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1. Candidacy and long-term outcomes of subcutaneous implantable cardioverter-defibrillators in current practice in patients with hypertrophic cardiomyopathy

Author

Rella, V., Maurizi, N., Bernardini, A., Brasca, F.M., Salerno, S., Meda, M., Mariani, D., Torchio, M., Ravaro, S., Cerea, P., Castelletti, S., Fumagalli, C., Conte, G., Auricchio, A., Girolami, F., Pieragnoli, P., Carrassa, G.M., Parati, G., Olivotto, I., Perego, G.B., Cecchi, F., and Crotti, L.

Published
2024
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3. Candidacy and long-term outcomes of subcutaneous implantable cardioverter-defibrillators in current practice in patients with hypertrophic cardiomyopathy

Author

Rella, V, Maurizi, N, Bernardini, A, Brasca, F, Salerno, S, Meda, M, Mariani, D, Torchio, M, Ravaro, S, Cerea, P, Castelletti, S, Fumagalli, C, Conte, G, Auricchio, A, Girolami, F, Pieragnoli, P, Carrassa, G, Parati, G, Olivotto, I, Perego, G, Cecchi, F, Crotti, L, Rella V., Maurizi N., Bernardini A., Brasca F. M., Salerno S., Meda M., Mariani D., Torchio M., Ravaro S., Cerea P., Castelletti S., Fumagalli C., Conte G., Auricchio A., Girolami F., Pieragnoli P., Carrassa G. M., Parati G., Olivotto I., Perego G. B., Cecchi F., Crotti L., Rella, V, Maurizi, N, Bernardini, A, Brasca, F, Salerno, S, Meda, M, Mariani, D, Torchio, M, Ravaro, S, Cerea, P, Castelletti, S, Fumagalli, C, Conte, G, Auricchio, A, Girolami, F, Pieragnoli, P, Carrassa, G, Parati, G, Olivotto, I, Perego, G, Cecchi, F, Crotti, L, Rella V., Maurizi N., Bernardini A., Brasca F. M., Salerno S., Meda M., Mariani D., Torchio M., Ravaro S., Cerea P., Castelletti S., Fumagalli C., Conte G., Auricchio A., Girolami F., Pieragnoli P., Carrassa G. M., Parati G., Olivotto I., Perego G. B., Cecchi F., and Crotti L.

Abstract

Background: In patients with Hypertrophic Cardiomyopathy (HCM) S-ICD is usually the preferred option as pacing is generally not indicated. However, limited data are available on its current practice adoption and long-term follow-up. Methods: Consecutive HCM patients with S-ICD implanted between 2013 and 2021 in 3 international centers were enrolled in this observational study. Baseline, procedural and follow-up data were regularly collected. Efficacy and safety were compared with a cohort of HCM patients implanted with a tv-ICD. Results: Seventy patients (64% males) were implanted with S-ICD at 41 ± 15 years, whereas 168 patients with tv-ICD at 49 ± 16 years. For S-ICD patients, mean ESC SCD risk score was 4,5 ± 1.9%: 25 (40%) at low-risk, 17 (27%) at intermediate and 20 (33%) at high-risk. Patients were followed-up for 5.1 ± 2.3 years. Two patients (0.6 per 100-person-years, vs 0.4 per 100 person-years with tv-ICD, p = 0.45) received an appropriate shock on VF, 17 (24%) were diagnosed with de-novo AF. Inappropriate shocks occurred in 4 patients (1.2 per 100-person-years, vs 0.9 per 100 person-years with tv-ICD, p = 0.74), all before Smart-Pass algorithm implementation. Four patients experienced device-related adverse events (1.2 per 100-person-years, vs 1 per 100 person-years with tv-ICD, p = 0.35%). Conclusions: S-ICDs were often implanted in patients with an overall low-intermediate ESC SCD risk, reflecting both the inclusion of additional risk markers and a lower decision threshold. S-ICDs in HCM patients followed for over 5 years showed to be effective in conversion of VF and safe. Greater scrutiny may be required to avoid overtreatment in patients with milder risk profiles.

Published
2024

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
2024

5. Long-term outcomes associated with subcutaneous implantable cardioverter-defibrillators in patients with hypertrophic cardiomyopathy

Author

Rella, V, primary, Maurizi, N, additional, Bernardini, A, additional, Brasca, F M, additional, Salerno, S, additional, Meda, M, additional, Mariani, D, additional, Torchio, M, additional, Conte, G, additional, Auricchio, A, additional, Parati, G, additional, Olivotto, I, additional, Perego, G B, additional, Cecchi, F, additional, and Crotti, L, additional

Published
2024
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6. Intermuscular technique for implantation of the subcutaneous implantable defibrillator: a propensity-matched case-control study

