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1. 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

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. Razionale e valore fondamentale della Rete Italiana integrata dell’Amiloidosi Cardiaca [Rationale and significance of the Italian Network for Cardiac Amyloidosis]

Author: Sinagra, G, Emdin, M, Merlo, M, Vergaro, G, Aimo, A, Biagini, E, Imazio, M, Porcari, A, Limongelli, G, Cipriani, A, Canepa, M, Musumeci, B, Cameli, M, Crotti, L, Di Bella, G, Di Lenarda, A, Cappelli, F, Chimenti, C, Obici, L, Iacoviello, M, Perlini, S, Pieroni, M, Metra, M, Oliva, F, Perrone Filardi, P, Colivicchi, F, Indolfi, C, Sinagra G., Emdin M., Merlo M., Vergaro G., Aimo A., Biagini E., Imazio M., Porcari A., Limongelli G., Cipriani A., Canepa M., Musumeci B., Cameli M., Crotti L., Di Bella G., Di Lenarda A., Cappelli F., Chimenti C., Obici L., Iacoviello M., Perlini S., Pieroni M., Metra M., Oliva F., Perrone Filardi P., Colivicchi F., Indolfi C., Sinagra, G, Emdin, M, Merlo, M, Vergaro, G, Aimo, A, Biagini, E, Imazio, M, Porcari, A, Limongelli, G, Cipriani, A, Canepa, M, Musumeci, B, Cameli, M, Crotti, L, Di Bella, G, Di Lenarda, A, Cappelli, F, Chimenti, C, Obici, L, Iacoviello, M, Perlini, S, Pieroni, M, Metra, M, Oliva, F, Perrone Filardi, P, Colivicchi, F, Indolfi, C, Sinagra G., Emdin M., Merlo M., Vergaro G., Aimo A., Biagini E., Imazio M., Porcari A., Limongelli G., Cipriani A., Canepa M., Musumeci B., Cameli M., Crotti L., Di Bella G., Di Lenarda A., Cappelli F., Chimenti C., Obici L., Iacoviello M., Perlini S., Pieroni M., Metra M., Oliva F., Perrone Filardi P., Colivicchi F., and Indolfi C.
Abstract: The perspective on amyloidosis has changed deeply over the last 10 years following major advances in diagnosis and treatment options, especially in cardiac amyloidosis. This intrinsically heterogeneous disease exposes to the risk of fragmentation of knowledge and requires the interaction among experts of different specialties and subspecialties. Suspicion of disease, timely recognition and confirmation of final diagnosis, prognostic stratification, clinical management and therapeutic strategies represent essential steps to be taken. Missing or delaying the diagnosis may have dramatic impact on patient outcome, as in the case of chemotherapy in unrecognized light-chain amyloidosis. Therefore, there is an urgent need for the foundation of an Italian Amyloidosis Network to deal with the challenges of this condition and orient clinical management at national and local levels. The present consensus document aims to provide the rationale and scopes of the Italian Amyloidosis Network, which has been conceived as an organizational framework for professionals managing patients with amyloidosis.
Published: 2023

5. Septal Ablation Versus Surgical Myomectomy for Hypertrophic Obstructive Cardiomyopathy

Author: Pelliccia, F., Seggewiss, H., Cecchi, F., Calabrò, P., Limongelli, G., Alfieri, O., Ferrazzi, P., Yacoub, M. H., and Olivotto, I.
Published: 2021
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6. Razionale e valore fondamentale della Rete Italiana integrata dell’Amiloidosi Cardiaca [Rationale and significance of the Italian Network for Cardiac Amyloidosis]

Author: Sinagra G., Emdin M., Merlo M., Vergaro G., Aimo A., Biagini E., Imazio M., Porcari A., Limongelli G., Cipriani A., Canepa M., Musumeci B., Cameli M., Crotti L., Di Bella G., Di Lenarda A., Cappelli F., Chimenti C., Obici L., Iacoviello M., Perlini S., Pieroni M., Metra M., Oliva F., Perrone Filardi P., Colivicchi F., Indolfi C., Sinagra, G, Emdin, M, Merlo, M, Vergaro, G, Aimo, A, Biagini, E, Imazio, M, Porcari, A, Limongelli, G, Cipriani, A, Canepa, M, Musumeci, B, Cameli, M, Crotti, L, Di Bella, G, Di Lenarda, A, Cappelli, F, Chimenti, C, Obici, L, Iacoviello, M, Perlini, S, Pieroni, M, Metra, M, Oliva, F, Perrone Filardi, P, Colivicchi, F, and Indolfi, C
Subjects: Treatment, Clinical management, Cardiac amyloidosi, Differential diagnosi, Perspective
Abstract: The perspective on amyloidosis has changed deeply over the last 10 years following major advances in diagnosis and treatment options, especially in cardiac amyloidosis. This intrinsically heterogeneous disease exposes to the risk of fragmentation of knowledge and requires the interaction among experts of different specialties and subspecialties. Suspicion of disease, timely recognition and confirmation of final diagnosis, prognostic stratification, clinical management and therapeutic strategies represent essential steps to be taken. Missing or delaying the diagnosis may have dramatic impact on patient outcome, as in the case of chemotherapy in unrecognized light-chain amyloidosis. Therefore, there is an urgent need for the foundation of an Italian Amyloidosis Network to deal with the challenges of this condition and orient clinical management at national and local levels. The present consensus document aims to provide the rationale and scopes of the Italian Amyloidosis Network, which has been conceived as an organizational framework for professionals managing patients with amyloidosis.
Published: 2023

7. 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

8. Multiple hormonal and metabolic deficiency syndrome in chronic heart failure: rationale, design, and demographic characteristics of the T.O.S.CA. Registry

Author: Bossone, E., Arcopinto, M., Iacoviello, M., Triggiani, V., Cacciatore, F., Maiello, C., Limongelli, G., Masarone, D., Perticone, F., Sciacqua, A., Perrone-Filardi, P., Mancini, A., Volterrani, M., Vriz, O., Castello, R., Passantino, A., Campo, M., Modesti, P. A., De Giorgi, A., Monte, I., Puzzo, A., Ballotta, A., Caliendo, L., D’Assante, R., Marra, A. M., Salzano, A., Suzuki, T., Cittadini, A., and On behalf of TOSCA Investigators
Published: 2018
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9. C63 PREVALENCE AND CLINICAL SIGNIFICANCE OF RIGHT VENTRICULAR PULMONARY ARTERIAL UNCOUPLING IN CARDIAC AMYLOIDOSIS

Author: Palmiero, G, primary, Monda, E, additional, Verrillo, F, additional, Dongiglio, F, additional, Caiazza, M, additional, Rubino, M, additional, Lioncino, M, additional, Diana, G, additional, Vetrano, E, additional, Fusco, A, additional, Cirillo, A, additional, Mauriello, A, additional, Ciccarelli, G, additional, De Rimini, M, additional, D’Alto, M, additional, Cerciello, G, additional, D‘Andrea, A, additional, Golino, P, additional, Calabrò, P, additional, Bossone, E, additional, and Limongelli, G, additional
Published: 2023
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10. Syncope in hypertrophic cardiomyopathy (part II): An expert consensus statement on the diagnosis and management

Author: Brignole, M, Cecchi, F, Anastasakis, A, Crotti, L, Deharo, J, Elliott, P, Fedorowski, A, Kaski, J, Limongelli, G, Maron, M, Olivotto, I, Ommen, S, Parati, G, Shen, W, Ungar, A, Wilde, A, Brignole, Michele, Cecchi, Franco, Anastasakis, Aris, Crotti, Lia, Deharo, Jean Claude, Elliott, Perry M, Fedorowski, Artur, Kaski, Juan Pablo, Limongelli, Giuseppe, Maron, Martin S, Olivotto, Iacopo, Ommen, Steve R, Parati, Gianfranco, Shen, Win, Ungar, Andrea, Wilde, Arthur, Brignole, M, Cecchi, F, Anastasakis, A, Crotti, L, Deharo, J, Elliott, P, Fedorowski, A, Kaski, J, Limongelli, G, Maron, M, Olivotto, I, Ommen, S, Parati, G, Shen, W, Ungar, A, Wilde, A, Brignole, Michele, Cecchi, Franco, Anastasakis, Aris, Crotti, Lia, Deharo, Jean Claude, Elliott, Perry M, Fedorowski, Artur, Kaski, Juan Pablo, Limongelli, Giuseppe, Maron, Martin S, Olivotto, Iacopo, Ommen, Steve R, Parati, Gianfranco, Shen, Win, Ungar, Andrea, and Wilde, Arthur
Abstract: Syncopal events in patients with hypertrophic cardiomyopathy (HCM) are of concern as they are a vital consideration in algorithms for risk stratification for sudden cardiac death (SCD) and ICD implantation. However, the cause of syncope is often under-investigated and/or unexplained. Current syncope guidelines do not provide a detailed definition of unexplained syncope. To address this important gap, an international panel of experts in the field of both syncope and HCM wrote a consensus document with the aim of providing practical guidance for the diagnosis and management of syncope in patients with HCM.
Published: 2023

11. The frequency of rare and monogenic diseases in pediatric organ transplant recipients in Italy

Author: Vaisitti T., Peritore D., Magistroni P., Ricci A., Lombardini L., Gringeri E., Catalano S., Spada M., Sciveres M., Di Giorgio A., Limongelli G., Varrenti M., Gerosa G., Terzi A., Napoleone C. P., Amodeo A., Ragni L., Strologo L., Benetti E., Fontana I., Testa S., Peruzzi L., Mitrotti A., Abbate S., Comai G., Gotti E., Schiavon M., Boffini M., De Angelis D., Bertani A., Pinelli D., Torre M., Poggi C., Deaglio S., Cardillo M., Amoroso A., Serena A., Giorgia C., Vaisitti, T., Peritore, D., Magistroni, P., Ricci, A., Lombardini, L., Gringeri, E., Catalano, S., Spada, M., Sciveres, M., Di Giorgio, A., Limongelli, G., Varrenti, M., Gerosa, G., Terzi, A., Napoleone, C. P., Amodeo, A., Ragni, L., Strologo, L., Benetti, E., Fontana, I., Testa, S., Peruzzi, L., Mitrotti, A., Abbate, S., Comai, G., Gotti, E., Schiavon, M., Boffini, M., De Angelis, D., Bertani, A., Pinelli, D., Torre, M., Poggi, C., Deaglio, S., Cardillo, M., Amoroso, A., Serena, A., and Giorgia, C.
Subjects: Registrie, Pediatrics, medicine.medical_specialty, Transplant outcome, Transplant Recipient, Monogenic diseases, Organ transplantation, Rare diseases, Child, Humans, Italy, Quality of Life, Registries, Transplant Recipients, Kidney Transplantation, Organ Transplantation, Disease, Quality of life, medicine, Pharmacology (medical), Monogenic disease, Genetics (clinical), Lung, business.industry, Research, Liver and kidney, General Medicine, Transplantation, medicine.anatomical_structure, Cohort, Medicine, business, Rare disease, Human
Abstract: Background Rare diseases are chronic and life-threatening disorders affecting Results To the purpose of our analysis, we considered heart, lung, liver and kidney transplants included in the Transplant Registry (TR) of the Italian National Transplantation Center in the 2002–2019 timeframe. Overall, 49,404 recipients were enrolled in the cohort, 5.1% of whom in the pediatric age. For 40,909 (82.8%) transplant recipients, a disease diagnosis was available, of which 38,615 in the adult cohort, while 8,495 patients (17.2%) were undiagnosed. There were 128 disease categories, and of these, 117 were listed in the main rare disease databases. In the pediatric cohort, 2,294 (5.6%) patients had a disease diagnosis: of the 2,126 (92.7%) patients affected by a rare disease, 1,402 (61.1%) presented with a monogenic condition. As expected, the frequencies of pathologies leading to organ failure were different between the pediatric and the adult cohort. Moreover, the pediatric group was characterized, compared to the adult one, by an overall better survival of the graft at ten years after transplant, with the only exception of lung transplants. When comparing survival considering rare vs non-rare diseases or rare and monogenic vs rare non-monogenic conditions, no differences were highlighted for kidney and lung transplants, while rare diseases had a better survival in liver as opposed to heart transplants. Conclusions This work represents the first national survey analyzing the main genetic causes and frequencies of rare and/or monogenic diseases leading to organ failure and requiring transplantation both in adults and children.
Published: 2021

