, M, additional, Masè, M, additional, Muratori, M, additional, Gallo, G, additional, Sclafani, M, additional, Carriere, C, additional, Zaffalon, D, additional, Salvioni, E, additional, Mattavelli, I, additional, Vignati, C, additional, De Martino, F, additional, Rovai, S, additional, Autore, C, additional, Sinagra, G, additional, and Agostoni, P, additional

Published
2022
Full Text
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19. Common genetic variants and modifiable risk factors underpin hypertrophic cardiomyopathy susceptibility and expressivity

Author

Harper, A. R., Goel, A., Grace, C., Thomson, K. L., Petersen, S. E., Xu, X., Waring, A., Ormondroyd, E., Kramer, C. M., C. Y., Ho, Neubauer, S., Kolm, P., Kwong, R., Dolman, S. F., Desvigne-Nickens, P., Dimarco, J. P., Geller, N., Kim, D. -Y., Zhang, C., Weintraub, W., Abraham, T., Anderson, L., Appelbaum, E., Autore, C., Berry, C., Biagini, E., Bradlow, W., Bucciarelli-Ducci, C., Chiribiri, A., Choudhury, L., Crean, A., Dawson, D., Desai, M. Y., Elstein, E., Flett, A., Friedrich, M., Heitner, S., Helms, A., Jacoby, D. L., Kim, H., Kim, B., Larose, E., Mahmod, M., Mahrholdt, H., Maron, M., Mccann, G., Michels, M., Mohiddin, S., Nagueh, S., Newby, D., Olivotto, I., Owens, A., Pierre-Mongeon, F., Prasad, S., Rimoldi, O., Salerno, M., Schulz-Menger, J., Sherrid, M., Swoboda, P., van Rossum, A., Weinsaft, J., White, J., Williamson, E., Tadros, R., Ware, J. S., Bezzina, C. R., Farrall, M., Watkins, H., Cardiology, and ACS - Heart failure & arrhythmias

Subjects
LOCI, Genome-wide association study, Blood Pressure, Bioinformatics, DISEASE, SYSTOLIC HYPERTENSION, 0302 clinical medicine, Adolescent, Adult, Aged, Cardiac Myosins, Cardiomyopathy, Hypertrophic, Carrier Proteins, Case-Control Studies, Formins, Genetic Predisposition to Disease, Genome-Wide Association Study, Heterozygote, Humans, Middle Aged, Myosin Heavy Chains, Risk Factors, Sarcomeres, Young Adult, Polymorphism, Single Nucleotide, 11 Medical and Health Sciences, Genetics & Heredity, 0303 health sciences, Hypertrophic cardiomyopathy, Single Nucleotide, 3. Good health, GENOTYPE, cardiovascular system, Life Sciences & Biomedicine, Cardiomyopathy, macromolecular substances, Biology, AMERICAN-COLLEGE, 03 medical and health sciences, Heart disorder, Mendelian randomization, Genetics, medicine, Expressivity (genetics), cardiovascular diseases, Risk factor, GENOME-WIDE ASSOCIATION, Polymorphism, 030304 developmental biology, HCMR Investigators, Science & Technology, Genetic heterogeneity, Case-control study, 06 Biological Sciences, medicine.disease, Hypertrophic, DISCOVERY, 030217 neurology & neurosurgery, Developmental Biology
Abstract

Hypertrophic cardiomyopathy (HCM) is a common, serious, genetic heart disorder. Rare pathogenic variants in sarcomere genes cause HCM, but with unexplained phenotypic heterogeneity. Moreover, most patients do not carry such variants. We report a genome-wide association study of 2,780 cases and 47,486 controls that identified 12 genome-wide-significant susceptibility loci for HCM. Single-nucleotide polymorphism heritability indicated a strong polygenic influence, especially for sarcomere-negative HCM (64% of cases; h2g = 0.34 ± 0.02). A genetic risk score showed substantial influence on the odds of HCM in a validation study, halving the odds in the lowest quintile and doubling them in the highest quintile, and also influenced phenotypic severity in sarcomere variant carriers. Mendelian randomization identified diastolic blood pressure (DBP) as a key modifiable risk factor for sarcomere-negative HCM, with a one standard deviation increase in DBP increasing the HCM risk fourfold. Common variants and modifiable risk factors have important roles in HCM that we suggest will be clinically actionable.

Published
2021

20. Low Sensitivity of Bone Scintigraphy in Detecting Phe64Leu Mutation-Related Transthyretin Cardiac Amyloidosis

Author

Musumeci, M. B., Cappelli, F., Russo, D., Tini, G., Canepa, M., Milandri, A., Bonfiglioli, R., Di Bella, G., My, F., Luigetti, Marco, Grandis, M., Autore, C., Perlini, S., Perfetto, F., Rapezzi, C., Luigetti M. (ORCID:0000-0001-7539-505X), Musumeci, M. B., Cappelli, F., Russo, D., Tini, G., Canepa, M., Milandri, A., Bonfiglioli, R., Di Bella, G., My, F., Luigetti, Marco, Grandis, M., Autore, C., Perlini, S., Perfetto, F., Rapezzi, C., and Luigetti M. (ORCID:0000-0001-7539-505X)

Abstract

Objectives: The aim of this study was to assess the diagnostic accuracy of bone scintigraphy in a large multicenter cohort of patients with cardiac amyloidotic involvement and Phe64Leu transthyretin (TTR) mutation. Background: Diagnostic accuracy of bone scintigraphy for transthyretin-related cardiac amyloidosis (TTR-CA) is considered extremely high, enabling this technique to be the noninvasive diagnostic standard for TTR-CA. Nevertheless, this approach has not been systematically validated across the entire spectrum of TTR mutations. Methods: A total of 55 patients with Phe64Leu TTR mutation were retrospectively analyzed and evaluated between 1993 and 2018 at 7 specialized Italian tertiary centers. Cardiac involvement was defined as presence of an end-diastolic interventricular septum thickness ≥12 mm, without other possible causes of left ventricular hypertrophy (i.e., arterial hypertension or valvulopathies). A technetium-99m (99mTc)–diphosphonate (DPD) or 99mTc–hydroxyl-methylene-diphosphonate (HMDP) bone scintigraphy was reviewed, and visual scoring was evaluated according to Perugini's method. Results: Among 26 patients with definite cardiac involvement, 19 underwent 99mTc-DPD or 99mTc-HMDP bone scintigraphy. Of them, 17 (89.5%) patients had low or absent myocardial bone tracer uptake, whereas only 2 (10.5%) showed high-grade myocardial uptake. The sensitivity and the accuracy of bone scintigraphy in detecting TTR-CA were 10.5% and 37%, respectively. Patients with cardiac involvement and low or absent bone tracer uptake were similar to those with high-grade myocardial uptake in terms of age, sex, and electrocardiographic and echocardiographic findings. Conclusions: The sensitivity of bone scintigraphy (DPD and HMDP) in detecting TTR-CA is extremely low in patients with Phe64Leu TTR mutation, suggesting the need to assess diagnostic accuracy of bone scintigraphy to identify cardiac involvement across a wider spectrum of TTR mutations.