Author

Botto, G, Ziacchi, M, Nigro, G, D'Onofrio, A, Dello Russo, A, Francia, P, Viani, S, Pisano, E, Bisignani, G, Caravati, F, Migliore, F, De Filippo, P, Ottaviano, L, Rordorf, R, Manzo, M, Canevese, F, Lovecchio, M, Valsecchi, S, Checchi, L, Casale, M, Schintu, B, Scalone, A, Tola, G, Setzu, A, Curcio, A, Santoro, A, Baiocchi, C, Gentilini, R, Lunghetti, S, Solimene, F, Shopova, G, Schillaci, V, Arestia, A, Agresta, A, Bianchi, S, Rossi, P, Cauti, F, La Greca, C, Pecora, D, Ammirati, F, Santini, L, Mahfouz, K, Colaiaco, C, Vicentini, A, Savastano, S, Petracci, B, Sanzo, A, Baldi, E, Casula, M, Perego, G, Rella, V, Bianchi, V, Tavoletta, V, De Vivo, S, Palmisano, P, Accogli, M, Milanese, G, Pepi, P, Nicolis, D, Mariani, M, Pagani, M, Bonfantino, M, Caccavo, V, Grimaldi, M, Katsouras, G, Forleo, G, Chieffo, E, Tavarelli, E, Brambilla, R, Pani, A, Giammaria, M, Lucciola, M, Amellone, C, Uran, C, Baroni, M, Ferrari, P, Leidi, C, Drago, F, Silvetti, M, Pazzano, V, Russo, S, Remoli, R, Battipaglia, I, Cazzoli, I, Saputo, F, Devecchi, C, Barbonaglia, L, Viscusi, M, Brignoli, M, Mattera, A, Pedretti, S, Lupi, A, Tommasi, S, Kol, A, Gatto, M, Persi, A, Gonella, A, Rossetti, G, Menardi, E, Rossini, R, Notarstefano, P, Nesti, M, Fraticelli, A, Carreras, G, Donzelli, S, Marini, C, Tordini, A, Lazzari, L, Sartori, P, Di Donna, P, Mascia, G, Capogrosso, P, Magliano, P, Colimodio, M, Sala, S, Mazzone, P, Della Bella, P, Coppolino, A, Arena, G, Borrello, V, Ratti, M, Bartoli, C, Palano, F, Adduci, C, Torriglia, A, Laffi, M, Esposito, C, Giano, A, Franculli, F, Pangallo, A, De Bonis, S, Sarubbi, B, Colonna, D, Correra, A, Romeo, E, Rapacciuolo, A, Liguori, V, Viggiano, A, Strisciullo, T, Biffi, M, Diemberger, I, Martignani, C, Piro, A, Lavalle, C, Magnocavallo, M, Ricciardi, D, Calabrese, V, Gioia, F, Picarelli, F, Licciardello, G, Busacca, G, Calvi, V, Lamberti, F, Lumia, G, Bellini, C, Bianchi, C, Savarese, G, Andreoli, C, Pimpinicchio, L, Pellegrini, D, Giorgi, D, Bovenzi, Busoni, F, Daleffe, E, Facchin, D, Rebellato, L, Stifano, G, Magliano, G, Sergi, D, Barone, L, Morgagni, R, Casella, M, Guerra, F, Cipolletta, L, Molini, S, De Lucia, R, Di Cori, A, Grifoni, G, Paperini, L, Segreti, L, Soldati, E, Zucchelli, G, Russo, V, Rago, A, Ammendola, E, Papa, A, Pieragnoli, P, Ricciardi, G, Perrotta, L, Botto G. L., Ziacchi M., Nigro G., D'Onofrio A., Dello Russo A., Francia P., Viani S., Pisano E., Bisignani G., Caravati F., Migliore F., De Filippo P., Ottaviano L., Rordorf R., Manzo M., Canevese F. L., Lovecchio M., Valsecchi S., Checchi L., Casale M. C., Schintu B., Scalone A., Tola G., Setzu A., Curcio A., Santoro A., Baiocchi C., Gentilini R., Lunghetti S., Solimene F., Shopova G., Schillaci V., Arestia A., Agresta A., Bianchi S., Rossi P., Cauti F. M., La Greca C., Pecora D., Ammirati F., Santini L., Mahfouz K., Colaiaco C., Vicentini A., Savastano S., Petracci B., Sanzo A., Baldi E., Casula M., Perego G. B., Rella V., Bianchi V., Tavoletta V., De Vivo S., Palmisano P., Accogli M., Milanese G., Pepi P., Nicolis D., Mariani M., Pagani M., Bonfantino M. V., Caccavo V., Grimaldi M., Katsouras G., Forleo G. B., Chieffo E., Tavarelli E., Brambilla R., Pani A., Giammaria M., Lucciola M. T., Amellone C., Uran C., Baroni M., Ferrari P., Leidi C., Drago F., Silvetti M. S., Pazzano V., Russo S., Remoli R., Battipaglia I., Cazzoli I., Saputo F., Devecchi C., Barbonaglia L., Viscusi M., Brignoli M., Mattera A., Pedretti S., Lupi A., Tommasi S., Kol A., Gatto M. C., Persi A., Gonella A., Rossetti G., Menardi E., Rossini R., Notarstefano P., Nesti M., Fraticelli A., Carreras G., Donzelli S., Marini C., Tordini A., Lazzari L., Sartori P., Di Donna P., Mascia G., Capogrosso P., Magliano P., Colimodio M., Sala S., Mazzone P., Della Bella P., Coppolino A., Arena G., Borrello V., Ratti M., Bartoli C., Palano F., Adduci C., Torriglia A., Laffi M., Esposito C., Giano A., Franculli F., Pangallo A., De Bonis S., Sarubbi B., Colonna D., Correra A., Romeo E., Rapacciuolo A., Liguori V., Viggiano A., Strisciullo T., Biffi M., Diemberger I., Martignani C., Piro A., Lavalle C., Magnocavallo M., Mariani M. V., Ricciardi D., Calabrese V., Gioia F., Picarelli F., Licciardello G., Busacca G., Calvi V. I., Lamberti F., Lumia G., Bellini C., Bianchi C., Savarese G., Andreoli C., Pimpinicchio L., Pellegrini D., Giorgi D., Busoni F., Daleffe E., Facchin D., Rebellato L., Stifano G., Magliano G., Sergi D., Barone L., Morgagni R., Casella M., Guerra F., Cipolletta L., Molini S., De Lucia R., Di Cori A., Grifoni G., Paperini L., Segreti L., Soldati E., Zucchelli G., Russo V., Rago A., Ammendola E., Papa A., Pieragnoli P., Ricciardi G., Perrotta L., Botto, G, Ziacchi, M, Nigro, G, D'Onofrio, A, Dello Russo, A, Francia, P, Viani, S, Pisano, E, Bisignani, G, Caravati, F, Migliore, F, De Filippo, P, Ottaviano, L, Rordorf, R, Manzo, M, Canevese, F, Lovecchio, M, Valsecchi, S, Checchi, L, Casale, M, Schintu, B, Scalone, A, Tola, G, Setzu, A, Curcio, A, Santoro, A, Baiocchi, C, Gentilini, R, Lunghetti, S, Solimene, F, Shopova, G, Schillaci, V, Arestia, A, Agresta, A, Bianchi, S, Rossi, P, Cauti, F, La Greca, C, Pecora, D, Ammirati, F, Santini, L, Mahfouz, K, Colaiaco, C, Vicentini, A, Savastano, S, Petracci, B, Sanzo, A, Baldi, E, Casula, M, Perego, G, Rella, V, Bianchi, V, Tavoletta, V, De Vivo, S, Palmisano, P, Accogli, M, Milanese, G, Pepi, P, Nicolis, D, Mariani, M, Pagani, M, Bonfantino, M, Caccavo, V, Grimaldi, M, Katsouras, G, Forleo, G, Chieffo, E, Tavarelli, E, Brambilla, R, Pani, A, Giammaria, M, Lucciola, M, Amellone, C, Uran, C, Baroni, M, Ferrari, P, Leidi, C, Drago, F, Silvetti, M, Pazzano, V, Russo, S, Remoli, R, Battipaglia, I, Cazzoli, I, Saputo, F, Devecchi, C, Barbonaglia, L, Viscusi, M, Brignoli, M, Mattera, A, Pedretti, S, Lupi, A, Tommasi, S, Kol, A, Gatto, M, Persi, A, Gonella, A, Rossetti, G, Menardi, E, Rossini, R, Notarstefano, P, Nesti, M, Fraticelli, A, Carreras, G, Donzelli, S, Marini, C, Tordini, A, Lazzari, L, Sartori, P, Di Donna, P, Mascia, G, Capogrosso, P, Magliano, P, Colimodio, M, Sala, S, Mazzone, P, Della Bella, P, Coppolino, A, Arena, G, Borrello, V, Ratti, M, Bartoli, C, Palano, F, Adduci, C, Torriglia, A, Laffi, M, Esposito, C, Giano, A, Franculli, F, Pangallo, A, De Bonis, S, Sarubbi, B, Colonna, D, Correra, A, Romeo, E, Rapacciuolo, A, Liguori, V, Viggiano, A, Strisciullo, T, Biffi, M, Diemberger, I, Martignani, C, Piro, A, Lavalle, C, Magnocavallo, M, Ricciardi, D, Calabrese, V, Gioia, F, Picarelli, F, Licciardello, G, Busacca, G, Calvi, V, Lamberti, F, Lumia, G, Bellini, C, Bianchi, C, Savarese, G, Andreoli, C, Pimpinicchio, L, Pellegrini, D, Giorgi, D, Bovenzi, Busoni, F, Daleffe, E, Facchin, D, Rebellato, L, Stifano, G, Magliano, G, Sergi, D, Barone, L, Morgagni, R, Casella, M, Guerra, F, Cipolletta, L, Molini, S, De Lucia, R, Di Cori, A, Grifoni, G, Paperini, L, Segreti, L, Soldati, E, Zucchelli, G, Russo, V, Rago, A, Ammendola, E, Papa, A, Pieragnoli, P, Ricciardi, G, Perrotta, L, Botto G. L., Ziacchi M., Nigro G., D'Onofrio A., Dello Russo A., Francia P., Viani S., Pisano E., Bisignani G., Caravati F., Migliore F., De Filippo P., Ottaviano L., Rordorf R., Manzo M., Canevese F. L., Lovecchio M., Valsecchi S., Checchi L., Casale M. C., Schintu B., Scalone A., Tola G., Setzu A., Curcio A., Santoro A., Baiocchi C., Gentilini R., Lunghetti S., Solimene F., Shopova G., Schillaci V., Arestia A., Agresta A., Bianchi S., Rossi P., Cauti F. M., La Greca C., Pecora D., Ammirati F., Santini L., Mahfouz K., Colaiaco C., Vicentini A., Savastano S., Petracci B., Sanzo A., Baldi E., Casula M., Perego G. B., Rella V., Bianchi V., Tavoletta V., De Vivo S., Palmisano P., Accogli M., Milanese G., Pepi P., Nicolis D., Mariani M., Pagani M., Bonfantino M. V., Caccavo V., Grimaldi M., Katsouras G., Forleo G. B., Chieffo E., Tavarelli E., Brambilla R., Pani A., Giammaria M., Lucciola M. T., Amellone C., Uran C., Baroni M., Ferrari P., Leidi C., Drago F., Silvetti M. S., Pazzano V., Russo S., Remoli R., Battipaglia I., Cazzoli I., Saputo F., Devecchi C., Barbonaglia L., Viscusi M., Brignoli M., Mattera A., Pedretti S., Lupi A., Tommasi S., Kol A., Gatto M. C., Persi A., Gonella A., Rossetti G., Menardi E., Rossini R., Notarstefano P., Nesti M., Fraticelli A., Carreras G., Donzelli S., Marini C., Tordini A., Lazzari L., Sartori P., Di Donna P., Mascia G., Capogrosso P., Magliano P., Colimodio M., Sala S., Mazzone P., Della Bella P., Coppolino A., Arena G., Borrello V., Ratti M., Bartoli C., Palano F., Adduci C., Torriglia A., Laffi M., Esposito C., Giano A., Franculli F., Pangallo A., De Bonis S., Sarubbi B., Colonna D., Correra A., Romeo E., Rapacciuolo A., Liguori V., Viggiano A., Strisciullo T., Biffi M., Diemberger I., Martignani C., Piro A., Lavalle C., Magnocavallo M., Mariani M. V., Ricciardi D., Calabrese V., Gioia F., Picarelli F., Licciardello G., Busacca G., Calvi V. I., Lamberti F., Lumia G., Bellini C., Bianchi C., Savarese G., Andreoli C., Pimpinicchio L., Pellegrini D., Giorgi D., Busoni F., Daleffe E., Facchin D., Rebellato L., Stifano G., Magliano G., Sergi D., Barone L., Morgagni R., Casella M., Guerra F., Cipolletta L., Molini S., De Lucia R., Di Cori A., Grifoni G., Paperini L., Segreti L., Soldati E., Zucchelli G., Russo V., Rago A., Ammendola E., Papa A., Pieragnoli P., Ricciardi G., and Perrotta L.