12. Indications and management of implantable cardioverter-defibrillator therapy in childhood hypertrophic cardiomyopathy

Author: Kaski, J.P., Kammeraad, J.A.E., Blom, N.A., Happonen, J.M., Janousek, J., Klaassen, S., Limongelli, G., Östman-Smith, I., Sarquella Brugada, G., and Ziolkowska, L.
Subjects: Cardiovascular and Metabolic Diseases
Abstract: Sudden cardiac death is the most common mode of death during childhood and adolescence in hypertrophic cardiomyopathy, and identifying those individuals at highest risk is a major aspect of clinical care. The mainstay of preventative therapy is the implantable cardioverter-defibrillator, which has been shown to be effective at terminating malignant ventricular arrhythmias in children with hypertrophic cardiomyopathy but can be associated with substantial morbidity. Accurate identification of those children at highest risk who would benefit most from implantable cardioverter-defibrillator implantation while minimising the risk of complications is, therefore, essential. This position statement, on behalf of the Association for European Paediatric and Congenital Cardiology (AEPC), reviews the currently available data on established and proposed risk factors for sudden cardiac death in childhood-onset hypertrophic cardiomyopathy and current approaches for risk stratification in this population. It also provides guidance on identification of individuals at risk of sudden cardiac death and optimal management of implantable cardioverter-defibrillators in children and adolescents with hypertrophic cardiomyopathy.
Published: 2023

13. Diagnostic issues faced by a rare disease healthcare network during Covid-19 outbreak: data from the Campania Rare Disease Registry

Author: Limongelli G, Iucolano S, Monda E, Elefante P, De Stasio C, Lubrano I, Caiazza M, Mazzella M, Fimiani F, Galdo M, De Marchi G, Esposito M, Rubino M, Cirillo A, Fusco A, Esposito A, Trama U, Esposito S, Scarano G, Sepe J, Andria G, Orlando V, Menditto E, Chiodini P, Campania Rare Disease, Iolascon A, Franzese A, Sanduzzi Zamparelli A, Tessitore A, Romano A, Venosa A, Nunzia Olivieri A, Bianco A, La Manna A, Cerbone AM, Spasiano A, Agnese Stanziola A, Colao A, De Bellis A, Gambale A, Toriello A, Tufano A, Ciampa A, Maria Risitano A, Pisani A, Russo A, Volpe A, De Martino B, Amato B, De Fusco C, Piscopo C, Selleri C, Tucci C, Pignata C, Cioffi D, Melis D, Pasquali D, De Brasi D, Spitaleri D, Russo D, Martellotta D, De Michele E, Varricchio E, Miraglia Del Giudice E, Coscioni E, Cimino E, Pane F, Tranfa F, Pollio F, Lonardo F, Nuzzi F, Simonelli F, Trojsi F, Habetswallner F, Valentini G, Cerbone G, Parenti G, Tedeschi G, Capasso G, Battista Rossi G, Gaglione G, Sarnelli G, Argenziano G, Bellastella G, De Michele G, Fiorentino G, Spadaro G, Scala I, Santoro L, Zeppa L, Auricchio L, Elio Adinolfi L, Alessio M, Amitrano M, Savanelli MC, Russo MG, Ferrucci MG, Carbone MT, Pellecchia MT, Salerno M, Melone M, Del Donno M, Vitale M, Triggiani M, Della Monica M, Lo Presti M, Tenuta M, Mignogna MD, Schiavulli M, Zacchia M, Brunetti-Pierri N, Iovino P, Moscato P, Iandoli R, Scarpa R, Russo R, Troisi S, Sbordone S, Perrotta S, Fecarotta S, Sampaolo S, Cicalese V., Limongelli, Giuseppe, Iucolano, Stefano, Monda, Emanuele, Elefante, Pasquale, De Stasio, Chiara, Lubrano, Imma, Caiazza, Martina, Mazzella, Marialuisa, Fimiani, Fabio, Galdo, Maria, De Marchi, Giulia, Esposito, Martina, Rubino, Marta, Cirillo, Annapaola, Fusco, Adelaide, Esposito, Augusto, Trama, Ugo, Esposito, Salvatore, Scarano, Gioacchino, Sepe, Joseph, Andria, Generoso, Orlando, Valentina, Menditto, Enrica, Chiodini, Paolo, Iolascon, Achille, Franzese, Adriana, Sanduzzi Zamparelli, Alessandro, Tessitore, Alessandro, Romano, Alfonso, Venosa, Alfredo, Nunzia Olivieri, Alma, Bianco, Andrea, La Manna, Angela, Cerbone, Anna Maria, Spasiano, Anna, Agnese Stanziola, Anna, Colao, Annamaria, De Bellis, Annamaria, Gambale, Antonella, Toriello, Antonella, Tufano, Antonella, Ciampa, Antonio, Maria Risitano, Antonio, Pisani, Antonio, Russo, Antonio, Volpe, Antonio, De Martino, Bernardo, Amato, Bruno, De Fusco, Carmela, Piscopo, Carmelo, Selleri, Carmine, Tucci, Celeste, Pignata, Claudio, Cioffi, Daniela, Melis, Daniela, Pasquali, Daniela, De Brasi, Daniele, Spitaleri, Daniele, De Brasi, Davide, Russo, Domenico, Martellotta, Donata, De Michele, Elisa, Varricchio, Elziario, Miraglia Del Giudice, Emanuele, Coscioni, Enrico, Cimino, Ernesto, Pane, Fabrizio, Tranfa, Fausto, Pollio, Filiberto, Lonardo, Fortunato, Nuzzi, Francesca, Simonelli, Francesca, Trojsi, Francesca, Habetswallner, Francesco, Valentini, Gabriele, Cerbone, Gaetana, Parenti, Giancarlo, Tedeschi, Gioacchino, Capasso, Giovambattista, Battista Rossi, Giovanni, Gaglione, Giovanni, Sarnelli, Giovanni, Argenziano, Giuseppe, Bellastella, Giuseppe, De Michele, Giuseppe, Fiorentino, Giuseppe, Spadaro, Giuseppe, Scala, Iri, Santoro, Lucio, Zeppa, Lucio, Auricchio, Luigi, Elio Adinolfi, Luigi, Alessio, Maria, Amitrano, Maria, Savanelli, Maria Cristina, Russo, Maria Giovanna, Ferrucci, Maria Grazia, Carbone, Maria Teresa, Pellecchia, Maria Teresa, Salerno, Mariacarolina, Melone, Marina, Del Donno, Mario, Vitale, Mario, Triggiani, Massimo, Della Monica, Matteo, Lo Presti, Maurizio, Tenuta, Maurizio, Mignogna, Michele Davide, Schiavulli, Michele, Zacchia, Miriam, Brunetti-Pierri, Nicola, Iovino, Paola, Moscato, Paolo, Iandoli, Raffaele, Scarpa, Raffaele, Russo, Romualdo, Troisi, Salvatore, Sbordone, Sandro, Perrotta, Silverio, Fecarotta, Simona, Sampaolo, Simone, Cicalese, Virgilio, Limongelli, G, Iucolano, S, Monda, E, Elefante, P, De Stasio, C, Lubrano, I, Caiazza, M, Mazzella, M, Fimiani, F, Galdo, M, De Marchi, G, Esposito, M, Rubino, M, Cirillo, A, Fusco, A, Esposito, A, Trama, U, Esposito, S, Scarano, G, Sepe, J, Andria, G, Orlando, V, Menditto, E, Chiodini, P, Campania Rare, Disease, Iolascon, A, Franzese, A, Sanduzzi Zamparelli, A, Tessitore, A, Romano, A, Venosa, A, Nunzia Olivieri, A, Bianco, A, La Manna, A, Cerbone, Am, Spasiano, A, Agnese Stanziola, A, Colao, A, De Bellis, A, Gambale, A, Toriello, A, Tufano, A, Ciampa, A, Maria Risitano, A, Pisani, A, Russo, A, Volpe, A, De Martino, B, Amato, B, De Fusco, C, Piscopo, C, Selleri, C, Tucci, C, Pignata, C, Cioffi, D, Melis, D, Pasquali, D, De Brasi, D, Spitaleri, D, Russo, D, Martellotta, D, De Michele, E, Varricchio, E, Miraglia Del Giudice, E, Coscioni, E, Cimino, E, Pane, F, Tranfa, F, Pollio, F, Lonardo, F, Nuzzi, F, Simonelli, F, Trojsi, F, Habetswallner, F, Valentini, G, Cerbone, G, Parenti, G, Tedeschi, G, Capasso, G, Battista Rossi, G, Gaglione, G, Sarnelli, G, Argenziano, G, Bellastella, G, De Michele, G, Fiorentino, G, Spadaro, G, Scala, I, Santoro, L, Zeppa, L, Auricchio, L, Elio Adinolfi, L, Alessio, M, Amitrano, M, Savanelli, Mc, Russo, Mg, Ferrucci, Mg, Carbone, Mt, Pellecchia, Mt, Salerno, M, Melone, M, Del Donno, M, Vitale, M, Triggiani, M, Della Monica, M, Lo Presti, M, Tenuta, M, Mignogna, Md, Schiavulli, M, Zacchia, M, Brunetti-Pierri, N, Iovino, P, Moscato, P, Iandoli, R, Scarpa, R, Russo, R, Troisi, S, Sbordone, S, Perrotta, S, Fecarotta, S, Sampaolo, S, and Cicalese, V.
Subjects: Pediatrics, medicine.medical_specialty, Coronavirus disease 2019 (COVID-19), Home therapy, rare diseases, 030204 cardiovascular system & hematology, Campania region, Covid-19, Italy, patient registry, Disease Outbreaks, 03 medical and health sciences, COVID-19 Testing, 0302 clinical medicine, Pandemic, Health care, medicine, Retrospective analysis, Humans, AcademicSubjects/MED00860, Registries, 030212 general & internal medicine, Medical diagnosis, Pandemics, Retrospective Studies, business.industry, Public Health, Environmental and Occupational Health, Outbreak, General Medicine, Original Article, business, Delivery of Health Care, Rare disease
Abstract: Background The aims of this study were: to investigate the capacity of the rare disease healthcare network in Campania to diagnose patients with rare diseases during the outbreak of Covid-19; and to shed light on problematic diagnoses during this period. Methods To describe the impact of the Covid-19 pandemic on the diagnosis of patients with rare diseases, a retrospective analysis of the Campania Region Rare Disease Registry was performed. A tailored questionnaire was sent to rare disease experts to investigate major issues during the emergency period. Results Prevalence of new diagnoses of rare disease in March and April 2020 was significantly lower than in 2019 (117 versus 317, P Conclusions This study describes the effects of the Covid-19 outbreak on the diagnosis of rare disease in a single Italian region and investigates potential issues of diagnosis and management during this period.
Published: 2021