Published
2020

24. Effectiveness of the 2014 european society of cardiology guideline on sudden cardiac death in hypertrophic cardiomyopathy: A systematic review and meta-analysis

Author

O'Mahony, C. Akhtar, M.M. Anastasiou, Z. Guttmann, O.P. Vriesendorp, P.A. Michels, M. Magrì, D. Autore, C. Fernández, A. Ochoa, J.P. Leong, K.M.W. Varnava, A.M. Monserrat, L. Anastasakis, A. Garcia-Pavia, P. Rapezzi, C. Biagini, E. Gimeno, J.R. Limongelli, G. Omar, R.Z. Elliott, P.M.

Subjects
cardiovascular diseases
Abstract

Objective In 2014, the European Society of Cardiology (ESC) recommended the use of a novel risk prediction model (HCM Risk-SCD) to guide use of implantable cardioverter defibrillators (ICD) for the primary prevention of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). We sought to determine the performance of HCM Risk-SCD by conducting a systematic review and meta-analysis of articles reporting on the prevalence of SCD within 5 years of evaluation in low, intermediate and high-risk patients as defined by the 2014 guidelines (predicted risk

Published
2019

25. Targeted next-generation sequencing helps to decipher the genetic and phenotypic heterogeneity of hypertrophic cardiomyopathy

Author

Cecconi, M., Parodi, M. I., Formisano, F., Spirito, P., Autore, C., Musumeci, M. B., Favale, S., Forleo, C., Rapezzi, C., Biagini, E., Davì, S., Canepa, E., Pennese, L., Castagnetta, M., Degiorgio, Dario, Coviello, Domenico, Collaborative Working Group, Cecconi, Massimiliano, Parodi, Maria I., Formisano, Francesco, Spirito, Paolo, Autore, Camillo, Musumeci, Maria B., Favale, Stefano, Forleo, Cinzia, Rapezzi, Claudio, Biagini, Elena, Davì, Sabrina, Canepa, Elisabetta, Pennese, Loredana, Castagnetta, Mauro, Degiorgio, Dario, and Coviello, Domenico A.

Subjects
0301 basic medicine, Arrhythmias, 030204 cardiovascular system & hematology, medicine.disease_cause, Non-sarcomeric genes, Genetic heterogeneity, 0302 clinical medicine, Myofibrils, Borderline HCM, Hypertrophic cardiomyopathy, Ion Torrent Personal Genome Machine, Next-generation sequencing, Phenotypic heterogeneity, Thick/intermediate and thin myofilament proteins, Adolescent, Adult, Aged, Arrhythmias, Cardiac, Cardiomyopathy, Hypertrophic, Child, Child, Preschool, Exons, High-Throughput Nucleotide Sequencing, Humans, Middle Aged, Mutation, Phenotype, Syndrome, Young Adult, Genetics, Non-sarcomeric gene, Sanger sequencing, Thick/intermediate and thin myofilament protein, Articles, General Medicine, Myosin binding, symbols, Cardiac, Cardiomyopathy, Biology, DNA sequencing, NO, 03 medical and health sciences, symbols.namesake, medicine, cardiovascular diseases, Preschool, Gene, Phenocopy, Ion Torrent Personal Genome Machine, borderline HCM, hypertrophic cardiomyopathy, next-generation sequencing, thick/intermediate and thin myofilament proteins, non-sarcomeric genes, genetic heterogeneity, phenotypic heterogeneity, 030104 developmental biology, Hypertrophic, MYH7
Abstract

Hypertrophic cardiomyopathy (HCM) is mainly associated with myosin, heavy chain 7 (MYH7) and myosin binding protein C, cardiac (MYBPC3) mutations. In order to better explain the clinical and genetic heterogeneity in HCM patients, in this study, we implemented a target-next generation sequencing (NGS) assay. An Ion AmpliSeq™ Custom Panel for the enrichment of 19 genes, of which 9 of these did not encode thick/intermediate and thin myofilament (TTm) proteins and, among them, 3 responsible of HCM phenocopy, was created. Ninety-two DNA samples were analyzed by the Ion Personal Genome Machine: 73 DNA samples (training set), previously genotyped in some of the genes by Sanger sequencing, were used to optimize the NGS strategy, whereas 19 DNA samples (discovery set) allowed the evaluation of NGS performance. In the training set, we identified 72 out of 73 expected mutations and 15 additional mutations: the molecular diagnosis was achieved in one patient with a previously wild-type status and the pre-excitation syndrome was explained in another. In the discovery set, we identified 20 mutations, 5 of which were in genes encoding non-TTm proteins, increasing the diagnostic yield by approximately 20%: a single mutation in genes encoding non-TTm proteins was identified in 2 out of 3 borderline HCM patients, whereas co-occuring mutations in genes encoding TTm and galactosidase alpha (GLA) altered proteins were characterized in a male with HCM and multiorgan dysfunction. Our combined targeted NGS-Sanger sequencing-based strategy allowed the molecular diagnosis of HCM with greater efficiency than using the conventional (Sanger) sequencing alone. Mutant alleles encoding non-TTm proteins may aid in the complete understanding of the genetic and phenotypic heterogeneity of HCM: co-occuring mutations of genes encoding TTm and non-TTm proteins could explain the wide variability of the HCM phenotype, whereas mutations in genes encoding only the non-TTm proteins are identifiable in patients with a milder HCM status.

Published
2016

29. Contemporary antithrombotic strategies in patients with acute coronary syndromes managed without revascularization: insights from the EYESHOT study