Abstract

Aims A previous randomized study demonstrated that the subcutaneous implantable cardioverter defibrillator (S-ICD) was noninferior to transvenous ICD with respect to device-related complications and inappropriate shocks. However, that was performed prior to the widespread adoption of pulse generator implantation in the intermuscular (IM) space instead of the traditional subcutaneous (SC) pocket. The aim of this analysis was to compare survival from device-related complications and inappropriate shocks between patients who underwent S-ICD implantation with the generator positioned in an IM position in comparison with an SC pocket. Methods and results We analysed 1577 consecutive patients who had undergone S-ICD implantation from 2013 to 2021 and were followed up until December 2021. Subcutaneous patients (n = 290) were propensity matched with patients of the IM group (n = 290), and their outcomes were compared. : During a median follow-up of 28 months, device-related complications were reported in 28 (4.8%) patients and inappropriate shocks were reported in 37 (6.4%) patients. The risk of complication was lower in the matched IM group than in the SC group [hazard ratio 0.41, 95% confidence interval (CI) 0.17-0.99, P = 0.041], as well as the composite of complications and inappropriate shocks (hazard ratio 0.50, 95% CI 0.30-0.86, P = 0.013). The risk of appropriate shocks was similar between groups (hazard ratio 0.90, 95% CI 0.50-1.61, P = 0.721). There was no significant interaction between generator positioning and variables such as gender, age, body mass index, and ejection fraction. Conclusion Our data showed the superiority of the IM S-ICD generator positioning in reducing device-related complications and inappropriate shocks.