14. Differences between familial and sporadic dilated cardiomyopathy: ESC EORP Cardiomyopathy & Myocarditis registry

Author: Asselbergs F. W., Sammani A., Elliott P., Gimeno J. R., Tavazzi L., Tendera M., Kaski J. P., Maggioni A. P., Rubis P. P., Jurcut R., Helio T., Calo L., Sinagra G., Zdravkovic M., Olivotto I., Kavoliuniene A., Laroche C., Caforio A. L. P., Charron P., Komissarova S., Chakova N., Niyazova S., Linhart A., Kuchynka P., Palecek T., Podzimkova J., Fikrle M., Nemecek E., Bundgaard H., Tfelt-Hansen J., Theilade J., Thune J. J., Axelsson A., Mogensen J., Henriksen F., Hey T., Nielsen S. K., Videbaek L., Andreasen S., Arnsted H., Saad A., Ali M., Lommi J., Nieminennew M. S., Dubourg O., Mansencal N., Arslan M., Siam Tsieu V., Damy T., Guellich A., Guendouz S., Tissot C. M., Lamine A., Rappeneau S., Hagege A., Desnos M., Bachet A., Hamzaoui M., Isnard R., Legrand L., Maupain C., Gandjbakhch E., Kerneis M., Pruny J. -F., Bauer A., Pfeiffer B., Felix S. B., Dorr M., Kaczmarek S., Lehnert K., Pedersen A. -L., Beug D., Bruder M., Bohm M., Kindermann I., Linicus Y., Werner C., Neurath B., Schild-Ungerbuehler M., Seggewiss H., Neugebauer A., McKeown P., Muir A., McOsker J., Jardine T., Divine G., Lorenzini M., Watkinson O., Wicks E., Iqbal H., Mohiddin S., O'Mahony C., Sekri N., Carr-White G., Bueser T., Rajani R., Clack L., Damm J., Jones S., Sanchez-Vidal R., Smith M., Walters T., Wilson K., Rosmini S., Anastasakis A., Ritsatos K., Vlagkouli V., Forster T., Sepp R., Borbas J., Nagy V., Tringer A., Kakonyi K., Szabo L. A., Maleki M., Noohi Bezanjani F., Amin A., Naderi N., Parsaee M., Taghavi S., Ghadrdoost B., Jafari S., Khoshavi M., Rapezzi C., Biagini E., Corsini A., Gagliardi C., Graziosi M., Longhi S., Milandri A., Ragni L., Palmieri S., Arretini A., Castelli G., Cecchi F., Fornaro A., Tomberli B., Spirito P., Devoto E., Della Bella P., Maccabelli G., Sala S., Guarracini F., Peretto G., Russo M. G., Calabro R., Pacileo G., Limongelli G., Masarone D., Pazzanese V., Rea A., Rubino M., Tramonte S., Valente F., Caiazza M., Cirillo A., Del Giorno G., Esposito A., Gravino R., Marrazzo T., Trimarco B., Losi M. -A., Di Nardo C., Giamundo A., Musella F., Pacelli F., Scatteia A., Canciello G., Caforio A., Iliceto S., Calore C., Leoni L., Perazzolo Marra M., Rigato I., Tarantini G., Schiavo A., Testolina M., Arbustini E., Di Toro A., Giuliani L. P., Serio A., Fedele F., Frustaci A., Alfarano M., Chimenti C., Drago F., Baban A., Lanzillo C., Martino A., Uguccioni M., Zachara E., Halasz G., Re F., Carriere C., Merlo M., Ramani F., Krivickiene A., Tamuleviciute-Prasciene E., Viezelis M., Celutkiene J., Balkeviciene L., Laukyte M., Paleviciute E., Pinto Y., Wilde A., Van Der Heijden J., Van Laake L., De Jonge N., Hassink R., Kirkels J. H., Ajuluchukwu J., Olusegun-Joseph A., Ekure E., Mizia-Stec K., Czekaj A., Sikora-Puz A., Skoczynska A., Wybraniec M., Rubis P., Dziewiecka E., Wisniowska-Smialek S., Bilinska Z., Chmielewski P., Foss-Nieradko B., Michalak E., Stepien-Wojno M., Mazek B., Rocha Lopes L., Almeida A. R., Cruz I., Gomes A. C., Pereira A. R., Brito D., Madeira H., Francisco A. R., Menezes M., Moldovan O., Oliveira Guimaraes T., Silva D., Ginghina C., Mursa A., Popescu B. A., Apetrei E., Militaru S., Mircea Coman I., Frigy A., Fogarasi Z., Kocsis I., Szabo I. A., Fehervari L., Nikitin I., Resnik E., Komissarova M., Lazarev V., Shebzukhova M., Ustyuzhanin D., Blagova O., Alieva I., Kulikova V., Lutokhina Y., Pavlenko E., Varionchik N., Ristic A. D., Seferovic P. M., Veljic I., Zivkovic I., Milinkovic I., Pavlovic A., Radovanovic G., Simeunovic D., Aleksic M., Djokic J., Hinic S., Klasnja S., Mircetic K., Monserrat L., Fernandez X., Garcia-Giustiniani D., Larranaga J. M., Ortiz-Genga M., Barriales-Villa R., Martinez-Veira C., Veira E., Cequier A., Salazar-Mendiguchia J., Manito N., Gonzalez J., Fernandez-Aviles F., Medrano C., Yotti R., Cuenca S., Espinosa M. A., Mendez I., Zatarain E., Alvarez R., Garcia-Pavia P., Briceno A., Cobo-Marcos M., Dominguez F., De Teresa Galvan E., Garcia Pinilla J. M., Abdeselam-Mohamed N., Lopez-Garrido M. A., Morcillo Hidalgo L., Ortega-Jimenez M. V., Robles Mezcua A., Guijarro-Contreras A., Gomez-Garcia D., Robles-Mezcua M., Gimeno Blanes J. R., Castro F. J., Munoz Esparza C., Sabater Molina M., Sorli Garcia M., Lopez Cuenca D., Ripoll-Vera T., Alvarez J., Nunez J., Gomez Y., Sanchez Fernandez P. L., Villacorta E., Avila C., Bravo L., Diaz-Pelaez E., Gallego-Delgado M., Garcia-Cuenllas L., Plata B., Lopez-Haldon J. E., Pena Pena M. L., Cantero Perez E. M., Zorio E., Arnau M. A., Sanz J., Marques-Sulex E., University Medical Center [Utrecht], University College of London [London] (UCL), Hospital Univeristario Virgen de la Arrixaca, University Hospital of Ferrara and Maria Cecilia Hospital, Medical University of Silesia, Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA), Chair of Medical Biochemistry, Jagiellonian University - Medical College, Chair of Medical Biochemistry, Emergency Hospital Floreasca Bucharest, Emergency Hospital Floreasca Bucharest, 8 Calea Floresca, Sector 1, 014461 Bucharest, Romania, University of Helsinki, Policlinico Casilino (Ospedale Policlinico Casilino), University of Trieste, University of Belgrade [Belgrade], Careggi University Hospital, Lithuanian University of health Sciences [Kaunas], Universita degli Studi di Padova, Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Institute of cardiometabolism and nutrition (ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hospital Clínico Universitario Virgen de la Arrixaca = University Hospital Virgen de la Arrixaca [Murcia], Medical University of Silesia (SUM), Université Nice Sophia Antipolis (1965 - 2019) (UNS), Helsingin yliopisto = Helsingfors universitet = University of Helsinki, Università degli studi di Trieste = University of Trieste, Università degli Studi di Padova = University of Padua (Unipd), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases (ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Institut de Cardiométabolisme et Nutrition = Institute of Cardiometabolism and Nutrition [CHU Pitié Salpêtrière] (IHU ICAN), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), HAL-SU, Gestionnaire, Asselbergs, F. W., Sammani, A., Elliott, P., Gimeno, J. R., Tavazzi, L., Tendera, M., Kaski, J. P., Maggioni, A. P., Rubis, P. P., Jurcut, R., Helio, T., Calo, L., Sinagra, G., Zdravkovic, M., Olivotto, I., Kavoliuniene, A., Laroche, C., Caforio, A. L. P., Charron, P., Komissarova, S., Chakova, N., Niyazova, S., Linhart, A., Kuchynka, P., Palecek, T., Podzimkova, J., Fikrle, M., Nemecek, E., Bundgaard, H., Tfelt-Hansen, J., Theilade, J., Thune, J. J., Axelsson, A., Mogensen, J., Henriksen, F., Hey, T., Nielsen, S. K., Videbaek, L., Andreasen, S., Arnsted, H., Saad, A., Ali, M., Lommi, J., Nieminennew, M. S., Dubourg, O., Mansencal, N., Arslan, M., Siam Tsieu, V., Damy, T., Guellich, A., Guendouz, S., Tissot, C. M., Lamine, A., Rappeneau, S., Hagege, A., Desnos, M., Bachet, A., Hamzaoui, M., Isnard, R., Legrand, L., Maupain, C., Gandjbakhch, E., Kerneis, M., Pruny, J. -F., Bauer, A., Pfeiffer, B., Felix, S. B., Dorr, M., Kaczmarek, S., Lehnert, K., Pedersen, A. -L., Beug, D., Bruder, M., Bohm, M., Kindermann, I., Linicus, Y., Werner, C., Neurath, B., Schild-Ungerbuehler, M., Seggewiss, H., Neugebauer, A., Mckeown, P., Muir, A., Mcosker, J., Jardine, T., Divine, G., Lorenzini, M., Watkinson, O., Wicks, E., Iqbal, H., Mohiddin, S., O'Mahony, C., Sekri, N., Carr-White, G., Bueser, T., Rajani, R., Clack, L., Damm, J., Jones, S., Sanchez-Vidal, R., Smith, M., Walters, T., Wilson, K., Rosmini, S., Anastasakis, A., Ritsatos, K., Vlagkouli, V., Forster, T., Sepp, R., Borbas, J., Nagy, V., Tringer, A., Kakonyi, K., Szabo, L. A., Maleki, M., Noohi Bezanjani, F., Amin, A., Naderi, N., Parsaee, M., Taghavi, S., Ghadrdoost, B., Jafari, S., Khoshavi, M., Rapezzi, C., Biagini, E., Corsini, A., Gagliardi, C., Graziosi, M., Longhi, S., Milandri, A., Ragni, L., Palmieri, S., Arretini, A., Castelli, G., Cecchi, F., Fornaro, A., Tomberli, B., Spirito, P., Devoto, E., Della Bella, P., Maccabelli, G., Sala, S., Guarracini, F., Peretto, G., Russo, M. G., Calabro, R., Pacileo, G., Limongelli, G., Masarone, D., Pazzanese, V., Rea, A., Rubino, M., Tramonte, S., Valente, F., Caiazza, M., Cirillo, A., Del Giorno, G., Esposito, A., Gravino, R., Marrazzo, T., Trimarco, B., Losi, M. -A., Di Nardo, C., Giamundo, A., Musella, F., Pacelli, F., Scatteia, A., Canciello, G., Caforio, A., Iliceto, S., Calore, C., Leoni, L., Perazzolo Marra, M., Rigato, I., Tarantini, G., Schiavo, A., Testolina, M., Arbustini, E., Di Toro, A., Giuliani, L. P., Serio, A., Fedele, F., Frustaci, A., Alfarano, M., Chimenti, C., Drago, F., Baban, A., Lanzillo, C., Martino, A., Uguccioni, M., Zachara, E., Halasz, G., Re, F., Carriere, C., Merlo, M., Ramani, F., Krivickiene, A., Tamuleviciute-Prasciene, E., Viezelis, M., Celutkiene, J., Balkeviciene, L., Laukyte, M., Paleviciute, E., Pinto, Y., Wilde, A., Van Der Heijden, J., Van Laake, L., De Jonge, N., Hassink, R., Kirkels, J. H., Ajuluchukwu, J., Olusegun-Joseph, A., Ekure, E., Mizia-Stec, K., Czekaj, A., Sikora-Puz, A., Skoczynska, A., Wybraniec, M., Rubis, P., Dziewiecka, E., Wisniowska-Smialek, S., Bilinska, Z., Chmielewski, P., Foss-Nieradko, B., Michalak, E., Stepien-Wojno, M., Mazek, B., Rocha Lopes, L., Almeida, A. R., Cruz, I., Gomes, A. C., Pereira, A. R., Brito, D., Madeira, H., Francisco, A. R., Menezes, M., Moldovan, O., Oliveira Guimaraes, T., Silva, D., Ginghina, C., Mursa, A., Popescu, B. A., Apetrei, E., Militaru, S., Mircea Coman, I., Frigy, A., Fogarasi, Z., Kocsis, I., Szabo, I. A., Fehervari, L., Nikitin, I., Resnik, E., Komissarova, M., Lazarev, V., Shebzukhova, M., Ustyuzhanin, D., Blagova, O., Alieva, I., Kulikova, V., Lutokhina, Y., Pavlenko, E., Varionchik, N., Ristic, A. D., Seferovic, P. M., Veljic, I., Zivkovic, I., Milinkovic, I., Pavlovic, A., Radovanovic, G., Simeunovic, D., Aleksic, M., Djokic, J., Hinic, S., Klasnja, S., Mircetic, K., Monserrat, L., Fernandez, X., Garcia-Giustiniani, D., Larranaga, J. M., Ortiz-Genga, M., Barriales-Villa, R., Martinez-Veira, C., Veira, E., Cequier, A., Salazar-Mendiguchia, J., Manito, N., Gonzalez, J., Fernandez-Aviles, F., Medrano, C., Yotti, R., Cuenca, S., Espinosa, M. A., Mendez, I., Zatarain, E., Alvarez, R., Garcia-Pavia, P., Briceno, A., Cobo-Marcos, M., Dominguez, F., De Teresa Galvan, E., Garcia Pinilla, J. M., Abdeselam-Mohamed, N., Lopez-Garrido, M. A., Morcillo Hidalgo, L., Ortega-Jimenez, M. V., Robles Mezcua, A., Guijarro-Contreras, A., Gomez-Garcia, D., Robles-Mezcua, M., Gimeno Blanes, J. R., Castro, F. J., Munoz Esparza, C., Sabater Molina, M., Sorli Garcia, M., Lopez Cuenca, D., Ripoll-Vera, T., Alvarez, J., Nunez, J., Gomez, Y., Sanchez Fernandez, P. L., Villacorta, E., Avila, C., Bravo, L., Diaz-Pelaez, E., Gallego-Delgado, M., Garcia-Cuenllas, L., Plata, B., Lopez-Haldon, J. E., Pena Pena, M. L., Cantero Perez, E. M., Zorio, E., Arnau, M. A., Sanz, J., Marques-Sulex, E., Cardiology, ACS - Heart failure & arrhythmias, HUS Heart and Lung Center, Clinicum, Department of Medicine, Kardiologian yksikkö, Helsinki University Hospital Area, and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)
Subjects: Registrie, lcsh:Diseases of the circulatory (Cardiovascular) system, EUROBSERVATIONAL RESEARCH-PROGRAM, Dilated cardiomyopathy, Europe, Familial, Genetic, Prognosis, Sporadic, Adult, Humans, Prospective Studies, Registries, Cardiomyopathies, Cardiomyopathy, Dilated, Myocarditis, Cardiomyopathy, 030204 cardiovascular system & hematology, 0302 clinical medicine, Original Research Articles, Dilated, PILOT, Original Research Article, 030212 general & internal medicine, Prospective cohort study, Ejection fraction, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, medicine.diagnostic_test, Guideline adherence, 3. Good health, Cardiology and Cardiovascular Medicine, Human, medicine.medical_specialty, Prognosi, FREQUENCY, 03 medical and health sciences, Internal medicine, medicine, Cardiomyopathie, Genetic testing, business.industry, medicine.disease, Prospective Studie, lcsh:RC666-701, 3121 General medicine, internal medicine and other clinical medicine, Heart failure, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: International audience; AimsDilated cardiomyopathy (DCM) is a complex disease where genetics interplay with extrinsic factors. This study aims to compare the phenotype, management, and outcome of familial DCM (FDCM) and non‐familial (sporadic) DCM (SDCM) across Europe.Methods and resultsPatients with DCM that were enrolled in the prospective ESC EORP Cardiomyopathy & Myocarditis Registry were included. Baseline characteristics, genetic testing, genetic yield, and outcome were analysed comparing FDCM and SDCM; 1260 adult patients were studied (238 FDCM, 707 SDCM, and 315 not disclosed). Patients with FDCM were younger (P < 0.01), had less severe disease phenotype at presentation (P < 0.02), more favourable baseline cardiovascular risk profiles (P ≤ 0.007), and less medication use (P ≤ 0.042). Outcome at 1 year was similar and predicted by NYHA class (HR 0.45; 95% CI [0.25–0.81]) and LVEF per % decrease (HR 1.05; 95% CI [1.02–1.08]. Throughout Europe, patients with FDCM received more genetic testing (47% vs. 8%, P < 0.01) and had higher genetic yield (55% vs. 22%, P < 0.01).ConclusionsWe observed that FDCM and SDCM have significant differences at baseline but similar short‐term prognosis. Whether modification of associated cardiovascular risk factors provide opportunities for treatment remains to be investigated. Our results also show a prevalent role of genetics in FDCM and a non‐marginal yield in SDCM although genetic testing is largely neglected in SDCM. Limited genetic testing and heterogeneity in panels provides a scaffold for improvement of guideline adherence.
Published: 2021