Author

De Luca, Leonardo, Leonardi, Sergio, Smecca, Ignazio Maria, Formigli, Dario, Lucci, Donata, Gonzini, Lucio, Tuccillo, Bernardino, Olivari, Zoran, Gulizia, Michele Massimo, Bovenzi, Francesco Maria, De Servi, Stefano, Caporale, R., Cavallini, C., Ceravolo, R., Lupi, A., Musumeci, G., Rakar, S., Maggioni, A. P., Lorimer, A., Orsini, G., Fabbri, Giorgio, Bianchini, E., Abrignani, M. G., Bonura, F., Trimarco, B., Galasso, Giorgia, Misuraca, G., Manes, M. T., Irace, Lorenzo, Totis, O., Ledda, A., Mauro, C., Boccalatte, M., Iliceto, S., Cacciavillani, L., Savonitto, S., Tortorella, G., Esposito, L., DE ROSA, Paolo, Calabrò, P., Bianchi, R., Napoletano, C., Lalla Piccioni, L., Pavesi, P. C., Boni, Allegra, Merenda, R., Wolff, S., De Ferrari, G. M., Camporotondo, R., Gambino, Paolo, Cutaia, A., Picariello, C., Cemin, R., Chiarella, F., Grazioli Gauthier, L., Mircoli, L., Del Pinto, M., Finocchiaro, M. L., Scioli, R., Farina, R., Naddeo, C., Scherillo, M., Santopietro, S., Metra, M., Costa, F., Calculli, G., Troito, G., Pennisi, V., Adornato, E. M. F., Pirelli, S., Fadin, B. M., Di Biase, M., Ieva, R., Zuin, G., Sanfilippo, N., Mancuso, LAURA CATERINA, Pani, Luisa Anna, Serra, Eleonora, Marenzi, G., Assanelli, E. M., Ansalone, G., Cacciotti, L., Morocutti, G., Fresco, C., Berti, S., Paradossi, U., Bozzano, A., Mauro, A., Noussan, P., Zanini, P., Bolognese, L., Falsini, G., Costa, P., Manca, G., Caldarola, P., Locuratolo, N., Cipolla, T., Becchina, M., Cocco, Gabriele, Scalera, G., Stefanelli, S., Giunta, N., Sinagra, G., Meloni, L., Lai, O., Chiaranda, G., Luca, G., Sleiman Helou, J., Biscottini, E., Magliari, F., Callerame, M., Uguccioni, M., Pugliese, M., Sanchez, F., Tartaglione, S., Ignone, G., Mavilio, G., Mantovan, R., Bini, R., Caico, S. I., Demolli, V., Proietti, F., Michisanti, M., Musmeci, G., Cantamessa, P., Sicuso, G., Micalef, S. S., Accogli, M., Zaccaria, MICHELA MARIA, Caputo, M., Di Paolo, G., Piatti, L., Farina, A., Vicinelli, P., Paloscia, L., Di Clemente, D., Felis, S., Castini, D., Rota, C., Casu, Gabriella, Bonano, S., Margheri, M., Ricci Lucchi, G., Serdoz, R., Proietti, P., Autore, C., Conti, E., Russo, V., Orlando, P., Ramondo, A. B., Bontorin, M., Marcolongo, M., Marrara, F., Maestroni, A., Vitti, P., Rodella, P., Bonetti, P., Elia, M., Lumare, R., Politi, A., Gritti, S., Poletti, F., Mafrici, A., Fusco, R., Bongo, A. S., Bacchini, S., Gasparetto, V., Ferraiuolo, G., Campana, C., Bonatti, R., Gaita, F., Bergerone, S., Bonmassari, R., Zeni, P., Langialonga, T., Scarcia, A., Caravita, L., Musacchio, E., Augello, G., Usmiani, T., Stomaci, B., Cirino, D., Pierini, S., Bottiglieri, G., Liso, A., Mussardo, M., Tosi, P., Sala, R., Belloni, A., Blengino, S., Lisi, E., Delfino, P., Auguadro, C., Brunazzi, M. C., Pacchioni, E., Fattore, L., Bosco, B., Blandizzi, S., Pajes, G., Patruno, N., Perna, G. P., Francioni, M., Favale, S., Vestito, D., Lombardi, A., Capecchi, A., Ferrero, P., De Vincenzo, C., Magri, G., Indolfi, C., De Rosa, S., Rossi, M., Collarini, L., Agnelli, D., Conti, G., Tonelli, C., Spadaro, C., Negroni, S., Di Noto, G., Lanari, A., Casolo, G., Del Meglio, J., Negrini, M., Celentano, A., Sifola, C., Rellini, G., Della Mattia, A., Molero, U., Piovaccari, G., Grosseto, D., Callegarin, L., Fiasconaro, G., Crivello, R., Thiebat, B., Leone, G., Tamburino, C., Caruso, G., Cassadonte, F., Sassone, B., Fuca, G., Sormani, L., Percoco, G. F., Mazzucco, R., Cazzani, E., Gianni, M., Limido, A., Luvini, M., Guglielmi, R., Mannarini, A., Moruzzi, P., Pastori, P., Golia, B., Marzano, A., Orazi, S., Marchese, I., Anselmi, M., Girardi, P., Nassiacos, D., Meloni, S., Busacca, P., Generali, C. A., Corda, S., Costanza, G., Montalto, S., Argenziano, L., Tommasini, P., Emdin, M., Pasanisi, E. M., Colivicchi, F., Tubaro, M., Azzolini, P., Luciani, C., Doronzo, B., Coppolino, A., Dellavesa, P., Zenone, F., Di Marco, A., De Conti, F., Piccinni, G. C., Gualtieri, M. R., Bisignani, G., Leone, A., Arcuri, G. M., Marinacci, L., Rossi, P., Perotti, S., Cotti Cometti, V., Arcidiacono, S., Tramontana, M., Bazzucchi, M., Mezzetti, P., Romano, M., Villani, R., Di Giovambattista, R., Volpe, B., Tedesco, L., Carini, M., Vinci, S., Paolini, E. A., Busoni, F., Piergentili, C., Navazio, A., Manca, F., Cocco, F., Pennetta, C. A., Maggiolini, S., Galbiati, R., Bruna, C., Ferrero, L., Brigido, S., Barducci, E., Musacchio, D., Manduca, B., Marchese, D., Patrassi, L. A., Pattarino, F. A., Rocchi, M., Briglia, S., Fanelli, R., Villella, M., Gronda, E., Massa, D., Lenti, V., Di Gregorio, L., Bottero, M., Bazzanini, F., Braggion, G., Antoniceli, R., Caraceni, D., Guzzo, V., Di Giovanni, P., Scarpini, S., Severgnini, B., Musolino, M. F., Della Casa, S., Gobbi, M., Arena, G., Bonizzato, S., Agnoletto, V., Sansoni, S., Pes, R. A. M., Denti, S., Polizzi, G. M., Pino, R., Commisso, B., Merlino, A., Di Lorenzo, L., Porchetta, I., Del Furia, F., Colombi, E., Covini, D., Cavalieri, F., Antonaci, S., Rubino, G., Ciulla, A., Bui, F., Casorelli, E., Caliendo, L., Laezza, A., Americo, L., Schillaci, A. M., Cordoni, M., Barsotti, L., Gaudio, C., Barilla, F., Cannone, M., Memeo, R., Truncellito, L., Andriani, A., Salituri, S., Verrina, F., Pafi, M., Sebastiani, M. L., Amico, A. F., Scolozzi, D., D'Alea, A., Catanzariti, D., Angheben, C., Ottaviano, A., and Levantesi, G.