Published
2023

7. 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

8. 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

9. 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

10. AB1127 THE RETENTION RATE OF ETANERCEPT AND ADALIMUMAB IN FIRST-LINE AND AFTER NON-MEDICAL SWITCH IN PATIENTS WITH PSORIATIC ARTHRITIS: A COMPARISON BETWEEN ORIGINATORS AND BIOSIMILARS. A SINGLE-CENTER RETROSPECTIVE STUDY.

Author

Rella, V., primary, Rotondo, C., additional, Maruotti, N., additional, Altomare, A., additional, Erroi, F., additional, Stefania, S., additional, Trotta, A., additional, Cantatore, F. P., additional, and Corrado, A., additional

Published
2023
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12. Incremental Value of Right Atrial Strain Analysis to Predict Atrial Fibrillation Recurrence After Electrical Cardioversion

Author

Tomaselli, M, Badano, L, Cannone, V, Radu, N, Curti, E, Perelli, F, Heilbron, F, Gavazzoni, M, Rella, V, Oliverio, G, Caravita, S, Baratto, C, Perego, G, Parati, G, Brasca, F, Muraru, D, Tomaselli, Michele, Badano, Luigi, Cannone, Vincenzo, Radu, Noela, Curti, Emanuele, Perelli, Francesco, Heilbron, Francesca, Gavazzoni, Mara, Rella, Valeria, Oliverio, Giorgio, Caravita, Sergio, Baratto, Claudia, Perego, Giovanni B, Parati, Gianfranco, Brasca, Francesco, Muraru, Denisa, Tomaselli, M, Badano, L, Cannone, V, Radu, N, Curti, E, Perelli, F, Heilbron, F, Gavazzoni, M, Rella, V, Oliverio, G, Caravita, S, Baratto, C, Perego, G, Parati, G, Brasca, F, Muraru, D, Tomaselli, Michele, Badano, Luigi, Cannone, Vincenzo, Radu, Noela, Curti, Emanuele, Perelli, Francesco, Heilbron, Francesca, Gavazzoni, Mara, Rella, Valeria, Oliverio, Giorgio, Caravita, Sergio, Baratto, Claudia, Perego, Giovanni B, Parati, Gianfranco, Brasca, Francesco, and Muraru, Denisa

Abstract

Background: Although the assessment of left atrial (LA) mechanics has been reported to refine atrial fibrillation (AF) risk prediction, it doesn't completely predict AF recurrences. The potential added role of right atrial (RA) function in this setting is unknown. Accordingly, this study sought to evaluate the added value of RA longitudinal reservoir strain (RASr) for the prediction of AF recurrence after electrical cardioversion (ECV). Methods: We retrospectively studied 132 consecutive patients with persistent AF who underwent elective ECV. Complete two-dimensional and speckle-tracking echocardiography analyses of LA and RA size and function were obtained in all patients before ECV. The endpoint was AF recurrence. Results: During a 12-month follow-up of, 63 patients (48%) showed AF recurrence. Both LA and RASr were significantly lower in patients experiencing AF recurrence than in patients with persistent sinus rhythm (LASr 10±6 vs 13±7%, and RASr 14±10 vs 20±9 %, respectively, p<0.001 for both). RASr (AUC=0.77, 95%IC 0.69-0.84, p<0.0001) was more strongly associated with the recurrence of AF after ECV than LASr (AUC=0.69 (95%IC 0.60-0.77), p<0.0001]. Kaplan-Meier curves showed that patients with both LASr≤10% and RASr ≤15% had a significantly increased risk for AF recurrences (log-rank, p<0.001). However, at multivariable Cox regression, RASr [HR 3.26, 95%CI (1.73-6.13), p< 0.001] was the only parameter independently associated with the AF recurrence. RASr was more strongly associated with the occurrence of AF relapse after ECV than LASr, LA and RA volumes. Conclusions: RASr was independently and more strongly associated than LASr with AF recurrence after elective ECV. This study highlights the importance of assessing the functional remodeling of both RA and LA in patients with persistent AF.

Published
2023

13. AMILOIDOSI CARDIACA ANALISI DI PREVALENZA IN DUE STUDI MULTICENTRICI ITALIANI

Author

CROTTI, LIA, Rella, V, GRASSI, GUIDO, PARATI, GIANFRANCO, RELLA, VALERIA, CROTTI, LIA, Rella, V, GRASSI, GUIDO, PARATI, GIANFRANCO, and RELLA, VALERIA

Abstract

Tra i pazienti con diagnosi iniziale di cardiomiopatia ipertrofica afferiti a Centri di Riferimento per le Cardiomiopatie, l’AC è la malattia non riconosciuta più comune con una prevalenza complessiva del 9%, e che aumenta con l'età (dall'1% nella fascia di età tra i 40-49 anni al 26% sopra gli 80 anni). Nella popolazione generale ≥55 anni più del 7% ha almeno un reperto ecocardiografico suggestivo di AC e l’ispessimento del setto interatriale è quello più frequente. I pazienti con elevato sospetto di AC (≥3 reperti) rappresentano l’1% della popolazione generale e il 4,9% di quelli con cuore non dilatato, ipertrofico e con FE normale., Among patients with initial diagnosis of HCM, cardiac amiloidosis has a prevalence of 9% and it increases with age. In the general population > 55 yo more than 7% has echocardiographic suspicion of the disease and echocardiography has an important role in the early diagnosis of the disease

Published
2022

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

17. 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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19. Impact of leaflet-tethering angle correction on the assessment of tricuspid regurgitation severity using the PISA method

Author

Tomaselli, M, primary, Gavazzoni, M, additional, Muraru, D, additional, Caravita, S, additional, Volpato, V, additional, Oliverio, G, additional, Ciambellotti, F, additional, Mene", R, additional, Florescu, DR, additional, Ciampi, P, additional, Heilbron, F, additional, Rella, V, additional, Parati, G, additional, and Badano, LP, additional

Published
2022
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21. INCREMENTAL VALUE OF RIGHT ATRIAL STRAIN ANALYSIS TO PREDICT ATRIAL FIBRILLATION RECURRENCE AFTER ELECTRICAL CARDIOVERSION

Author

Tomaselli, Michele, Badano, Luigi, Cannone, Vincenzo, Radu, Noela, Curti, Emanuele, Perelli, Francesco, Heilbron, Francesca, Gavazzoni, Mara, Rella, Valeria, Oliverio, Giorgio, Caravita, Sergio, Baratto, Claudia, Perego, Giovanni B, Parati, Gianfranco, Brasca, Francesco, Muraru, Denisa, Tomaselli, M, Badano, L, Cannone, V, Radu, N, Curti, E, Perelli, F, Heilbron, F, Gavazzoni, M, Rella, V, Oliverio, G, Caravita, S, Baratto, C, Perego, G, Parati, G, Brasca, F, and Muraru, D