15. HCM-AF South Italy score for prediction of new-onset atrial fibrillation in patients with hypertrophic cardiomyopathy: data from a derivation and validation italian cohorts

Author: Todde, G, primary, Monda, E, additional, Canciello, G, additional, Lioncino, M, additional, Lombardi, R, additional, Rubino, M, additional, Borrelli, F, additional, Caiazza, M, additional, Paoletta, D, additional, De Simone, G, additional, Pacileo, G, additional, Esposito, G, additional, Limongelli, G, additional, and Losi, M A, additional
Published: 2022
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16. Clinical phenotype and long-term outcome of patients with Anderson-Fabry disease followed at a multidisciplinary cardiomyopathy centre

Author: Verrillo, F, primary, Fumagalli, C, additional, Tassetti, L, additional, Zocchi, C, additional, Tanini, I, additional, Zampieri, M, additional, Maurizi, N, additional, Tomberli, A, additional, Baldini, K, additional, Argiro', A, additional, Cappelli, F, additional, Girolami, F, additional, Limongelli, G, additional, and Olivotto, I, additional
Published: 2022
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17. Association between common cardiovascular risk factors and clinical phenotype in patients with hypertrophic cardiomyopathy from the European Society of Cardiology (ESC) EurObservational Research Programme (EORP) Cardiomyopathy/Myocarditis registry