Subjects
Male, Ticagrelor, medicine.medical_specialty, Acute coronary syndrome, medicine.medical_treatment, Conservative strategy, Population, Acute coronary syndromes, Revascularization, acute coronary syndromes, anticoagulant, antithrombotic therapy, conservative strategy, prasugrel, ticagrelor, aged, coronary care units, female, fibrinolytic agents, follow-up studies, hospital mortality, humans, iItaly, length of stay, male, myocardial revascularization, retrospective studies, survival rate, thrombolytic therapy, practice guidelines as topic, Fibrinolytic Agents, Anticoagulant, Antithrombotic therapy, Prasugrel, Acute Coronary Syndrome, Aged, Coronary Care Units, Female, Follow-Up Studies, Hospital Mortality, Humans, Italy, Length of Stay, Myocardial Revascularization, Retrospective Studies, Survival Rate, Thrombolytic Therapy, Practice Guidelines as Topic, Cardiology and Cardiovascular Medicine, Pharmacology (medical), Internal medicine, Antithrombotic, medicine, education, Survival rate, education.field_of_study, business.industry, Clopidogrel, medicine.disease, Cardiology, business, Fibrinolytic agent, medicine.drug
Abstract

Aims Patients with acute coronary syndromes (ACSs) who are managed without coronary revascularization represent a mixed and understudied population that seems to receive suboptimal pharmacological treatment. Methods and results We assessed patterns of antithrombotic therapies employed during the hospitalization and in-hospital clinical events of medically managed patients with ACS enrolled in the prospective, multicentre, nationwide EYESHOT (EmploYEd antithrombotic therapies in patients with acute coronary Syndromes HOspitalized in iTalian cardiac care units) registry. Among the 2585 consecutive ACS patients enrolled in EYESHOT, 783 (30.3%) did not receive any revascularization during hospital admission. Of these, 478 (61.0%) underwent coronary angiography (CA), whereas 305 (39.0%) did not. The median GRACE and CRUSADE risk scores were significantly higher among patients who did not undergo CA compared with those who did (180 vs. 145, P < 0.0001 and 50 vs. 33, P < 0.0001, respectively). Antithrombotic therapies employed during hospitalization significantly differ between patients who received CA and those who did not with unfractioned heparin and novel P2Y12 inhibitors more frequently used in the first group, and low-molecular-weight heparins and clopidogrel in the latter group. During the index hospitalization, patients who did not receive CA presented a higher incidence of ischaemic cerebrovascular events and of mortality compared with those who underwent CA (1.6 vs. 0.2%, P = 0.04 and 7.9 vs. 2.7%, P = 0.0009, respectively). Conclusion Almost one-third of ACS patients are managed without revascularization during the index hospitalization. In this population, a lower use of recommended antiplatelet therapy and worse clinical outcome were observed in those who did not undergo CA when compared with those who did. Clinical Trial Registration Unique identifier: [NCT02015624][1], . [10.1093/ehjcvp/pvv017][2] [1]: /lookup/external-ref?link_type=CLINTRIALGOV&access_num=NCT02015624&atom=%2Fehjcardpharm%2F1%2F3%2F168.atom [2]: /lookup/doi/10.1093/ehjcvp/pvv017

Published
2015

30. Spatial QT dispersion predicts non-sustained ventricula tachycardia and correlates with a confined systo-diastolic dysfunction un hypertrophic cardiomyopathy

Author

Magri, D, Piccirillo, G, Ricotta, A, De Cecco, C, Mastromarino, V, Serdoz, A, Muscogiuri, G, Gregori, M, Casenghi, M, Cauti, F, Oliviero, G, Musumeci, M, Maruotti, A, Autore, C, Magri D., Piccirillo G., Ricotta A., De Cecco C. N., Mastromarino V., Serdoz A., Muscogiuri G., Gregori M., Casenghi M., Cauti F. M., Oliviero G., Musumeci M. B., Maruotti A., Autore C., Magri, D, Piccirillo, G, Ricotta, A, De Cecco, C, Mastromarino, V, Serdoz, A, Muscogiuri, G, Gregori, M, Casenghi, M, Cauti, F, Oliviero, G, Musumeci, M, Maruotti, A, Autore, C, Magri D., Piccirillo G., Ricotta A., De Cecco C. N., Mastromarino V., Serdoz A., Muscogiuri G., Gregori M., Casenghi M., Cauti F. M., Oliviero G., Musumeci M. B., Maruotti A., and Autore C.

Abstract

Objectives: An increased dispersion of myocardial repolarization represents one of the mechanisms underlying the arrhythmic risk in hypertrophic cardiomyopathy (HCM). We investigated spatial myocardial repolarization dispersion indices in HCM patients with nonsustained ventricular tachycardia (NSVT) and, contextually, their main clinical determinants. Methods: Fifty-two well-matched HCM outpatients were categorized into two groups according to the presence or the absence of NSVT at 24-hour Holter electrocardiogram (ECG) monitoring. Each patient underwent a clinical examination, including Doppler echocardiogram integrated with tissue Doppler imaging, cardiac magnetic resonance, and 12-lead surface ECG to calculate the dispersion for the following intervals: QRS, Q-Tend (QTe), Q-Tpeak, Tpeak-Tend (TpTe), J-Tpeak, and J-Tend. Results: The NSVT group showed only QTe dispersion and TpTe dispersion values to be significantly higher than their counterparts. NSVT occurrence was independently predicted by late gadolinium enhancement presence (p = 0.021) and QTe Bazett dispersion (p = 0.030), the latter strongly associated with the myocardial performance index (MPI) obtained at the basal segment of the interventricular septum (p = 0.0004). Conclusion: Our data support QTe dispersion as an easy and noninvasive tool for identifying HCM patients with NSVT propensity. The strong relationship between QTe dispersion and MPI allows us to hypothesize an intriguing link between electrical instability and confined myocardial areas of systodiastolic dysfunction.