Subjects
atrial function, echocardiography, atrial fibrillation, right atrial strain, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, right atrium, speckle-tracking echocardiography
Abstract

Background: Although the assessment of left atrial (LA) mechanics has been reported to refine atrial fibrillation (AF) risk prediction, it doesn't completely predict AF recurrences. The potential added role of right atrial (RA) function in this setting is unknown. Accordingly, this study sought to evaluate the added value of RA longitudinal reservoir strain (RASr) for the prediction of AF recurrence after electrical cardioversion (ECV). Methods: We retrospectively studied 132 consecutive patients with persistent AF who underwent elective ECV. Complete two-dimensional and speckle-tracking echocardiography analyses of LA and RA size and function were obtained in all patients before ECV. The endpoint was AF recurrence. Results: During a 12-month follow-up of, 63 patients (48%) showed AF recurrence. Both LA and RASr were significantly lower in patients experiencing AF recurrence than in patients with persistent sinus rhythm (LASr 10±6 vs 13±7%, and RASr 14±10 vs 20±9 %, respectively, p

Published
2023

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

Author

Marco Merlo, Linda Pagura, Aldostefano Porcari, Matteo Cameli, Giuseppe Vergaro, Beatrice Musumeci, Elena Biagini, Marco Canepa, Lia Crotti, Massimo Imazio, Cinzia Forleo, Francesco Cappelli, Federico Perfetto, Stefano Favale, Gianluca Di Bella, Franca Dore, Francesca Girardi, Daniela Tomasoni, Rita Pavasini, Valeria Rella, Giuseppe Palmiero, Martina Caiazza, Maria Cristina Carella, Andrea Igoren Guaricci, Giovanna Branzi, Angelo Giuseppe Caponetti, Giulia Saturi, Giovanni La Malfa, Andrea Carlo Merlo, Alessandro Andreis, Francesco Bruno, Francesca Longo, Maddalena Rossi, Guerino Giuseppe Varrà, Riccardo Saro, Luca Di Ienno, Giuseppe De Carli, Elisa Giacomin, Chiara Arzilli, Giuseppe Limongelli, Camillo Autore, Iacopo Olivotto, Luigi Badano, Gianfranco Parati, Stefano Perlini, Marco Metra, Michele Emdin, Claudio Rapezzi, Gianfranco Sinagra, Merlo, Marco, Pagura, Linda, Porcari, Aldostefano, Cameli, Matteo, Vergaro, Giuseppe, Musumeci, Beatrice, Biagini, Elena, Canepa, Marco, Crotti, Lia, Imazio, Massimo, Forleo, Cinzia, Cappelli, Francesco, Perfetto, Federico, Favale, Stefano, Di Bella, Gianluca, Dore, Franca, Girardi, Francesca, Tomasoni, Daniela, Pavasini, Rita, Rella, Valeria, Palmiero, Giuseppe, Caiazza, Martina, Carella, Maria Cristina, Igoren Guaricci, Andrea, Branzi, Giovanna, Caponetti, Angelo Giuseppe, Saturi, Giulia, La Malfa, Giovanni, Merlo, Andrea Carlo, Andreis, Alessandro, Bruno, Francesco, Longo, Francesca, Rossi, Maddalena, Varrà, Guerino Giuseppe, Saro, Riccardo, Di Ienno, Luca, De Carli, Giuseppe, Giacomin, Elisa, Arzilli, Chiara, Limongelli, Giuseppe, Autore, Camillo, Olivotto, Iacopo, Badano, Luigi, Parati, Gianfranco, Perlini, Stefano, Metra, Marco, Emdin, Michele, Rapezzi, Claudio, Sinagra, Gianfranco, 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, and Michele, Emdin

Subjects
Amyloid cardiomyopathy, Echocardiography, Epidemiology, Light chain amyloidosis, Red flags, Transthyretin amyloidosis, Heart Failure, Left, Stroke Volume, Amyloidosis, Middle Aged, Ventricular Function, Left, Humans, Prevalence, Prospective Studies, Cardiomyopathies, Ventricular Function, Light chain amyloidosi, Red flag, Cardiology and Cardiovascular Medicine
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

23. AMILOIDOSI CARDIACA ANALISI DI PREVALENZA IN DUE STUDI MULTICENTRICI ITALIANI

Author

RELLA, VALERIA, Rella, V, GRASSI, GUIDO, and PARATI, GIANFRANCO

Subjects
ecocardiografia, cardiac amyloidosi, prevalenza, amiloidosi cardiaca, prevalence, terapia specifica, echocardiography, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, HCM
Abstract

Tra i pazienti con diagnosi iniziale di cardiomiopatia ipertrofica afferiti a Centri di Riferimento per le Cardiomiopatie, l’AC è la malattia non riconosciuta più comune con una prevalenza complessiva del 9%, e che aumenta con l'età (dall'1% nella fascia di età tra i 40-49 anni al 26% sopra gli 80 anni). Nella popolazione generale ≥55 anni più del 7% ha almeno un reperto ecocardiografico suggestivo di AC e l’ispessimento del setto interatriale è quello più frequente. I pazienti con elevato sospetto di AC (≥3 reperti) rappresentano l’1% della popolazione generale e il 4,9% di quelli con cuore non dilatato, ipertrofico e con FE normale. Among patients with initial diagnosis of HCM, cardiac amiloidosis has a prevalence of 9% and it increases with age. In the general population > 55 yo more than 7% has echocardiographic suspicion of the disease and echocardiography has an important role in the early diagnosis of the disease

Published
2022

24. Glucocorticoids treatment and adverse infectious events in rheumatic diseases.

Author

Rella V, Rotondo C, Barile R, Erroi F, Cantatore FP, and Corrado A

Abstract

It is well known that rheumatic diseases are characterized by an increased infection risk, due to several factors, such as an intrinsically dysfunctional immune system, disease activity, and the use of immunosuppressive drugs. Glucocorticoids are widely used therapeutic agents for treating several chronic inflammatory and immune diseases, due to their anti-inflammatory and immunosuppressive effects. Their use is burdened by well-known side effects in dose- and duration of use-dependent manner. Physicians need to be aware of the mechanism of action of glucocorticoids, their side effects, particularly infectious side effects, and the significance of cumulative dose and duration of glucocorticoid treatment. Additionally, physicians shoultdleveld have knowledge of each patient and their comorbidities. They could use appropriate tools for assessing glucocorticoid-related toxicity and morbidity, particularly in the context of chronic glucocorticoid administration. This comprehensive understanding is crucial for ensuring the proper and safe use of these drugs, particularly in terms of minimizing infectious risks. The aim of this review is to focus on available data concerning the infectious risk associated to glucocorticoid treatment in rheumatic diseases, highlighting the role of the correct drug management in clinical practice and the role of the disease itself in the occurrence of this worthy side effect. We conducted a review of randomized controlled trials and observational studies about glucocorticoid use in autoimmune/rheumatic diseases, analyzing the infectious risk during glucocorticoid therapy, and its relationship with the used dose and duration of treatment.