Author: Lopes, Luis R, Losi, Maria-Angela, Sheikh, Nabeel, Laroche, Cécile, Charron, Philippe, Gimeno, Juan, Kaski, Juan P, Maggioni, Aldo P, Tavazzi, Luigi, Arbustini, Eloisa, Brito, Dulce, Celutkiene, Jelena, Hagege, Albert, Linhart, Ales, Mogensen, Jens, Garcia-Pinilla, José Manuel, Ripoll-Vera, Tomas, Seggewiss, Hubert, Villacorta, Eduardo, Caforio, Alida, Elliott, Perry M, Komissarova, S, Chakova, N, Niyazova, S, Linhart, A, Kuchynka, P, Palecek, T, Podzimkova, J, Fikrle, M, Nemecek, E, Bundgaard, H, Tfelt-Hansen, J, Theilade, J, Thune, J J, Axelsson, A, Mogensen, J, Henriksen, F, Hey, T, Nielsen, S K, Videbaek, L, Andreasen, S, Arnsted, H, Saad, A, Ali, M, Lommi, J, Helio, T, Nieminen, M S, Dubourg, O, Mansencal, N, Arslan, M, Tsieu, V Siam, Damy, T, Guellich, A, Guendouz, S, Tissot, C M, Lamine, A, Rappeneau, S, Hagege, A, Desnos, M, Bachet, A, Hamzaoui, M, Charron, P, Isnard, R, Legrand, L, Maupain, C, Gandjbakhch, E, Kerneis, M, Pruny, J-F, Bauer, A, Pfeiffer, B, Felix, S B, Dorr, M, Kaczmarek, S, Lehnert, K, Pedersen, A-L, Beug, D, Bruder, M, Böhm, M, Kindermann, I, Linicus, Y, Werner, C, Neurath, B, Schild-Ungerbuehler, M, Seggewiss, H, Neugebauer, A, Mckeown, P, Muir, A, Mcosker, J, Jardine, T, Divine, G, Elliott, P, Lorenzini, M, Watkinson, O, Wicks, E, Iqbal, H, Mohiddin, S, O'Mahony, C, Sekri, N, Carr-White, G, Bueser, T, Rajani, R, Clack, L, Damm, J, Jones, S, Sanchez-Vidal, R, Smith, M, Walters, T, Wilson, K, Rosmini, S, Anastasakis, A, Ritsatos, K, Vlagkouli, V, Forster, T, Sepp, R, Borbas, J, Nagy, V, Tringer, A, Kakonyi, K, Szabo, L A, Maleki, M, Bezanjani, F Noohi, Amin, A, Naderi, N, Parsaee, M, Taghavi, S, Ghadrdoost, B, Jafari, S, Khoshavi, M, Rapezzi, C, Biagini, E, Corsini, A, Gagliardi, C, Graziosi, M, Longhi, S, Milandri, A, Ragni, L, Palmieri, S, Olivotto, I, Arretini, A, Castelli, G, Cecchi, F, Fornaro, A, Tomberli, B, Spirito, P, Devoto, E, Bella, P Della, Maccabelli, G, Sala, S, Guarracini, F, Peretto, G, Russo, M G, Calabro, R, Pacileo, G, Limongelli, G, Masarone, D, Pazzanese, V, Rea, A, Rubino, M, Tramonte, S, Valente, F, Caiazza, M, Cirillo, A, Del Giorno, G, Esposito, A, Gravino, R, Marrazzo, T, Trimarco, B, Losi, M-A, Nardo, C Di, Giamundo, A, Musella, F, Pacelli, F, Scatteia, A, Canciello, G, Caforio, A, Iliceto, S, Calore, C, Leoni, L, Marra, M Perazzolo, Rigato, I, Tarantini, G, Schiavo, A, Testolina, M, Arbustini, E, Toro, A Di, Giuliani, L P, Serio, A, Fedele, F, Frustaci, A, Alfarano, M, Chimenti, C, Drago, F, Baban, A, Calò, L, Lanzillo, C, Martino, A, Uguccioni, M, Zachara, E, Halasz, G, Re, F, Sinagra, G, Carriere, C, Merlo, M, Ramani, F, Kavoliuniene, A, Krivickiene, A, Tamuleviciute-Prasciene, E, Viezelis, M, Celutkiene, J, Balkeviciene, L, Laukyte, M, Paleviciute, E, Pinto, Y, Wilde, A, Asselbergs, F W, Sammani, A, Van Der Heijden, J, Van Laake, L, De Jonge, N, Hassink, R, Kirkels, J H, Ajuluchukwu, J, Olusegun-Joseph, A, Ekure, E, Mizia-Stec, K, Tendera, M, Czekaj, A, Sikora-Puz, A, Skoczynska, A, Wybraniec, M, Rubis, P, Dziewiecka, E, Wisniowska-Smialek, S, Bilinska, Z, Chmielewski, P, Nieradko, B Foss, Michalak, E, Stepien-Wojno, M, Mazek, B, Lopes, L Rocha, Almeida, A R, Cruz, I, Gomes, A C, Pereira, A R, Brito, D, Madeira, H, Francisco, A R, Menezes, M, Moldovan, O, Guimaraes, T Oliveira, Silva, D, Ginghina, C, Jurcut, R, Mursa, A, Popescu, B A, Apetrei, E, Militaru, S, Coman, I Mircea, Frigy, A, Fogarasi, Z, Kocsis, I, Szabo, I A, Fehervari, L, Nikitin, I, Resnik, E, Komissarova, M, Lazarev, V, Shebzukhova, M, Ustyuzhanin, D, Blagova, O, Alieva, I, Kulikova, V, Lutokhina, Y, Pavlenko, E, Varionchik, N, Ristic, A D, Seferovic, P M, Veljic, I, Zivkovic, I, Milinkovic, I, Pavlovic, A, Radovanovic, G, Simeunovic, D, Zdravkovic, M, Aleksic, M, Djokic, J, Hinic, S, Klasnja, S, Mircetic, K, Monserrat, L, Fernandez, X, Garcia-Giustiniani, D, Larrañaga, J M, Ortiz-Genga, M, Barriales-Villa, R, Martinez-Veira, C, Veira, E, Cequier, A, Salazar-Mendiguchia, J, Manito, N, Gonzalez, J, Fernández-Avilés, F, Medrano, C, Yotti, R, Cuenca, S, Espinosa, M A, Mendez, I, Zatarain, E, Alvarez, R, Pavia, P Garcia, Briceno, A, Cobo-Marcos, M, Dominguez, F, Galvan, E De Teresa, Pinilla, J M García, Abdeselam-Mohamed, N, Lopez-Garrido, M A, Hidalgo, L Morcillo, Ortega-Jimenez, M V, Mezcua, A Robles, Guijarro-Contreras, A, Gomez-Garcia, D, Robles-Mezcua, M, Blanes, J R Gimeno, Castro, F J, Esparza, C Munoz, Molina, M Sabater, García, M Sorli, Cuenca, D Lopez, Ripoll-Vera, T, Alvarez, J, Nunez, J, Gomez, Y, Fernandez, P L Sanchez, Villacorta, E, Avila, C, Bravo, L, Diaz-Pelaez, E, Gallego-Delgado, M, Garcia-Cuenllas, L, Plata, B, Lopez-Haldon, J E, Pena Pena, M L, Perez, E M Cantero, Zorio, E, Arnau, M A, Sanz, J, Marques-Sule, E, Gale, Christopher Peter, Beleslin, Branko, Budaj, Andrzej, Chioncel, Ovidiu, Dagres, Nikolaos, Danchin, Nicolas, Erlinge, David, Emberson, Jonathan, Glikson, Michael, Gray, Alastair, Kayikcioglu, Meral, Maggioni, Aldo, Nagy, Klaudia Vivien, Nedoshivin, Aleksandr, Petronio, Anna-Sonia, Hesselink, Jolien Roo, Wallentin, Lars, Zeymer, Uwe, Caforio, Alida, Blanes, Juan Ramon Gimeno, Charron, Philippe, Elliott, Perry, Kaski, Juan Pablo, Maggioni, Aldo P, Tavazzi, Luigi, Tendera, Michal, Komissarova, S., Chakova, N., Niyazova, S., Linhart, A., Kuchynka, P., Palecek, T., Podzimkova, J., Fikrle, M., Nemecek, E., Bundgaard, H., Tfelt-Hansen, J., Theilade, J., Thune, J J, Axelsson, A., Mogensen, J., Henriksen, F., Hey, T., Nielsen, S K, Videbaek, L., Andreasen, S., Arnsted, H., Saad, A., Ali, M., Lommi, J., Helio, T., Nieminen, M S, Dubourg, O., Mansencal, N., Arslan, M., Tsieu, V Siam, Damy, T., Guellich, A., Guendouz, S., Tissot, C M, Lamine, A., Rappeneau, S., Hagege, A., Desnos, M., Bachet, A., Hamzaoui, M., Charron, P., Isnard, R., Legrand, L., Maupain, C., Gandjbakhch, E., Kerneis, M., Pruny, J-F, Bauer, A., Pfeiffer, B., Felix, S B, Dorr, M., Kaczmarek, S., Lehnert, K., Pedersen, A-L, Beug, D., Bruder, M., Böhm, M., Kindermann, I., Linicus, Y., Werner, C., Neurath, B., Schild-Ungerbuehler, M., Seggewiss, H., Neugebauer, A., McKeown, P., Muir, A., McOsker, J., Jardine, T., Divine, G., Elliott, P., Lorenzini, M., Watkinson, O., Wicks, E., Iqbal, H., Mohiddin, S., O'Mahony, C., Sekri, N., Carr-White, G., Bueser, T., Rajani, R., Clack, L., Damm, J., Jones, S., Sanchez-Vidal, R., Smith, M., Walters, T., Wilson, K., Rosmini, S., Anastasakis, A., Ritsatos, K., Vlagkouli, V., Forster, T., Sepp, R., Borbas, J., Nagy, V., Tringer, A., Kakonyi, K., Szabo, L A, Maleki, M., Bezanjani, F Noohi, Amin, A., Naderi, N., Parsaee, M., Taghavi, S., Ghadrdoost, B., Jafari, S., Khoshavi, M., Rapezzi, C., Biagini, E., Corsini, A., Gagliardi, C., Graziosi, M., Longhi, S., Milandri, A., Ragni, L., Palmieri, S., Olivotto, I., Arretini, A., Castelli, G., Cecchi, F., Fornaro, A., Tomberli, B., Spirito, P., Devoto, E., Bella, P Della, Maccabelli, G., Sala, S., Guarracini, F., Peretto, G., Russo, M G, Calabro, R., Pacileo, G., Limongelli, G., Masarone, D., Pazzanese, V., Rea, A., Rubino, M., Tramonte, S., Valente, F., Caiazza, M., Cirillo, A., Del Giorno, G., Esposito, A., Gravino, R., Marrazzo, T., Trimarco, B., Losi, M-A, Di Nardo, C., Giamundo, A., Musella, F., Pacelli, F., Scatteia, A., Canciello, G., Caforio, A., Iliceto, S., Calore, C., Leoni, L., Marra, M Perazzolo, Rigato, I., Tarantini, G., Schiavo, A., Testolina, M., Arbustini, E., Di Toro, A., Giuliani, L P, Serio, A., Fedele, F., Frustaci, A., Alfarano, M., Chimenti, C., Drago, F., Baban, A., Calò, L., Lanzillo, C., Martino, A., Uguccioni, M., Zachara, E., Halasz, G., Re, F., Sinagra, G., Carriere, C., Merlo, M., Ramani, F., Kavoliūnienė, Aušra, Krivickienė, Aušra, Tamulevičiūtė-Prascienė, Eglė, Vieželis, Mindaugas, Balkevičienė, Laura, Laukytė, M., Palevičiūtė, Eglė, Pinto, Y., Wilde, A., Asselbergs, F W, Sammani, A., Van Der Heijden, J., Van Laake, L., De Jonge, N., Hassink, R., Kirkels, J H, Ajuluchukwu, J., Olusegun-Joseph, A., Ekure, E., Mizia-Stec, K., Tendera, M., Czekaj, A., Sikora-Puz, A., Skoczynska, A., Wybraniec, M., Rubis, P., Dziewiecka, E., Wisniowska-Smialek, S., Bilinska, Z., Chmielewski, P., Foss-Nieradko, B., Michalak, E., Stepien-Wojno, M., Mazek, B., Lopes, L Rocha, Almeida, A R, Cruz, I., Gomes, A C, Pereira, A R, Brito, D., Madeira, H., Francisco, A R, Menezes, M., Moldovan, O., Guimaraes, T Oliveira, Silva, D., Ginghina, C., Jurcut, R., Mursa, A., Popescu, B A, Apetrei, E., Militaru, S., Coman, I Mircea, Frigy, A., Fogarasi, Z., Kocsis, I., Szabo, I A, Fehervari, L., Nikitin, I., Resnik, E., Komissarova, M., Lazarev, V., Shebzukhova, M., Ustyuzhanin, D., Blagova, O., Alieva, I., Kulikova, V., Lutokhina, Y., Pavlenko, E., Varionchik, N., Ristic, A D, Seferovic, P M, Veljic, I., Zivkovic, I., Milinkovic, I., Pavlovic, A., Radovanovic, G., Simeunovic, D., Zdravkovic, M., Aleksic, M., Djokic, J., Hinic, S., Klasnja, S., Mircetic, K., Monserrat, L., Fernandez, X., Garcia-Giustiniani, D., Larrañaga, J M, Ortiz-Genga, M., Barriales-Villa, R., Martinez-Veira, C., Veira, E., Cequier, A., Salazar-Mendiguchia, J., Manito, N., Gonzalez, J., Fernández-Avilés, F., Medrano, C., Yotti, R., Cuenca, S., Espinosa, M A, Mendez, I., Zatarain, E., Alvarez, R., Pavia, P Garcia, Briceno, A., Cobo-Marcos, M., Dominguez, F., Galvan, E De Teresa, Pinilla, J M García, Abdeselam-Mohamed, N., Lopez-Garrido, M A, Hidalgo, L Morcillo, Ortega-Jimenez, M V, Mezcua, A Robles, Guijarro-Contreras, A., Gomez-Garcia, D., Robles-Mezcua, M., Blanes, J R Gimeno, Castro, F J, Esparza, C Munoz, Molina, M Sabater, García, M Sorli, Cuenca, D Lopez, de Mallorca, Palma, Ripoll-Vera, T., Alvarez, J., Nunez, J., Gomez, Y., Fernandez, P L Sanchez, Villacorta, E., Avila, C., Bravo, L., Diaz-Pelaez, E., Gallego-Delgado, M., Garcia-Cuenllas, L., Plata, B., Lopez-Haldon, J E, Pena Pena, M L, Perez, E M Cantero, Zorio, E., Arnau, M A, Sanz, J., Marques-Sule, E., Repositório da Universidade de Lisboa, Lopes, Lr, Losi, Ma, Sheikh, N, Laroche, C, Charron, P, Gimeno, J, Kaski, Jp, Maggioni, Ap, Tavazzi, L, Arbustini, E, Brito, D, Celutkiene, J, Hagege, A, Linhart, A, Mogensen, J, Garcia-Pinilla, Jm, Ripoll-Vera, T, Seggewiss, H, Villacorta, E, Caforio, A, and Elliott, Pm
Subjects: Genotype, Health Policy, Diabetes, Cardiovascular risk factors, Hypertension, Hypertrophic cardiomyopathy, Obesity, Cardiomyopathy, Hypertrophic, Ventricular Dysfunction, Left, diabete, Cardiovascular Diseases, Risk Factors, Heart Disease Risk Factors, cardiovascular risk factor, Humans, Female, 03.02. Klinikai orvostan, Cardiology and Cardiovascular Medicine, Cardiomyopathies, obesity
Abstract: © The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited., Aims: The interaction between common cardiovascular risk factors (CVRF) and hypertrophic cardiomyopathy (HCM) is poorly studied. We sought to explore the relation between CVRF and the clinical characteristics of patients with HCM enrolled in the EURObservational Research Programme (EORP) Cardiomyopathy registry. Methods and results: 1739 patients with HCM were studied. The relation between hypertension (HT), diabetes (DM), body mass index (BMI) and clinical traits was analyzed. Analyses were stratified according to the presence or absence of a pathogenic variant in a sarcomere gene.The prevalence of HT, DM and obesity (Ob) was 37%, 10%, and 21%, respectively. HT, DM and Ob were associated with older age (p
Published: 2022

18. Old and new equations for maximal heart rate prediction in patients with heart failure and reduced ejection fraction on beta-blockers treatment: results from the MECKI score data set

Author: Magri D., Piepoli M., Gallo G., Corra U., Metra M., Paolillo S., Filardi P. P., Maruotti A., Salvioni E., Mapelli M., Vignati C., Senni M., Limongelli G., Lagioia R., Scrutinio D., Emdin M., Passino C., Parati G., Sinagra G., Correale M., Badagliacca R., Sciomer S., Di Lenarda A., Agostoni P., Apostolo A., Palermo P., Contini M., Farina S., De Martino F., Mantegazza V., Bonomi A., Mattavelli I., Rocca M. D., Pezzuto B., Bandera F., Rovai S., Giordano A., Ricci R., Ferraironi A., Arcari L., Lombardi C., Carubelli V., Matassini M., Shkoza M., Malfatto G., Caravita S., Pacileo G., Cicoira M., Passantino A., Raimondo R., Confalonieri M., Zaffalon D., Carriere C., Ferraretti A., Bussotti M., Marchese G., Iorio A., Pastormerlo L., Gargiulo P., Halasz G., Capelli B., Villani G. Q., Oliva F., Santolamazza C., Re F., La Franca E., Herberg R., Magri, D., Piepoli, M., Gallo, G., Corra, U., Metra, M., Paolillo, S., Filardi, P. P., Maruotti, A., Salvioni, E., Mapelli, M., Vignati, C., Senni, M., Limongelli, G., Lagioia, R., Scrutinio, D., Emdin, M., Passino, C., Parati, G., Sinagra, G., Correale, M., Badagliacca, R., Sciomer, S., Di Lenarda, A., Agostoni, P., Apostolo, A., Palermo, P., Contini, M., Farina, S., De Martino, F., Mantegazza, V., Bonomi, A., Mattavelli, I., Rocca, M. D., Pezzuto, B., Bandera, F., Rovai, S., Giordano, A., Ricci, R., Ferraironi, A., Arcari, L., Lombardi, C., Carubelli, V., Matassini, M., Shkoza, M., Malfatto, G., Caravita, S., Pacileo, G., Cicoira, M., Passantino, A., Raimondo, R., Confalonieri, M., Zaffalon, D., Carriere, C., Ferraretti, A., Bussotti, M., Marchese, G., Iorio, A., Pastormerlo, L., Gargiulo, P., Halasz, G., Capelli, B., Villani, G. Q., Oliva, F., Santolamazza, C., Re, F., La Franca, E., and Herberg, R.
Subjects: MECKI score, cardiopulmonary exercise test, chronotropic incompetence, heart failure, maximal heart rate, kidney, Epidemiology, exercise test, Chronotropic incompetence, ventricular dysfunction, Heart failure, test, mecki score, adrenergic beta-antagonists, heart rate, humans, stroke volume, left, Ventricular Dysfunction, Left, Cardiology and Cardiovascular Medicine, Maximal heart rate, Cardiopulmonary exercise test
Abstract: Aims Predicting maximal heart rate (MHR) in heart failure with reduced ejection fraction (HFrEF) still remains a major concern. In such a context, the Keteyian equation is the only one derived in a HFrEF cohort on optimized β-blockers treatment. Therefore, using the Metabolic Exercise combined with Cardiac and Kidney Indexes (MECKI) data set, we looked for a possible MHR equation, for an external validation of Keteyien formula and, contextually, for accuracy of the historical MHR formulas and their relationship with the HR measured at the anaerobic threshold (AT). Methods and results Data from 3487 HFrEF outpatients on optimized β-blockers treatment from the MECKI data set were analyzed. Besides excluding all possible confounders, the new equation was derived by using HR data coming from maximal cardiopulmonary exercise test. The simplified derived equation was [109–(0.5*age) + (0.5*HR rest) + (0.2*LVEF)–(5 if haemoglobin Conclusion The derived equation to estimate the MHR in HFrEF patients, by accounting also for the systolic dysfunction degree and anaemia, improved slightly the Keteyian formula. Both formulas might be helpful in identifying the true maximal effort during an exercise test and the intensity domain during a rehabilitation programme.
Published: 2022