Published
2015

31. Prevalence of Anderson-Fabry disease in patients with hypertrophic cardiomyopathy: the European Anderson-Fabry Disease Survey

Author

Elliott P, Baker R, Quarta G, Ebrahim H, Mehta AB, Hughes DA, ACES study group, Anastasakis A, Autore C, Musumeci MB, Frenneaux M, Gimeno J, Tiina H, Kuusisto J, Aalto Setäla K, McKeown P, Monserrat L, Fernandez X, Pacileo G, Limongelli G, Cate FJ, Wilde AA, Pinto YM, Christiaans I, Zachara E., PASQUALE, FERDINANDO, RAPEZZI, CLAUDIO, BIAGINI, ELENA, Elliott P, Baker R, Pasquale F, Quarta G, Ebrahim H, Mehta AB, Hughes DA, ACES study group, Anastasakis A, Autore C, Musumeci MB, Frenneaux M, Gimeno J, Tiina H, Kuusisto J, Aalto-Setäla K, McKeown P, Monserrat L, Fernandez X, Pacileo G, Limongelli G, Rapezzi C, Biagini E, Cate FJ, Wilde AA, Pinto YM, Christiaans I, Zachara E., ACS - Amsterdam Cardiovascular Sciences, Cardiology, and Human Genetics

Subjects
Male, Pathology, medicine.medical_specialty, Cross-sectional study, Gene mutation, Left ventricular hypertrophy, NO, Internal medicine, Epidemiology, Prevalence, medicine, Humans, Genetic Testing, cardiovascular diseases, Aged, Fabry disease, HYPERTROPHIC CARDIOMYOPATHY, business.industry, Hypertrophic cardiomyopathy, SURVEY, Enzyme replacement therapy, Cardiomyopathy, Hypertrophic, Middle Aged, medicine.disease, Europe, Cross-Sectional Studies, alpha-Galactosidase, Mutation, Cohort, Female, Cardiology and Cardiovascular Medicine, business
Abstract

Objectives The prevalence of Anderson–Fabry disease (AFD) in patients presenting with unexplained left ventricular hypertrophy (LVH) is controversial. The aim of this study was to determine the prevalence of AFD in a large, consecutive cohort of patients with hypertrophic cardiomyopathy (HCM) using rapid mutation screening. Design, Setting and Patients A European multicentre cross-sectional study involving 13 referral centres. Inclusion criteria for the study were: men aged at least 35 years and women aged at least 40 years with unexplained LVH (maximum left ventricular wall thickness ≥1.5 cm). All patients were screened using a denaturing high-performance liquid chromatography protocol for rapid mutation screening of the α-galactosidase A (α-Gal A) gene and, if a sequence variant was found, direct sequencing was performed. 1386 patients (63.9% men, mean age 57.9±12.0 years) were enrolled in the study. Results Seven (0.5%) patients (age 57.4±9.0 years (45–72); three (43%) men) had pathogenic α-galactosidase A mutations. Polymorphisms were identified in 283 patients (20.4%). Maximal left ventricular wall thickness in patients carrying a disease-causing mutation was 18±2 mm (range 15–22); four patients had concentric LVH and the remainder had asymmetric septal hypertrophy. Conclusions The prevalence of AFD gene mutations in a large, consecutive cohort of European patients with unexplained LVH is 0.5%.

Published
2011

32. Antithrombotic strategies in the catheterization laboratory for patients with acute coronary syndromes undergoing percutaneous coronary interventions: insights from the EmploYEd antithrombotic therapies in patients with acute coronary Syndromes HOspitalized in iTalian cardiac care units Registry