Published
2024
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25. Early diagnosis, disease stage and prognosis in wild-type transthyretin amyloid cardiomyopathy: The DIAMOND study.

Author

Tini G, Musumeci B, Milani P, Zampieri M, Caponetti AG, Fabris F, Foli A, Argirò A, Mazzoni C, Gagliardi C, Longhi S, Saturi G, Vergaro G, Aimo A, De Fazio L, Varrà GG, Serenelli M, Fabbri G, De Michieli L, Palmiero G, Ciliberti G, Carigi S, Zanoletti M, Mandoli GE, Lucchi GR, Rella V, Monti E, Gardini E, Bartolotti M, Crotti L, Merli E, Mussinelli R, Vianello PF, Cameli M, Marzo F, Guerra F, Limongelli G, Cipriani A, Perlini S, Obici L, Perfetto F, Barbato E, Porto I, Sinagra G, Merlo M, Emdin M, Biagini E, Cappelli F, Palladini G, and Canepa M

Abstract

Aims: Disease staging and prognostic scoring in wild-type transthyretin-related cardiac amyloidosis (ATTRwt-CA) can be captured by two systems (NAC and Columbia scores). However, uncertainty remains as epidemiology of the disease is evolving rapidly. We evaluated features associated with staging systems across ATTRwt-CA patients from different diagnostic pathways, and their association with prognosis., Methods: We performed an analysis on DIAMOND patients with available data to evaluate NAC and Columbia score. DIAMOND was a retrospective study from 17 Italian referral centres for CA, enrolling 1281 patients diagnosed between 2016 and 2021, and aimed at describing characteristics of pathways leading to ATTRwt-CA diagnosis. Of the original cohort, 811 patients were included in this analysis. Each patient had NAC and Columbia score calculated. Patients were grouped according to NAC and Columbia scoring classes. We described characteristics of patients according to staging classes and diagnostic pathways at diagnosis. Prevalence of early diagnoses, defined as NAC Ia, NYHA class I, no use of diuretics, no history of heart failure (HF) hospitalizations nor of atrial fibrillation prior to diagnosis, was investigated. Finally, prognostic variables were tested alone and grouped as NAC or Columbia scores in Cox univariate and multivariate regression analyses. Prognosis was investigated as all-cause mortality, in the whole population and dividing patients in HF versus other diagnostic pathways., Results: Only 1% of the study population had an early ATTRwt-CA diagnosis. Distribution of prognostic variables and of NAC and Columbia classes was heterogeneous across diagnostic pathways. The prevalence of NAC III and Columbia III was higher in the HF diagnostic pathway, but all NAC and Columbia classes were present in all pathways. Both NAC and Columbia scores were associated with all-cause mortality at univariate Cox regression analysis in the whole population, in patients from the HF diagnostic pathway and in those from other pathways. At multivariate analysis, Columbia score remained significantly associated with the outcome, together with age at diagnosis, left ventricular ejection fraction and maximal wall thickness., Conclusions: In this contemporary nationwide cohort, an ATTRwt-CA early diagnosis was very rare. Disease staging with NAC and Columbia scoring systems determined classes of patients with heterogeneous features. Both scores were significantly associated with mortality, but other variables also had prognostic significance., (© 2024 The Author(s). ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Published
2024
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27. Enhancing Buoyant force learning through a visuo-haptic environment: a case study.

Author

Neri L, Noguez J, Escobar-Castillejos D, Robledo-Rella V, García-Castelán RMG, Gonzalez-Nucamendi A, Magana AJ, and Benes B

Abstract

Introduction: This study aimed to develop, implement, and test a visuo-haptic simulator designed to explore the buoyancy phenomenon for freshman engineering students enrolled in physics courses. The primary goal was to enhance students' understanding of physical concepts through an immersive learning tool. Methods: The visuo-haptic simulator was created using the VIS-HAPT methodology, which provides high-quality visualization and reduces development time. A total of 182 undergraduate students were randomly assigned to either an experimental group that used the simulator or a control group that received an equivalent learning experience in terms of duration and content. Data were collected through pre- and post-tests and an exit-perception questionnaire. Results: Data analysis revealed that the experimental group achieved higher learning gains than the control group ( p = 0.079). Additionally, students in the experimental group expressed strong enthusiasm for the simulator, noting its positive impact on their understanding of physical concepts. The VIS-HAPT methodology also reduced the average development time compared to similar visuo-haptic simulators. Discussion: The results demonstrate the efficacy of the buoyancy visuo-haptic simulator in improving students' learning experiences and validate the utility of the VIS-HAPT method for creating immersive educational tools in physics., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Neri, Noguez, Escobar-Castillejos, Robledo-Rella, García-Castelán, Gonzalez-Nucamendi, Magana and Benes.)

Published
2024
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28. Combined peripheral and central ultrasound for the diagnosis of PAH-SSc patients.