19. Unexplained sudden cardiac arrest in children: clinical and genetic characteristics of survivors

Author: Monda, E, Sarubbi, B, Russo, M, Caiazza, M, Mazzaccara, C, Magrelli, J, Rubino, M, Esposito, A, Perna, A, Passariello, A, Bossone, E, Romeo, E, Colonna, D, Esposito, M, D'Argenio, V, Salvatore, F, Pacileo, G, Crotti, L, Frisso, G, Limongelli, G, Russo, MG, Esposito, MV, Limongelli, G., Monda, E, Sarubbi, B, Russo, M, Caiazza, M, Mazzaccara, C, Magrelli, J, Rubino, M, Esposito, A, Perna, A, Passariello, A, Bossone, E, Romeo, E, Colonna, D, Esposito, M, D'Argenio, V, Salvatore, F, Pacileo, G, Crotti, L, Frisso, G, Limongelli, G, Russo, MG, Esposito, MV, and Limongelli, G.
Published: 2021

20. Comorbidities in chronic heart failure: An update from Italian Society of Cardiology (SIC) Working Group on Heart Failure

Author: Correale, M, Paolillo, S, Mercurio, V, Limongelli, G, Barilla, F, Ruocco, G, Palazzuoli, A, Scrutinio, D, Lagioia, R, Lombardi, C, Lupi, L, Magri, D, Masarone, D, Pacileo, G, Scicchitano, P, Matteo Ciccone, M, Parati, G, Tocchetti, C, Nodari, S, Correale M., Paolillo S., Mercurio V., Limongelli G., Barilla F., Ruocco G., Palazzuoli A., Scrutinio D., Lagioia R., Lombardi C., Lupi L., Magri D., Masarone D., Pacileo G., Scicchitano P., Matteo Ciccone M., Parati G., Tocchetti C. G., Nodari S., Correale, M, Paolillo, S, Mercurio, V, Limongelli, G, Barilla, F, Ruocco, G, Palazzuoli, A, Scrutinio, D, Lagioia, R, Lombardi, C, Lupi, L, Magri, D, Masarone, D, Pacileo, G, Scicchitano, P, Matteo Ciccone, M, Parati, G, Tocchetti, C, Nodari, S, Correale M., Paolillo S., Mercurio V., Limongelli G., Barilla F., Ruocco G., Palazzuoli A., Scrutinio D., Lagioia R., Lombardi C., Lupi L., Magri D., Masarone D., Pacileo G., Scicchitano P., Matteo Ciccone M., Parati G., Tocchetti C. G., and Nodari S.
Abstract: The increasing number of patients with heart failure HF and comorbidities is due to aging population and increase of life expectancy of patients with cardiovascular disease. Encouraging results derived by recent trials may suggest some comorbidities as new targets for new drugs, highlighting the need for a better understanding of the comorbidities’ effects in HF patients and the need of a multidisciplinary approach for the management of chronic HF with comorbidities. We report a brief review about main cardiovascular and non-cardiovascular comorbidities in HF patients in order to update physicians and researchers engaged in the HF research or in “fight against heart failure.”
Published: 2020

21. Cardiovascular Death Risk in Recovered Mid-Range Ejection Fraction Heart Failure: Insights From Cardiopulmonary Exercise Test

Author: Magrì, D, Piepoli, M, Corrà, U, Gallo, G, Maruotti, A, Vignati, C, Salvioni, E, Mapelli, M, Paolillo, S, Perrone Filardi, P, Girola, D, Metra, M, Scardovi, A, Lagioia, R, Limongelli, G, Senni, M, Scrutinio, D, Emdin, M, Passino, C, Lombardi, C, Cattadori, G, Parati, G, Cicoira, M, Correale, M, Frigerio, M, Clemenza, F, Bussotti, M, Guazzi, M, Badagliacca, R, Sciomer, S, DI Lenarda, A, Maggioni, A, Sinagra, G, Volpe, M, Agostoni, P, MagrÌ D, Piepoli M, CorrÀ U, Gallo G, Maruotti A, Vignati C, Salvioni E, Mapelli M, Paolillo S, Perrone Filardi P, Girola D, Metra M, Scardovi AB, Lagioia R, Limongelli G, Senni M, Scrutinio D, Emdin M, Passino C, Lombardi C, Cattadori G, Parati G, Cicoira M, Correale M, Frigerio M, Clemenza F, Bussotti M, Guazzi M, Badagliacca R, Sciomer S, DI Lenarda A, Maggioni A, Sinagra G, Volpe M, Agostoni P, Magrì, D, Piepoli, M, Corrà, U, Gallo, G, Maruotti, A, Vignati, C, Salvioni, E, Mapelli, M, Paolillo, S, Perrone Filardi, P, Girola, D, Metra, M, Scardovi, A, Lagioia, R, Limongelli, G, Senni, M, Scrutinio, D, Emdin, M, Passino, C, Lombardi, C, Cattadori, G, Parati, G, Cicoira, M, Correale, M, Frigerio, M, Clemenza, F, Bussotti, M, Guazzi, M, Badagliacca, R, Sciomer, S, DI Lenarda, A, Maggioni, A, Sinagra, G, Volpe, M, Agostoni, P, MagrÌ D, Piepoli M, CorrÀ U, Gallo G, Maruotti A, Vignati C, Salvioni E, Mapelli M, Paolillo S, Perrone Filardi P, Girola D, Metra M, Scardovi AB, Lagioia R, Limongelli G, Senni M, Scrutinio D, Emdin M, Passino C, Lombardi C, Cattadori G, Parati G, Cicoira M, Correale M, Frigerio M, Clemenza F, Bussotti M, Guazzi M, Badagliacca R, Sciomer S, DI Lenarda A, Maggioni A, Sinagra G, Volpe M, and Agostoni P
Abstract: Background: Heart failure with midrange ejection fraction (HFmrEF) represents a heterogeneous category where phenotype, as well as prognostic assessment, remains debated. The present study explores a specific HFmrEF subset, namely those who recovered from a reduced EF (rec-HFmrEF) and, particularly, it focuses on the possible additive prognostic role of cardiopulmonary exercise testing. Methods and Results: We analyzed data from 4535 patients with HFrEF and 1176 patients with rec-HFmrEF from the Metabolic Exercise combined with Cardiac and Kidney Indexes database. The end point was cardiovascular death at 5 years. The median follow-up was 1343 days (25th–75th range 627–2403 days). Cardiovascular death occurred in 552 HFrEF and 61 rec-HFmrEF patients. The multivariate analysis confirmed an independent role of the MECKI score's variables in HFrEF (C-index = 0.744) whereas, in the rec-HFmrEF group, only age and peak oxygen uptake (pVO2) remained associated to the end point (C-index = 0.745). A peak oxygen uptake of ≤55% of predicted and a ventilatory efficiency of ≥31 resulted as the most accurate cut-off values in the outcome prediction. Conclusions: Present data support the cardiopulmonary exercise test and, particularly, the peak oxygen uptake, as a useful tool in the rec-HFmrEF prognostic assessment. A peak VO2 of ≤55% predicted and ventilatory efficiency of ≥31 might help to identify a high-risk rec-HFmrEF subgroup.
Published: 2020

22. Progressive right ventricular dysfunction and exercise impairment in patients with heart failure and diabetes mellitus: insights from the T.O.S.CA. Registry

Author: Salzano, A, D'Assante, R, Iacoviello, M, Triggiani, V, Rengo, G, Cacciatore, F, Maiello, C, Limongelli, G, Masarone, D, Sciacqua, A, Perrone Filardi, P, Mancini, A, Volterrani, M, Vriz, O, Castello, R, Passantino, A, Campo, M, Modesti, Pa, De Giorgi, A, Arcopinto, M, Gargiulo, P, Perticone, M, Colao, A, Milano, S, Garavaglia, A, Napoli, R, Suzuki, T, Bossone, E, Marra, Am, Cittadini, A, Saccà, L, Monti, Mg, Matarazzo, M, Stagnaro, Fm, Piccioli, L, Lombardi, A, Panicara, V, Flora, M, Golia, L, Faga, V, Ruocco, A, Della Polla, D, Franco, R, Schiavo, A, Gigante, A, Spina, E, Sicuranza, M, Monaco, F, Apicella, M, Miele, C, Campanino, Ag, Mazza, L, Abete, R, Farro, A, Luciano, F, Polizzi, R, Ferrillo, G, De Luca, M, Crisci, G, Giardino, F, Barbato, M, Ranieri, B, Ferrara, F, Russo, V, Malinconico, M, Citro, R, Guastalamacchia, E, Leone, M, Giagulli, Va, Amarelli, C, Mattucci, I, Calabrò, P, Calabrò, R, D'Andrea, A, Maddaloni, V, Pacileo, G, Scarafile, R, Belfiore, A, Cimellaro, A, Casaretti, L, Paolillo, S, Favuzzi, Amr, Di Segni, C, Bruno, C, Vergani, E, Massaro, R, Grimaldi, F, Frigo, A, Sorrentino, Mr, Malandrino, D, Manfredini, R, Fabbian, F, Puzzo, A, Ragusa, L, Caliendo, L, Carbone, L, Frigiola, A, Generali, T, Giacomazzi, F, De Vincentiis, C, Ballotta, A, Garofalo, P, Malizia, G, Misiano, G, Israr, Mz, Bernieh, D, Cassambai, S, Yazaki, Y, Heaney, Lm, Eagle, Ka, Ventura, Ho, Bruzzese, D, Salzano, Andrea, D'Assante, Roberta, Iacoviello, Massimo, Triggiani, Vincenzo, Rengo, Giuseppe, Cacciatore, Francesco, Maiello, Ciro, Limongelli, Giuseppe, Masarone, Daniele, Sciacqua, Angela, Filardi, Pasquale Perrone, Mancini, Antonio, Volterrani, Maurizio, Vriz, Olga, Castello, Roberto, Passantino, Andrea, Campo, Michela, Modesti, Pietro A, De Giorgi, Alfredo, Arcopinto, Michele, Gargiulo, Paola, Perticone, Maria, Colao, Annamaria, Milano, Salvatore, Garavaglia, Agnese, Napoli, Raffaele, Suzuki, Toru, Bossone, Eduardo, Marra, Alberto M, Cittadini, Antonio, and Misiano, Gabriella
Subjects: Registrie, Heart Failure, Endocrinology, Diabetes and Metabolism, Ventricular Dysfunction, Right, Diabetes, Insulins, Socio-culturale, Stroke Volume, Insulin resistance, Diabete, Cardiopulmonary exercise test, Chronic heart failure, Diabetes, Insulin resistance, Right ventricle, TOSCA Registry, Chronic heart failure, Diabetes Mellitus, Type 2, TOSCA Registry, Exercise Test, Ventricular Function, Right, Humans, Insulin, Right ventricle, Registries, Cardiology and Cardiovascular Medicine, Cardiopulmonary exercise test, TOSCA, Human, LS4_7
Abstract: Background Findings from the T.O.S.CA. Registry recently reported that patients with concomitant chronic heart failure (CHF) and impairment of insulin axis (either insulin resistance—IR or diabetes mellitus—T2D) display increased morbidity and mortality. However, little information is available on the relative impact of IR and T2D on cardiac structure and function, cardiopulmonary performance, and their longitudinal changes in CHF. Methods Patients enrolled in the T.O.S.CA. Registry performed echocardiography and cardiopulmonary exercise test at baseline and at a patient-average follow-up of 36 months. Patients were divided into three groups based on the degree of insulin impairment: euglycemic without IR (EU), euglycemic with IR (IR), and T2D. Results Compared with EU and IR, T2D was associated with increased filling pressures (E/e′ratio: 15.9 ± 8.9, 12.0 ± 6.5, and 14.5 ± 8.1 respectively, p 2) in TD2 vs EU and IR patients was recorded (respectively, 15.8 ± 3.8 ml/Kg/min, 18.4 ± 4.3 ml/Kg/min and 16.5 ± 4.3 ml/Kg/min, p 2 in the T2D group (+ 13% increase in RV dimension, − 21% decline in TAPSE/PAPS ratio and − 20% decrease in peak VO2). Conclusion The higher risk of death and CV hospitalizations exhibited by HF-T2D patients in the T.O.S.CA. Registry is associated with progressive RV ventricular dysfunction and exercise impairment when compared to euglycemic CHF patients, supporting the pivotal importance of hyperglycaemia and right chambers in HF prognosis. Trial registration ClinicalTrials.gov identifier: NCT023358017
Published: 2022

23. Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy.