Author

De Luca, L., Musumeci, G., Leonardi, S., Gonzini, L., Cavallini, C., Calabro, P., Mauro, C., Cacciavillani, L., Savonitto, S., De Servi, S., Caporale, R., Ceravolo, R., Formigli, D., Lupi, A., Rakar, S., Smecca, I. M., Maggioni, A. P., Lucci, D., Lorimer, A., Orsini, G., Fabbri, G., Bianchini, E., Abrignani, M. G., Bonura, F., Trimarco, B., Galasso, G., Misuraca, G., Manes, M. T., Tuccillo, B., Irace, L., Olivari, Z., Totis, O., Ledda, A., Boccalatte, M., Iliceto, S., Tortorella, G., Esposito, L., De Rosa, P., Bianchi, R., Napoletano, C., Piccioni, L. L., Pavesi, P. C., Bovenzi, F. M., Boni, A., Merenda, R., Wolff, S., De Ferrari, G. M., Camporotondo, R., Gambino, P., Cutaia, A., Picariello, C., Cemin, R., Chiarella, F., Gauthier, L. G., Mircoli, L., Del Pinto, M., Finocchiaro, M. L., Scioli, R., Farina, R., Naddeo, C., Scherillo, M., Santopietro, S., Metra, M., Costa, F., Calculli, G., Troito, G., Pennisi, V., Adornato, E. M. F., Pirelli, S., Fadin, B. M., DI Biase, M., Ieva, R., Zuin, G., Sanfilippo, N., Mancuso, L., Pani, A., Serra, E., Marenzi, G., Assanelli, E. M., Ansalone, G., Cacciotti, L., Morocutti, G., Fresco, C., Berti, S., Paradossi, U., Bozzano, A., Mauro, A., Noussan, P., Zanini, P., Bolognese, L., Falsini, G., Costa, P., Manca, G., Caldarola, P., Locuratolo, N., Cipolla, T., Becchina, M., Cocco, G., Scalera, G., Stefanelli, S., Giunta, N., Sinagra, G., Meloni, L., Lai, O., Chiaranda, G., Luca, G., Helou, J. S., Biscottini, E., Magliari, F., Callerame, M., Uguccioni, M., Pugliese, M., Sanchez, F., Tartaglione, S., Ignone, G., Mavilio, G., Mantovan, R., Bini, R., Caico, S. I., Demolli, V., Proietti, F., Michisanti, M., Musmeci, G., Cantamessa, P., Sicuso, G., Micalef, S. S., Accogli, M., Zaccaria, M., Caputo, M., DI Paolo, G., Piatti, L., Farina, A., Vicinelli, P., Paloscia, L., DI Clemente, D., Felis, S., Castini, D., Rota, C., Casu, G., Bonano, S., Margheri, M., Lucchi, G. R., Serdoz, R., Proietti, P., Autore, C., Conti, E., Russo, V., Orlando, P., Ramondo, A. B., Bontorin, M., Marcolongo, M., Santagostino, M., Maestroni, A., Vitti, P., Rodella, P., Bonetti, P., Elia, M., Lumare, R., Politi, A., Gritti, S., Poletti, F., Mafrici, A., Fusco, R., Bongo, A. S., Bacchini, S., Gasparetto, V., Ferraiuolo, G., De Luca, M., Campana, C., Bonatti, R., Gaita, F., Bergerone, S., Bonmassari, R., Zeni, P., Langialonga, T., Scarcia, A., Caravita, L., Musacchio, E., Augello, G., Usmiani, T., Stomaci, B., Cirino, D., Pierini, S., Bottiglieri, G., Liso, A., Mussardo, M., Tosi, P., Sala, R., Belloni, A., Blengino, S., Lisi, E., Delfino, P., Auguadro, C., Brunazzi, M. C., Pacchioni, E., Fattore, L., Bosco, B., Blandizzi, S., Pajes, G., Patruno, N., Perna, G. P., Francioni, M., Favale, S., Vestito, D., Lombardi, A., Capecchi, A., Ferrero, P., De Vincenzo, C., Magri, G., Indolfi, C., De Rosa, S., Rossi, M., Collarini, L., Agnelli, D., Conti, G., Tonelli, C., Spadaro, C., Negroni, S., DI Noto, G., Lanari, A., Casolo, G., Del Meglio, J., Negrini, M., Celentano, A., Sifola, C., Rellini, G., Mattia, A. D., Molero, U., Piovaccari, G., Grosseto, D., Callegarin, L., Fiasconaro, G., Crivello, R., Thiebat, B., Leone, G., Tamburino, C., Caruso, G., Cassadonte, F., Sassone, B., Fuca, G., Sormani, L., Percoco, G. F., Mazzucco, R., Cazzani, E., Gianni, M., Limido, A., Luvini, M., Guglielmi, R., Mannarini, A., Moruzzi, P., Pastori, P., Golia, B., Marzano, A., Orazi, S., Marchese, I., Anselmi, M., Girardi, P., Nassiacos, D., Meloni, S., Busacca, P., Generali, C. A., Corda, S., Costanza, G., Montalto, S., Argenziano, L., Tommasini, P., Emdin, M., Pasanisi, E. M., Colivicchi, F., Tubaro, M., Azzolini, P., Luciani, C., Doronzo, B., Coppolino, A., Dellavesa, P., Zenone, F., DI Marco, A., De Conti, F., Piccinni, G. C., Gualtieri, M. R., Bisignani, G., Leone, A., Arcuri, G. M., Marinacci, L., Rossi, P., Perotti, S., Cometti, V. C., Arcidiacono, S., Tramontana, M., Bazzucchi, M., Mezzetti, P., Romano, M., Villani, R., DI Giovambattista, R., Volpe, B., Tedesco, L., Carini, M., Vinci, S., Paolini, E. A., Busoni, F., Piergentili, C., Navazio, A., Manca, F., Cocco, F., Pennetta, C. A., Maggiolini, S., Galbiati, R., Bruna, C., Ferrero, L., Brigido, S., Barducci, E., Musacchio, D., Manduca, B., Marchese, D., Patrassi, L. A., Pattarino, F. A., Rocchi, M., Briglia, S., Fanelli, R., Villella, M., Gronda, E., Massa, D., Lenti, V., DI Gregorio, L., Bottero, M., Bazzanini, F., Braggion, G., Antoniceli, R., Caraceni, D., Guzzo, V., DI Giovanni, P., Scarpini, S., Severgnini, B., Musolino, M. F., Casa, S. D., Gobbi, M., Arena, G., Bonizzato, S., Agnoletto, V., Sansoni, S., Pes, R. A. M., Denti, S., Polizzi, G. M., Pino, R., Commisso, B., Merlino, A., DI Lorenzo, L., Porchetta, I., Del Furia, F., Colombi, E., Covini, D., Cavalieri, F., Antonaci, S., Rubino, G., Ciulla, A., Bui, F., Casorelli, E., Caliendo, L., Laezza, A., Americo, L., Schillaci, A. M., Cordoni, M., Barsotti, L., Gaudio, C., Barilla, F., Cannone, M., Memeo, R., Truncellito, L., Andriani, A., Salituri, S., Verrina, F., Pafi, M., Sebastiani, M. L., Amico, A. F., Scolozzi, D., Lupi, G., D'Alea, A., Catanzariti, D., Angheben, C., Ottaviano, A., Levantesi, G., de Luca, Leonardo, Musumeci, Giuseppe, Leonardi, Sergio, Gonzini, Lucio, Cavallini, Claudio, Calabrò, Paolo, Mauro, Ciro, Cacciavillani, Luisa, Savonitto, Stefano, de Servi, Stefano, Caporale, Roberto, Ceravolo, Roberto, Formigli, Dario, Lupi, Alessandro, Rakar, Sadir, Smecca, Ivan, Maggioni, Aldo Pietro, Lucci, Donata, Lorimer, Andrea, Orsini, Giampietro, Fabbri, Gianna, Bianchini, Elisa, Abrignani, Maurizio Giuseppe, Bonura, Francesc, Trimarco, Bruno, Galasso, Gennaro, Misuraca, Gianfranco, Manes, Maria Teresa, Tuccillo, Bernardino, and Irace, Luigi.

Subjects
Male, Prasugrel, medicine.medical_treatment, Myocardial Infarction, antithrombotic therapy, 030204 cardiovascular system & hematology, acute coronary syndromes, bivalirudin, heparins, percutaneous coronary intervention, prasugrel, ticagrelor, 0302 clinical medicine, Antithrombotic, 80 and over, Bivalirudin, 030212 general & internal medicine, Myocardial infarction, Prospective Studies, Registries, Aged, 80 and over, General Medicine, Hirudins, Middle Aged, Recombinant Proteins, Italy, Female, Cardiology and Cardiovascular Medicine, Ticagrelor, medicine.drug, medicine.medical_specialty, Platelet Glycoprotein GPIIb-IIIa Complex, NO, 03 medical and health sciences, Percutaneous Coronary Intervention, Internal medicine, medicine, Humans, Acute Coronary Syndrome, Aged, Aspirin, business.industry, Heparin, Percutaneous coronary intervention, Anticoagulants, medicine.disease, Peptide Fragments, Clinical trial, Cross-Sectional Studies, Logistic Models, Conventional PCI, Multivariate Analysis, business
Abstract

Aims In the last decades, several new therapies have emerged for the treatment of acute coronary syndromes (ACS). We sought to describe real-world patterns of use of antithrombotic treatments in the catheterization laboratory for ACS patients undergoing percutaneous coronary interventions (PCI). Methods EmploYEd antithrombotic therapies in patients with acute coronary Syndromes HOspitalized in iTalian cardiac care units was a nationwide, prospective registry aimed to evaluate antithrombotic strategies employed in ACS patients in Italy. Results Over a 3-week period, a total of 2585 consecutive ACS patients have been enrolled in 203 cardiac care units across Italy. Among these patients, 1755 underwent PCI (923 with ST-elevation myocardial infarction and 832 with non-ST-elevation ACS). In the catheterization laboratory, unfractioned heparin was the most used antithrombotic drug in both ST-elevation myocardial infarction (64.7%) and non-ST-elevation ACS (77.5%) undergoing PCI and, as aspirin, bivalirudin and glycoprotein IIb/IIIa inhibitors (GPIs) more frequently employed before or during PCI compared with the postprocedural period. Any crossover of heparin therapy occurred in 36.0% of cases, whereas switching from one P2Y12 inhibitor to another occurred in 3.7% of patients. Multivariable analysis yielded several independent predictors of GPIs and of bivalirudin use in the catheterization laboratory, mainly related to clinical presentation, PCI complexity and presence of complications during the procedure. Conclusion In our contemporary, nationwide, all-comers cohort of ACS patients undergoing PCI, antithrombotic therapies were commonly initiated before the catheterization laboratory. In the periprocedural period, the most frequently employed drugs were unfractioned heparin, leading to a high rate of crossover, followed by GPIs and bivalirudin, mainly used during complex PCI. Clinical trial registration URL: http://www.clinicaltrials.gov. Unique identifier: NCT02015624.