Author

Correale M, Rotondo C, Bevere EML, Tricarico L, Rella V, Villani D, Granato M, Migliozzi C, Cantatore FP, Brunetti ND, and Corrado A

Subjects
Humans, Female, Male, Middle Aged, Hypertension, Pulmonary physiopathology, Hypertension, Pulmonary complications, Hypertension, Pulmonary diagnostic imaging, Adult, Scleroderma, Systemic complications, Scleroderma, Systemic physiopathology, Echocardiography methods
Abstract

Background: Systemic Sclerosis (SSc), an intricate autoimmune disease causing tissue fibrosis, introduces cardiovascular complexities, notably pulmonary hypertension (PH), affecting both survival and quality of life. This study centers on evaluating echocardiographic parameters and endothelial function using flow-mediated dilatation (FMD) in SSc patients, aiming to differentiate those with and without pulmonary arterial hypertension (PAH). The emphasis lies in early detection, given the heightened vulnerability of the right ventricle (RV) in the presence of PH., Methods: Fifty-nine SSc patients and 48 healthy subjects participated, undergoing clinical examinations, echocardiography, FMD assessments, blood analyses, and right heart catheterization (RHC) according to the ESC/ERS guidelines for diagnosis and treatment of PH., Results: SSc-PAH patients displayed lower FMD, higher frequency of TAPSE < 18 mm, RA area > 18 cm 2 , act RVOT < 105 ms and TRV > 280 cm/s compared to those without PAH and healthy controls. Resting resistivity index (RI) was higher in SSc patients, with no significant difference between those with and without PAH. Lower FMD% serves as a predictive marker for adverse cardiovascular outcomes in both SSc and SSc-PAH patients. Stratification by TRV levels and PAH presence reveals notable FMD% variations, emphasizing its potential utility., Conclusions: Early identification of endothelial dysfunction and impaired RV echocardiographic parameters, such as TAPSE and TRV, could aid in predicting right ventricular dysfunction and PAH in SSc patients., (© 2024 Wiley Periodicals LLC.)

Published
2024
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29. Flow Mediated Dilation in Systemic Sclerosis: Association with clinical findings, capillaroscopic patterns and endothelial circulating markers.

Author

Corrado A, Mansueto N, Correale M, Rella V, Tricarico L, Altomare A, Brunetti ND, Cantatore FP, and Rotondo C

Subjects
Humans, Microscopic Angioscopy, Vascular Endothelial Growth Factor A, Angiopoietin-2, Dilatation, Vascular Cell Adhesion Molecule-1, Pulmonary Arterial Hypertension, Scleroderma, Systemic diagnosis, Scleroderma, Systemic complications, Scleroderma, Systemic pathology, Skin Ulcer
Abstract

Aim: Endothelial dysfunction represents a key feature of the pathological process underlying micro and macro-vascular damage in Systemic Sclerosis (SSc). This study aims to improve knowledge of the physiopathology of vascular damage in SSc through the assessment of the endothelial dysfunction by Flow Mediated Dilation (FMD) and serum levels of circulating endothelial dysfunction markers and the correlation of macrovascular damage with clinical findings and microvascular capillaroscopic patterns., Methods: 57 SSc patients and 37 healthy subjects were recruited. All included subjects underwent radial artery FMD test and Nailfold Video-Capillaroscopy; serum levels of Vascular Endothelial Growth Factor (VEGF), Vascular Cell Adhesion Molecule-1 (VCAM-1) and angiopoietin-2 were evaluated., Results: Compared to healthy subjects, in SSc patients lower FMD and higher time needed to obtain the maximal FMD responsewere observed, whereas serum levels of VEGF, VCAM-1, and angiopoietin-2 were significantly higher. The impairment of FMD values was associated with disease duration, pulmonary arterial hypertension, and digital ulcers and correlates with greater microvascular damage evaluated by Nailfold Video-Capillaroscopy… An inverse relationship between VEGF, angiopoietin-2, VCAM-1 levels and FMD was observed, but only VEGF and angiopoietin-2 were significantly higher in patients with digital ulcers and pulmonary arterial hypertension., Conclusions: FMD ultrasound test and circulating levels of endothelial dysfuncion markers could be useful as biomarkers of vasculopathy and could be a helpful tool in the overall assessment of vascular injury in Systemic Sclerosis patients., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023. Published by Elsevier Inc.)

Published
2024
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30. Incremental Value of Right Atrial Strain Analysis to Predict Atrial Fibrillation Recurrence After Electrical Cardioversion.

Author

Tomaselli M, Badano LP, Cannone V, Radu N, Curti E, Perelli F, Heilbron F, Gavazzoni M, Rella V, Oliverio G, Caravita S, Baratto C, Perego GB, Parati G, Brasca F, and Muraru D

Subjects
Humans, Electric Countershock methods, Retrospective Studies, Heart Atria diagnostic imaging, Echocardiography methods, Recurrence, Atrial Fibrillation diagnostic imaging, Atrial Fibrillation therapy
Abstract

Background: Although the assessment of left atrial (LA) mechanics has been reported to refine atrial fibrillation (AF) risk prediction, it doesn't completely predict AF recurrence. The potential added role of right atrial (RA) function in this setting is unknown. Accordingly, this study sought to evaluate the added value of RA longitudinal reservoir strain (RASr) for the prediction of AF recurrence after electrical cardioversion (ECV)., Methods: We retrospectively studied 132 consecutive patients with persistent AF who underwent elective ECV. Complete two-dimensional and speckle-tracking echocardiography analyses of LA and RA size and function were obtained in all patients before ECV. The end point was AF recurrence., Results: During a 12-month follow-up, 63 patients (48%) showed AF recurrence. Both LASr and RASr were significantly lower in patients experiencing AF recurrence than in patients with persistent sinus rhythm (LASr, 10% ± 6% vs 13% ± 7%; RASr, 14% ± 10% vs 20% ± 9%, respectively; P < .001 for both). Right atrial longitudinal reservoir strain (area under the curve = 0.77; 95% CI, 0.69-0.84; P < .0001) was more strongly associated with the recurrence of AF after ECV than LASr (area under the curve = 0.69; 95% CI, 0.60-0.77; P < .0001). Kaplan-Meier curves showed that patients with both LASr ≤ 10% and RASr ≤ 15% had a significantly increased risk for AF recurrence (log-rank, P < .001). However, at multivariable Cox regression, RASr (hazard ratio, 3.26; 95% CI, 1.73-6.13; P < .001) was the only parameter independently associated with AF recurrence. Right atrial longitudinal reservoir strain was more strongly associated with the occurrence of AF relapse after ECV than LASr, and LA and RA volumes., Conclusion: Right atrial longitudinal reservoir strain was independently and more strongly associated than LASr with AF recurrence after elective ECV. This study highlights the importance of assessing the functional remodeling of both the RA and LA in patients with persistent AF., (Copyright © 2023 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Published
2023
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31. Clinical course of hypertrophic cardiomyopathy patients implanted with a transvenous or subcutaneous defibrillator.

Author

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

Subjects
Humans, Bradycardia, Disease Progression, Adenosine Triphosphate, Cardiomyopathy, Hypertrophic complications, Cardiomyopathy, Hypertrophic therapy, Defibrillators, Implantable
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., Competing Interests: Conflict of interest: P.F. received speaker fees from Boston Scientific (BS) and research grants from Abbott and BS. M.Z. received speaker fees from Abbott, Biotronik, and BS. P.D.F. received speaker fees from Abbott, BS, and Medtronic. M.L. is an employee of BS., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Published
2023
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32. Diagnostic pathways to wild-type transthyretin amyloid cardiomyopathy: a multicentre network study.