Author: Norrish, G, Cleary, A, Field, E, Cervi, E, Boleti, O, Ziółkowska, L, Olivotto, I, Khraiche, D, Limongelli, G, Anastasakis, A, Weintraub, R, Biagini, E, Ragni, L, Prendiville, T, Duignan, S, McLeod, K, Ilina, M, Fernandez, A, Marrone, C, Bökenkamp, R, Baban, A, Kubus, P, Daubeney, PEF, Sarquella-Brugada, G, Cesar, S, Klaassen, S, Ojala, TH, Bhole, V, Medrano, C, Uzun, O, Brown, E, Gran, F, Sinagra, G, Castro, FJ, Stuart, G, Yamazawa, H, Barriales-Villa, R, Garcia-Guereta, L, Adwani, S, Linter, K, Bharucha, T, Gonzales-Lopez, E, Siles, A, Rasmussen, TB, Calcagnino, M, Jones, CB, De Wilde, H, Kubo, T, Felice, T, Popoiu, A, Mogensen, J, Mathur, S, Centeno, F, Reinhardt, Z, Schouvey, S, Elliott, PM, Kaski, JP, Norrish, G, Cleary, A, Field, E, Cervi, E, Boleti, O, Ziółkowska, L, Olivotto, I, Khraiche, D, Limongelli, G, Anastasakis, A, Weintraub, R, Biagini, E, Ragni, L, Prendiville, T, Duignan, S, McLeod, K, Ilina, M, Fernandez, A, Marrone, C, Bökenkamp, R, Baban, A, Kubus, P, Daubeney, PEF, Sarquella-Brugada, G, Cesar, S, Klaassen, S, Ojala, TH, Bhole, V, Medrano, C, Uzun, O, Brown, E, Gran, F, Sinagra, G, Castro, FJ, Stuart, G, Yamazawa, H, Barriales-Villa, R, Garcia-Guereta, L, Adwani, S, Linter, K, Bharucha, T, Gonzales-Lopez, E, Siles, A, Rasmussen, TB, Calcagnino, M, Jones, CB, De Wilde, H, Kubo, T, Felice, T, Popoiu, A, Mogensen, J, Mathur, S, Centeno, F, Reinhardt, Z, Schouvey, S, Elliott, PM, and Kaski, JP
Abstract: BACKGROUND: Up to one-half of childhood sarcomeric hypertrophic cardiomyopathy (HCM) presents before the age of 12 years, but this patient group has not been systematically characterized. OBJECTIVES: The aim of this study was to describe the clinical presentation and natural history of patients presenting with nonsyndromic HCM before the age of 12 years. METHODS: Data from the International Paediatric Hypertrophic Cardiomyopathy Consortium on 639 children diagnosed with HCM younger than 12 years were collected and compared with those from 568 children diagnosed between 12 and 16 years. RESULTS: At baseline, 339 patients (53.6%) had family histories of HCM, 132 (20.9%) had heart failure symptoms, and 250 (39.2%) were prescribed cardiac medications. The median maximal left ventricular wall thickness z-score was 8.7 (IQR: 5.3-14.4), and 145 patients (27.2%) had left ventricular outflow tract obstruction. Over a median follow-up period of 5.6 years (IQR: 2.3-10.0 years), 42 patients (6.6%) died, 21 (3.3%) underwent cardiac transplantation, and 69 (10.8%) had life-threatening arrhythmic events. Compared with those presenting after 12 years, a higher proportion of younger patients underwent myectomy (10.5% vs 7.2%; P = 0.045), but fewer received primary prevention implantable cardioverter-defibrillators (18.9% vs 30.1%; P = 0.041). The incidence of mortality or life-threatening arrhythmic events did not differ, but events occurred at a younger age. CONCLUSIONS: Early-onset childhood HCM is associated with a comparable symptom burden and cardiac phenotype as in patients presenting later in childhood. Long-term outcomes including mortality did not differ by age of presentation, but patients presenting at younger than 12 years experienced adverse events at younger ages.
Published: 2022

24. Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy.

Author: Norrish, G, Ding, T, Field, E, Cervi, E, Ziółkowska, L, Olivotto, I, Khraiche, D, Limongelli, G, Anastasakis, A, Weintraub, R, Biagini, E, Ragni, L, Prendiville, T, Duignan, S, McLeod, K, Ilina, M, Fernández, A, Marrone, C, Bökenkamp, R, Baban, A, Kubus, P, Daubeney, PEF, Sarquella-Brugada, G, Cesar, S, Klaassen, S, Ojala, TH, Bhole, V, Medrano, C, Uzun, O, Brown, E, Gran, F, Sinagra, G, Castro, FJ, Stuart, G, Vignati, G, Yamazawa, H, Barriales-Villa, R, Garcia-Guereta, L, Adwani, S, Linter, K, Bharucha, T, Garcia-Pavia, P, Siles, A, Rasmussen, TB, Calcagnino, M, Jones, CB, De Wilde, H, Kubo, T, Felice, T, Popoiu, A, Mogensen, J, Mathur, S, Centeno, F, Reinhardt, Z, Schouvey, S, O'Mahony, C, Omar, RZ, Elliott, PM, Kaski, JP, Norrish, G, Ding, T, Field, E, Cervi, E, Ziółkowska, L, Olivotto, I, Khraiche, D, Limongelli, G, Anastasakis, A, Weintraub, R, Biagini, E, Ragni, L, Prendiville, T, Duignan, S, McLeod, K, Ilina, M, Fernández, A, Marrone, C, Bökenkamp, R, Baban, A, Kubus, P, Daubeney, PEF, Sarquella-Brugada, G, Cesar, S, Klaassen, S, Ojala, TH, Bhole, V, Medrano, C, Uzun, O, Brown, E, Gran, F, Sinagra, G, Castro, FJ, Stuart, G, Vignati, G, Yamazawa, H, Barriales-Villa, R, Garcia-Guereta, L, Adwani, S, Linter, K, Bharucha, T, Garcia-Pavia, P, Siles, A, Rasmussen, TB, Calcagnino, M, Jones, CB, De Wilde, H, Kubo, T, Felice, T, Popoiu, A, Mogensen, J, Mathur, S, Centeno, F, Reinhardt, Z, Schouvey, S, O'Mahony, C, Omar, RZ, Elliott, PM, and Kaski, JP
Abstract: BACKGROUND: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. METHODS: The study cohort comprised 1075 children (mean age, 10.2 years [±4.4]) diagnosed with HCM (1-16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). RESULTS: MLVWT Z score was <10 in 598 (58.1%), ≥10 to <20 in 334 (31.1%), and ≥20 in 143 (13.3%). Higher MLVWT Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score ≥20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3-9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores <10, ≥10 to <20, and ≥20 was 95.6%, 87.4%, and 86.0, respectively. The estimated SCD risk at 5 years had a nonlinear, inverted U-shaped relationship with MLVWT Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. CONCLUSIONS: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter def
Published: 2022

25. 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

26. 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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27. Pick Your Threshold: A Comparison Among Different Methods of Anaerobic Threshold Evaluation in Heart Failure Prognostic Assessment

Author: Salvioni, E., Mapelli, M., Bonomi, A., Magri, D., Piepoli, M., Frigerio, M., Paolillo, S., Corra, U., Raimondo, R., Lagioia, R., Badagliacca, R., Filardi, P. P., Senni, M., Correale, M., Cicoira, M., Perna, E., Metra, M., Guazzi, M., Limongelli, G., Sinagra, G., Parati, G., Cattadori, G., Bandera, F., Bussotti, M., Re, F., Vignati, C., Lombardi, C., Scardovi, A. B., Sciomer, S., Passantino, A., Emdin, M., Passino, C., Santolamazza, C., Girola, D., Zaffalon, D., De Martino, F., Agostoni, P., Farina, S., Pezzuto, B., Apostolo, A., Palermo, P., Contini, M., Gugliandolo, P., Mattavelli, I., Della Rocca, M., Gallo, G., Moscucci, F., Iorio, A., Halasz, G., Capelli, B., Binno, S., Pacileo, G., Valente, F., Vastarella, R., Carriere, C., Mase, M., Cittar, M., Di Lenarda, A., Caravita, S., Vigano, E., Marchese, G., Ricci, R., Arcari, L., Scrutinio, D., Battaia, E., Moretti, M., Matassini, M. V., Shkoza, M., Herberg, R., Cittadini, A., Salzano, A., Marra, A., Lafranca, E., and Vitale, G.
Subjects: heart failure, prognosis, anaerobic threshold, cardiopulmonary exercise test
Published: 2022

28. Feasibility of semi-recumbent bicycle exercise Doppler echocardiography for the evaluation of the right heart and pulmonary circulation unit in different clinical conditions: the RIGHT heart international NETwork (RIGHT-NET)

Author: Ferrara F., Gargani L., Naeije R., Rudski L., Armstrong W. F., Wierzbowska-Drabik K., Argiento P., Bandera F., Cademartiri F., Citro R., Cittadini A., Cocchia R., Contaldi C., D'Alto M., D'Andrea A., Grunig E., Guazzi M., Kolias T. J., Limongelli G., Marra A. M., Mauro C., Moreo A., Ranieri B., Saggar R., Salzano A., Stanziola A. A., Vriz O., Vannan M., Kasprzak J. D., Bossone E., Capuano F., Benvenga R., Bellino M., Radano I., Marra A., D'Assante R., Rega S., Mazzola M., Raciti M., Dellegrottaglie S., De Luca N., Rozza F., Russo V., Di Salvo G., Ghio S., Guida S., Eichstaedt C. A., Labate V., La Gerche A., Pacileo G., Verrengia M., Kovacs G., Douschan P., Casadei F., De Chiara B., Ostenfeld E., Pedrizzetti G., Pieri F., Mori F., Moggi-Pignone A., Pratali L., Pugliese N., Selton-Suty C., Huttin O., Venner C., Serra W., Tafuni F., Stanziola A., Martino M., Caccavo G., Szabo I., Varga A., Agoston G., Voilliot D., Mobasseri S., Flueckiger P., Liu S., Ferrara, F., Gargani, L., Naeije, R., Rudski, L., Armstrong, W. F., Wierzbowska-Drabik, K., Argiento, P., Bandera, F., Cademartiri, F., Citro, R., Cittadini, A., Cocchia, R., Contaldi, C., D'Alto, M., D'Andrea, A., Grunig, E., Guazzi, M., Kolias, T. J., Limongelli, G., Marra, A. M., Mauro, C., Moreo, A., Ranieri, B., Saggar, R., Salzano, A., Stanziola, A. A., Vriz, O., Vannan, M., Kasprzak, J. D., Bossone, E., Capuano, F., Benvenga, R., Bellino, M., Radano, I., Marra, A., D'Assante, R., Rega, S., Mazzola, M., Raciti, M., Dellegrottaglie, S., De Luca, N., Rozza, F., Russo, V., Di Salvo, G., Ghio, S., Guida, S., Eichstaedt, C. A., Labate, V., La Gerche, A., Pacileo, G., Verrengia, M., Kovacs, G., Douschan, P., Casadei, F., De Chiara, B., Ostenfeld, E., Pedrizzetti, G., Pieri, F., Mori, F., Moggi-Pignone, A., Pratali, L., Pugliese, N., Selton-Suty, C., Huttin, O., Venner, C., Serra, W., Tafuni, F., Stanziola, A., Martino, M., Caccavo, G., Szabo, I., Varga, A., Agoston, G., Voilliot, D., Mobasseri, S., Flueckiger, P., and Liu, S.
Subjects: Adult, medicine.medical_specialty, Pulmonary Circulation, Adolescent, Ventricular Dysfunction, Right, Atrial Pressure, Population, Right heart, Predictive Value of Test, Regurgitation (circulation), 030204 cardiovascular system & hematology, Doppler echocardiography, Pulmonary hypertension, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Predictive Value of Tests, Internal medicine, medicine.artery, medicine, Ventricular outflow tract, Humans, Radiology, Nuclear Medicine and imaging, education, Cardiac imaging, Exercise Doppler echocardiography, Aged, Aged, 80 and over, education.field_of_study, medicine.diagnostic_test, business.industry, Middle Aged, medicine.disease, Echocardiography, Doppler, Bicycling, Feasibility Studie, 030228 respiratory system, Pulmonary artery, Cardiology, cardiovascular system, Ventricular Function, Right, Right ventricle, Feasibility Studies, Female, Cardiology and Cardiovascular Medicine, business, Human
Abstract: Exercise Doppler echocardiography (EDE) is a well-validated tool in ischemic and valvular heart diseases. However, its use in the assessment of the right heart and pulmonary circulation unit (RH-PCU) is limited. The aim of this study is to assess the semi-recumbent bicycle EDE feasibility for the evaluation of RH-PCU in a large multi-center population, from healthy individuals and elite athletes to patients with overt or at risk of developing pulmonary hypertension (PH). From January 2019 to July 2019, 954 subjects [mean age 54.2 ± 16.4years, range 16–96, 430 women] underwent standardized semi-recumbent bicycle EDE with an incremental workload of 25 watts every 2min, were prospectively enrolled among 7 centers participating to the RIGHT Heart International NETwork (RIGHT-NET). EDE parameters of right heart structure, function and pressures were obtained according to current recommendations. Right ventricular (RV) function at peak exercise was feasible in 903/940 (96%) by tricuspid annular plane systolic excursion (TAPSE), 667/751 (89%) by tissue Doppler-derived tricuspid lateral annular systolic velocity (S′) and 445/672 (66.2%) by right ventricular fractional area change (RVFAC). RV—right atrial pressure gradient [RV–RA gradient = 4 × tricuspid regurgitation velocity2 (TRV)] was feasible in 894/954 patients (93.7%) at rest and in 816/954 (85.5%) at peak exercise. The feasibility rate in estimating pulmonary artery pressure improved to more than 95%, if both TRV and/or right ventricular outflow tract acceleration time (RVOT AcT) were considered. In high specialized echocardiography laboratories semi-recumbent bicycle EDE is a feasible tool for the assessment of the RH-PCU pressure and function.
Published: 2021