Published
2017

33. SPATIAL QT DISPERSION PREDICTS NON-SUSTAINED VENTRICULAR TACHYCARDIA AND CORRELATES WITH A CONFINED SYSTO-DIASTOLIC DYSFUNCTION IN HYPERTROPHIC CARDIOMYOPATHY

Author

Magrì D, Piccirillo G, Ricotta A, De Cecco CN, Mastromarino V, Serdoz A, Muscogiuri G, Gregori M, Casenghi M, Cauti FM, Oliviero G, Musumeci MB, Autore C., MARUOTTI, ANTONELLO, Magrì, D, Piccirillo, G, Ricotta, A, De Cecco, Cn, Mastromarino, V, Serdoz, A, Muscogiuri, G, Gregori, M, Casenghi, M, Cauti, Fm, Oliviero, G, Musumeci, Mb, Maruotti, Antonello, and Autore, C.

Published
2015

34. THE BROAD PHENOTYPIC SPECTRUM OF ARRHYTHMOGENIC CARDIOMYOPATHY

Author

Mistrulli, R, Salerno, L, Tiberi, P, Francia, P, Luca, P, Autore, C, Rubattu, S, Barbato, E, and Pagannone, E

Abstract

Arrhythmogenic cardiomyopathy (ACM) is a rare inherited cardiomyopathy (CMP) characterized by progressive fibro–adipose replacement of the myocardium. It is mainly caused by variants in desmosomal genes with autosomal dominant transmission and incomplete penetrance. Originally, ACM was considered a CMP with main if not exclusive involvement of the right ventricle (RV) and more rarely both ventricles. To date, recent data have also highlighted the possibility of isolated left ventricular (LV) involvement, which often results in a disease with peculiar and distinct features. The case we propose is that of a 70–year–old man with no cardiology history but a family history of cardiomyopathy (brother has unspecified cardiomyopathy and is an ICD carrier). In 2015 due to occasional finding of ventricular extrasystole, the patient underwent CVG which was negative and cardiac MRI which documented an area of late–Gadolinium Enhancement (LGE) of the mid–basal portion of the inferior and lateral wall, with greater involvement of the subepicardial versant of the LV of possible post–myocarditis involvement. In September 2023, he was admitted to our cardiology department following a syncopal episode not preceded by prodromes. The ECG showed poor R–wave progression, low voltages and diffuse repolarization changes. The echocardiogram showed dilated LV with reduced EF(35%). Both CVG and cardiac MRI was repeated. The first was still negative and cardiac MRI showed a considerable progression of LGE areas at the mesomyocardial and subendocardial levels, with an almost ubiquitous distribution in the LV. Additional areas of LGE were also appreciated at the atrioventricular valves, the interatrial septum, and in the RV, but the latter was not dysfunctional. These findings appeared compatible with LV ACM with initial biventricular extension. It was decided to implant ICD in primary prevention and to request genetic analysis, which is still ongoing. Beyond the unusual appearance in extent and locations of areas of LGE on cardiac MRI, our clinical case highlights the close correlation that is often present between left ACM and acute myocarditis. It is not well known whether the inflammation associated with acute myocarditis is a primary injury that results in fibroadipose replacement or whether it is the degeneration of the cardiomyocytes themselves that results in these inflammatory phases (hot–phases) with myocarditis–like expression.

Published
2024
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35. Myocardial repolarization dispersion and late gadolinium enhancement in patients with hypertrophic cardiomyopathy

Author

Magri, D, De Cecco, C, Piccirillo, G, Mastromarino, V, Serdoz, A, Muscogiuri, G, Ricotta, A, Gregori, M, Marino, L, Cauti, F, Pagannone, E, Musumeci, M, Maruotti, A, Autore, C, Magri D., De Cecco C. N., Piccirillo G., Mastromarino V., Serdoz A., Muscogiuri G., Ricotta A., Gregori M., Marino L., Cauti F. M., Pagannone E., Musumeci M. B., Maruotti A., Autore C., Magri, D, De Cecco, C, Piccirillo, G, Mastromarino, V, Serdoz, A, Muscogiuri, G, Ricotta, A, Gregori, M, Marino, L, Cauti, F, Pagannone, E, Musumeci, M, Maruotti, A, Autore, C, Magri D., De Cecco C. N., Piccirillo G., Mastromarino V., Serdoz A., Muscogiuri G., Ricotta A., Gregori M., Marino L., Cauti F. M., Pagannone E., Musumeci M. B., Maruotti A., and Autore C.

Abstract

Background: Growing evidence suggests that late gadolinium enhancement (LGE) at cardiac magnetic resonance (CMR) is an additive marker of disease severity, and possibly of arrhythmic risk, in hypertrophic cardiomyopathy (HCM). We investigated the possible relationship between LGE and markers of myocardial repolarization dispersion in HCM. Methods and Results: Eighty-five HCM outpatients underwent CMR and short-period electrocardiogram analysis to calculate the temporal myocardial repolarization dispersion through the QT variance normalized for QT mean (QTVN) and the QT variability index (QTVI). The QT dispersion in the spatial domain was also obtained. Patients with LGE (62%) had higher left atrial volume, maximum wall thickness, and left ventricular mass (P<0.0001), as well as a greater prevalence of non-sustained ventricular tachycardia (P<0.0001) and hypotensive blood pressure response (P=0.044). Both QTVN and QTVI were higher in the group with LGE (P<0.0001). At multivariate analysis, using QTVI as the dependent variable, %LGE (P<0.0001), age (P<0.0001), left ventricular outflow obstruction (P=0.038), and sudden cardiac death risk factor burden (P=0.020) reached statistical significance. Otherwise, only %LGE (P=0.005) and left ventricular mass index (P=0.015) remained associated with QTVN. Conclusions: Temporal myocardial repolarization dispersion correlates with LGE extent. Whether these variables could be useful in HCM clinical management warrants confirmation by larger prospective studies.

Published
2014

36. Relationship between myocardial repolarization dispersion and late gadolinium enhancement in patients with hypertrophic cardiomyopathy

Author

Magrì D, De Cecco C. N, Piccirillo G, Mastromarino V, Serdoz A, Muscogiuri G, Ricotta A, Gregori M, Marino L, Cauti F. M, Pagannone E, Musumeci M. B, Autore C., MARUOTTI, ANTONELLO, Magrì, D, De Cecco C., N, Piccirillo, G, Mastromarino, V, Serdoz, A, Muscogiuri, G, Ricotta, A, Gregori, M, Marino, L, Cauti F., M, Pagannone, E, Musumeci M., B, Maruotti, Antonello, and Autore, C.