Author

Tini G, Milani P, Zampieri M, Caponetti AG, Fabris F, Foli A, Argirò A, Mazzoni C, Gagliardi C, Longhi S, Saturi G, Vergaro G, Aimo A, Russo D, Varrà GG, Serenelli M, Fabbri G, De Michieli L, Palmiero G, Ciliberti G, Carigi S, Sessarego E, Mandoli GE, Ricci Lucchi G, Rella V, Monti E, Gardini E, Bartolotti M, Crotti L, Merli E, Mussinelli R, Vianello PF, 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

Subjects
Humans, Prealbumin genetics, Prealbumin metabolism, Retrospective Studies, Amyloid Neuropathies, Familial diagnosis, Amyloid Neuropathies, Familial epidemiology, Amyloid Neuropathies, Familial complications, Heart Failure diagnosis, Heart Failure epidemiology, Heart Failure complications, Cardiomyopathies diagnosis, Cardiomyopathies epidemiology, Cardiomyopathies complications
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., (© 2023 The Authors. European Journal of Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Published
2023
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33. Vitamin D Status and Psoriatic Arthritis: Association with the Risk for Sacroiliitis and Influence on the Retention Rate of Methotrexate Monotherapy and First Biological Drug Survival-A Retrospective Study.

Author

Rotondo C, Cantatore FP, Cici D, Erroi F, Sciacca S, Rella V, and Corrado A

Subjects
Humans, Adolescent, Vitamin D therapeutic use, Retrospective Studies, Methotrexate therapeutic use, Prospective Studies, Vitamins therapeutic use, Arthritis, Psoriatic, Sacroiliitis drug therapy, Sacroiliitis complications, Vitamin D Deficiency complications, Antirheumatic Agents therapeutic use
Abstract

A growing body of evidence on the importance of vitamin D in immune modulation has increased the interest in its possible impact on the course of rheumatological diseases. The scope of our study is to assess if the presence of different statuses of vitamin D could interfere in the clinical subsets, in methotrexate monotherapy discontinuation, and biological drug (b-DMARDs) survival in psoriatic arthritis patients (PsA). We conducted a retrospective study on PsA patients and split them into three groups based on their vitamin D status: the group with 25(OH)D ≤ 20 ng/mL, the group with levels of 25(OH)D between 20 and 30 ng/mL, and the group with serum levels of 25(OH)D ≥ 30 ng/mL. All patients were required to fulfill the CASPAR criteria for psoriatic arthritis and to have the evaluation of vitamin D serum levels at baseline visit and at clinical follow-up visits. The exclusion criteria were ages less than 18 years old, the presence of HLA B27, and satisfaction of rheumatoid arthritis classification criteria (during the study time). Statistical significance was set at p ≤ 0.05. Furthermore, 570 patients with PsA were screened and 233 were recruited. A level of 25(OH)D ≤ 20 ng/mL was present in 39% of patients; levels of 25(OH)D between 20 and 30 ng/mL presented in 25% of patients; 65% of patients with sacroiliitis presented 25 (OH)D ≤ 20 ng/mL. Methotrexate monotherapy discontinuation for failure was higher in the group with 25 (OH)D ≤ 20 ng/mL (survival time: 92 ± 10.3 weeks vs. 141.9 ± 24.1 weeks vs. 160.1 ± 23.6 weeks; p = 0.02) with higher discontinuation risk (HR = 2.168, 95% CI 1.334, 3.522; p = 0.002) than those with 25(OH)D between 20 and 30 ng/mL and those with 25(OH)D ≥ 30 ng/mL. Significantly shorter survival of first b-DMARDs was assessed in the group with 25 (OH)D ≤ 20 ng/mL versus the other groups (133.6 ± 11 weeks vs. 204.8 ± 35.8 weeks vs. 298.9 ± 35.4; p = 0.028) (discontinuation risk 2.129, 95% CI 1.186, 3.821; p = 0.011). This study highlights significant differences in clinical presentation, in particular sacroiliac involvement and on drug survival (methotrexate and b-DMARDs) in PsA patients with vitamin D deficiency. Further prospective studies, including a larger sample of patients, are needed to validate these data and to assess if the supplementation of vitamin D could improve the b-DMARDs response in PsA patients.

Published
2023
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34. 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 MC, Igoren Guaricci A, Branzi G, Caponetti AG, Saturi G, La Malfa G, Merlo AC, Andreis A, Bruno F, Longo F, Rossi M, Varrà GG, 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

Subjects
Humans, Middle Aged, Prevalence, Prospective Studies, Stroke Volume, Ventricular Function, Left, Amyloidosis diagnosis, Cardiomyopathies diagnostic imaging, Cardiomyopathies epidemiology, Heart Failure
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., (© 2022 European Society of Cardiology.)

Published
2022
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35. Bone Involvement in Systemic Lupus Erythematosus.

Author

Rella V, Rotondo C, Altomare A, Cantatore FP, and Corrado A

Subjects
Humans, Quality of Life, Autoimmune Diseases diagnosis, Lupus Erythematosus, Systemic genetics, Osteoporosis etiology
Abstract

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by a wide variability of clinical manifestations due to the potential involvement of several tissues and internal organs, with a relapsing and remitting course. Dysregulation of innate and adaptive immune systems, due to genetic, hormonal and environmental factors, may be responsible for a broad spectrum of clinical manifestations, affecting quality of life, morbidity and mortality. Bone involvement represents one of the most common cause of morbidity and disability in SLE. Particularly, an increased incidence of osteoporosis, avascular necrosis of bone and osteomyelitis has been observed in SLE patients compared to the general population. Moreover, due to the improvement in diagnosis and therapy, the survival of SLE patient has improved, increasing long-term morbidities, including osteoporosis and related fractures. This review aims to highlight bone manifestations in SLE patients, deepening underlying etiopathogenetic mechanisms, diagnostic tools and available treatment.

Published
2022
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36. 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 CM, Pavasini R, Rella V, Palmiero G, Caiazza M, Albanese M, Guaricci AI, Branzi G, Caponetti AG, Saturi G, La Malfa G, Merlo AC, 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, and Sinagra G

Subjects
Humans, Echocardiography, Prevalence, Amyloidosis diagnostic imaging, Amyloidosis epidemiology, Cardiomyopathies diagnostic imaging, Cardiomyopathies epidemiology
Published
2022
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