Published
2014

40. Prevention of sudden cardiac death with implantable cardioverter- defibrillators in children and adolescents with hypertrophic cardiomyopathy

Author

Maron, B.J. Spirito, P. Ackerman, M.J. Casey, S.A. Semsarian, C. Estes III, N.A.M. Shannon, K.M. Ashley, E.A. Day, S.M. Pacileo, G. Formisano, F. Devoto, E. Anastasakis, A. Bos, J.M. Woo, A. Autore, C. Pass, R.H. Boriani, G. Garberich, R.F. Almquist, A.K. Russell, M.W. Boni, L. Berger, S. Maron, M.S. Link, M.S.

Abstract

Objectives: The aim of this study was to determine the efficacy of implantable cardioverter-defibrillators (ICDs) in children and adolescents with hypertrophic cardiomyopathy (HCM). Background: HCM is the most common cause of sudden death in the young. The availability of ICDs over the past decade for HCM has demonstrated the potential for sudden death prevention, predominantly in adult patients. Methods: A multicenter international registry of ICDs implanted (1987 to 2011) in 224 unrelated children and adolescents with HCM judged at high risk for sudden death was assembled. Patients received ICDs for primary (n = 188) or secondary (n = 36) prevention after undergoing evaluation at 22 referral and nonreferral institutions in the United States, Canada, Europe, and Australia. Results: Defibrillators were activated appropriately to terminate ventricular tachycardia or ventricular fibrillation in 43 of 224 patients (19%) over a mean of 4.3 ± 3.3 years. ICD intervention rates were 4.5% per year overall, 14.0% per year for secondary prevention after cardiac arrest, and 3.1% per year for primary prevention on the basis of risk factors (5-year cumulative probability 17%). The mean time from implantation to first appropriate discharge was 2.9 ± 2.7 years (range to 8.6 years). The primary prevention discharge rate terminating ventricular tachycardia or ventricular fibrillation was the same in patients who underwent implantation for 1, 2, or ≥3 risk factors (12 of 88 [14%], 10 of 71 [14%], and 4 of 29 [14%], respectively, p = 1.00). Extreme left ventricular hypertrophy was the most common risk factor present (alone or in combination with other markers) in patients experiencing primary prevention interventions (17 of 26 [65%]). ICD-related complications, particularly inappropriate shocks and lead malfunction, occurred in 91 patients (41%) at 17 ± 5 years of age. Conclusions: In a high-risk pediatric HCM cohort, ICD interventions terminating life-threatening ventricular tachyarrhythmias were frequent. Extreme left ventricular hypertrophy was most frequently associated with appropriate interventions. The rate of device complications adds a measure of complexity to ICD decisions in this age group. © 2013 American College of Cardiology Foundation.

Published
2013

45. Update on phase II studies of erythropoietin in acute myocardial infarction. Rationale and design of Exogenous erythroPoietin in Acute Myocardial Infarction: New Outlook aNd Dose Association Study (EPAMINONDAS)

Author

Andreotti, Felicita, Agati, L, Conti, Elena, Santucci, Eleonora, Rio, Teresa, Tarantino, Federica, Natale, Luigi, Berardi, Donato, Mattatelli, A, Musumeci, B, Bonomo, Lorenzo, Volpe, M, Crea, F, Autore, C, Andreotti, Felicita (ORCID:0000-0002-1456-6430), Natale, Luigi (ORCID:0000-0002-7949-5119), Bonomo, Lorenzo (ORCID:0000-0001-5101-9367), Crea, F (ORCID:0000-0001-9404-8846), Andreotti, Felicita, Agati, L, Conti, Elena, Santucci, Eleonora, Rio, Teresa, Tarantino, Federica, Natale, Luigi, Berardi, Donato, Mattatelli, A, Musumeci, B, Bonomo, Lorenzo, Volpe, M, Crea, F, Autore, C, Andreotti, Felicita (ORCID:0000-0002-1456-6430), Natale, Luigi (ORCID:0000-0002-7949-5119), Bonomo, Lorenzo (ORCID:0000-0001-5101-9367), and Crea, F (ORCID:0000-0001-9404-8846)

Abstract

Erythropoietin (Epo) is a hematopoietic hormone produced mainly by the kidneys in response to hypoxia. Recent acquisitions in the fields of hematology, neurology, cardiology, and experimental medicine show cytoprotective, angiogenetic and antiinflammatory effects of Epo. Exogenous erythroPoietin in Acute Myocardial Infarction: New Outlook aNd Dose Association Study (EPAMINONDAS, EudraCTno. 200500485386) is one of four ongoing randomized controlled trials, each testing the effects of Epo in >or=100 patients with STEMI. EPAMINONDAS is a multicenter, prospective, double-blind, placebo-controlled, dose-finding study assessing intravenous moderate doses of human recombinant Epo (epoietin-alpha, 100 or 200 IU/kg/die) versus placebo, given on the first 3 days, in 102 patients with first ST-segment elevation myocardial infarction. Initial dosing is within 12 h of primary percutaneous coronary revascularization. The primary endpoint is infarct size, quantified by CK-MB time-concentration curve, left ventricular wall motion score index, and pattern of contrast-enhanced magnetic resonance imaging. Secondary endpoints are ischemic recurrences, ventricular remodelling, and safety events, assessed in-hospital and at 12 months' follow-up. The results of current phase II studies will help define the safety/efficacy profile of Epo for patients with STEMI.

Published
2009

48. Pulmonary embolism in pregnancy

Author

Conti, E., primary, Zezza, L., additional, Ralli, E., additional, Comito, C., additional, Sada, L., additional, Passerini, J., additional, Caserta, D., additional, Rubattu, S., additional, Autore, C., additional, Moscarini, M., additional, and Volpe, M., additional

Published
2013
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49. Simultaneous Takotsubo syndrome in two sisters

Author

Musumeci, B., primary, Saponaro, A., additional, Pagannone, E., additional, Proietti, G., additional, Mastromarino, V., additional, Conti, E., additional, Tubaro, M., additional, Volpe, M., additional, and Autore, C., additional

Published
2013
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50. 'QRS duration and QRS fractionation on surface electrocardiogram are markers of right ventricular dysfunction and atrialization in patients with Ebstein anomaly' [Eur Heart J 2012;34:191-200, doi:10.1093/eurheartj/ehs362]

Author

Assenza, G. E., primary, Valente, A. M., additional, Geva, T., additional, Graham, D., additional, Pluchinotta, F. R., additional, Sanders, S. P., additional, Autore, C., additional, Volpe, M., additional, Landzberg, M. J., additional, and Cecchin, F., additional

Published
2013
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