29. Multiple hormonal and metabolic deficiency syndrome predicts outcome in heart failure: the T.O.S.CA. Registry

Author: Cittadini A., Salzano A., Iacoviello M., Triggiani V., Rengo G., Cacciatore F., Maiello C., Limongelli G., Masarone D., Perticone F., Cimellaro A., Filardi P. P., Paolillo S., Mancini A., Volterrani M., Vriz O., Castello R., Passantino A., Campo M., Modesti P. A., de Giorgi A., Monte I. P., Puzzo A., Ballotta A., D'Assante R., Arcopinto M., Gargiulo P., Sciacqua A., Bruzzese D., Colao A., Napoli R., Suzuki T., Eagle K. A., Ventura H. O., Marra A. M., Bossone E., Sacca L., Monti M. G., Matarazzo M., Stagnaro F. M., Piccioli L., Lombardi A., Panicara V., Flora M., Golia L., Faga V., Ruocco A., della Polla D., Franco R., Schiavo A., Gigante A., Spina E., Sicuranza M., Monaco F., Apicella M., Miele C., Campanino A. G., Mazza L., Abete R., Farro A., Luciano F., Polizzi R., Ferrillo G., de Luca M., Crisci G., Giardino F., Barbato M., Ranieri B., Ferrara F., Russo V., Malinconico M., Citro R., Guastalamacchia E., Leone M., Giagulli V. A., Amarelli C., Mattucci I., Calabro P., Calabro R., D'Andrea A., Maddaloni V., Pacileo G., Scarafile R., Belfiore A., Casaretti L., Favuzzi A. M. R., Di Segni C., Bruno C., Vergani E., Massaro R., Grimaldi F., Frigo A., Campo M. R., Sorrentino M. R., Malandrino D., Manfredini R., Fabbian F., Ragusa L., Caliendo L., Carbone L., Frigiola A., Generali T., Giacomazzi F., de Vincentiis C., Garofalo P., Malizia G., Milano S., Misiano G., Israr M. Z., Bernieh D., Cassambai S., Yazaki Y., Heaney L. M., Cittadini, Antonio, Salzano, Andrea, Iacoviello, Massimo, Triggiani, Vincenzo, Rengo, Giuseppe, Cacciatore, Francesco, Maiello, Ciro, Limongelli, Giuseppe, Masarone, Daniele, Perticone, Francesco, Cimellaro, Antonio, Perrone Filardi, Pasquale, Paolillo, Stefania, Mancini, Antonio, Volterrani, Maurizio, Vriz, Olga, Castello, Roberto, Passantino, Andrea, Campo, Michela, Modesti, Pietro A, De Giorgi, Alfredo, Monte, Ines P, Puzzo, Alfonso, Ballotta, Andrea, D'Assante, Roberta, Arcopinto, Michele, Gargiulo, Paola, Sciacqua, Angela, Bruzzese, Dario, Colao, Annamaria, Napoli, Raffaele, Suzuki, Toru, Eagle, Kim A, Ventura, Hector O, Marra, Alberto M, Bossone, Eduardo, Cittadini, A., Salzano, A., Iacoviello, M., Triggiani, V., Rengo, G., Cacciatore, F., Maiello, C., Limongelli, G., Masarone, D., Perticone, F., Cimellaro, A., Filardi, P. P., Paolillo, S., Mancini, A., Volterrani, M., Vriz, O., Castello, R., Passantino, A., Campo, M., Modesti, P. A., de Giorgi, A., Monte, I. P., Puzzo, A., Ballotta, A., D'Assante, R., Arcopinto, M., Gargiulo, P., Sciacqua, A., Bruzzese, D., Colao, A., Napoli, R., Suzuki, T., Eagle, K. A., Ventura, H. O., Marra, A. M., Bossone, E., Sacca, L., Monti, M. G., Matarazzo, M., Stagnaro, F. M., Piccioli, L., Lombardi, A., Panicara, V., Flora, M., Golia, L., Faga, V., Ruocco, A., della Polla, D., Franco, R., Schiavo, A., Gigante, A., Spina, E., Sicuranza, M., Monaco, F., Apicella, M., Miele, C., Campanino, A. G., Mazza, L., Abete, R., Farro, A., Luciano, F., Polizzi, R., Ferrillo, G., de Luca, M., Crisci, G., Giardino, F., Barbato, M., Ranieri, B., Ferrara, F., Russo, V., Malinconico, M., Citro, R., Guastalamacchia, E., Leone, M., Giagulli, V. A., Amarelli, C., Mattucci, I., Calabro, P., Calabro, R., D'Andrea, A., Maddaloni, V., Pacileo, G., Scarafile, R., Belfiore, A., Casaretti, L., Favuzzi, A. M. R., Di Segni, C., Bruno, C., Vergani, E., Massaro, R., Grimaldi, F., Frigo, A., Campo, M. R., Sorrentino, M. R., Malandrino, D., Manfredini, R., Fabbian, F., Ragusa, L., Caliendo, L., Carbone, L., Frigiola, A., Generali, T., Giacomazzi, F., de Vincentiis, C., Garofalo, P., Malizia, G., Milano, S., Misiano, G., Israr, M. Z., Bernieh, D., Cassambai, S., Yazaki, Y., and Heaney, L. M.
Subjects: medicine.medical_specialty, Multiple hormonal and metabolic deficiency syndrome, Epidemiology, Prognosi, Anabolic deficiency, Socio-culturale, Heart failure, 030204 cardiovascular system & hematology, Ventricular Function, Left, 03 medical and health sciences, 0302 clinical medicine, Insulin resistance, Diabetes mellitus, Internal medicine, Multiple hormonal, medicine, Clinical endpoint, Humans, 030212 general & internal medicine, Prospective Studies, Registries, TOSCA, LS4_7, Ejection fraction, business.industry, Hazard ratio, Metabolic deficiency syndrome, Heart failure • Anabolic deficiency • Multiple hormonal and metabolic deficiency syndrome • Hormones • Prognosis • TOSCA, Stroke Volume, medicine.disease, Prognosis, Hormone, Confidence interval, Heart failure, Anabolic deficiency, Multiple hormonal and metabolic deficiency syndrome, Hormones, Prognosis, TOSCA, Hormones, Hospitalization, Observational study, Cardiology and Cardiovascular Medicine, business
Abstract: Aims Recent evidence supports the occurrence of multiple hormonal and metabolic deficiency syndrome (MHDS) in chronic heart failure (CHF). However, no large observational study has unequivocally demonstrated its impact on CHF progression and outcome. The T.O.S.CA. (Trattamento Ormonale nello Scompenso CArdiaco; Hormone Treatment in Heart Failure) Registry has been specifically designed to test the hypothesis that MHDS affects morbidity and mortality in CHF patients. Methods and Results The T.O.S.CA. Registry is a prospective, multicentre, observational study involving 19 Italian centres. Thyroid hormones, insulin-like growth factor-1, total testosterone, dehydropianoandrosterone sulfate, insulin resistance, and the presence of diabetes were evaluated. A MHDS was defined as the presence of ≥2 hormone deficiencies (HDs). Primary endpoint was a composite of all-cause mortality and cardiovascular hospitalizations. Four hundred and eighty heart failure patients with ejection fraction ≤45% were enrolled. MHDS or diabetes was diagnosed in 372 patients (77.5%). A total of 271 events (97 deaths and 174 cardiovascular hospitalizations) were recorded, 41% in NO-MHDS and 62% in MHDS (P Conclusion MHDS is common in CHF and independently associated with increased all-cause mortality and cardiovascular hospitalization, representing a promising therapeutic target. Trial registration ClinicalTrials.gov identifier: NCT023358017
Published: 2021

30. Current use of cardiac magnetic resonance in tertiary referral centres for the diagnosis of cardiomyopathy: the ESC EORP Cardiomyopathy/Myocarditis Registry

Author: Mizia-Stec K, Charron P, Gimeno Blanes JR, Elliott P, Kaski JP, Maggioni AP, Tavazzi L, Tendera M, Felix SB, Dominguez F, Ojrzynska N, Losi MA, Limongelli G, Barriales-Villa R, Seferovic PM, Biagini E, Wybraniec M, Laroche C, Caforio ALP, Mizia-Stec, K, Charron, P, Gimeno Blanes, Jr, Elliott, P, Kaski, Jp, Maggioni, Ap, Tavazzi, L, Tendera, M, Felix, Sb, Dominguez, F, Ojrzynska, N, Losi, Ma, Limongelli, G, Barriales-Villa, R, Seferovic, Pm, Biagini, E, Wybraniec, M, Laroche, C, and Caforio, Alp
Subjects: transthoracic echocardiography, registry, cardiomyopathy, cardiac magnetic resonance
Abstract: ims: Cardiac magnetic resonance (CMR) is recommended in the diagnosis of cardiomyopathies, but it is time-consuming, expensive, and limited in availability in some European regions. The aim of this study was to determine the use of CMR in cardiomyopathy patients enrolled into the European Society of Cardiology (ESC) cardiomyopathy registry [part of the EURObservational Research Programme (EORP)]. Methods and results: Three thousand, two hundred, and eight consecutive adult patients (34.6% female; median age: 53.0 ± 15 years) with cardiomyopathy were studied: 1260 with dilated (DCM), 1739 with hypertrophic (HCM), 66 with restrictive (RCM), and 143 with arrhythmogenic right ventricular cardiomyopathy (ARVC). CMR scans were performed at baseline in only 29.4% of patients. CMR utilization was variable according to cardiomyopathy subtypes: from 51.1% in ARVC to 36.4% in RCM, 33.8% in HCM, and 20.6% in DCM (P < 0.001). CMR use in tertiary referral centres located in different European countries varied from 1% to 63.2%. Patients undergoing CMR were younger, less symptomatic, less frequently had implantable cardioverter-defibrillator (ICD)/pacemaker implanted, had fewer cardiovascular risk factors and comorbidities (P < 0.001). In 28.6% of patients, CMR was used along with transthoracic echocardiography (TTE); 67.6% patients underwent TTE alone, and 0.9% only CMR. Conclusion: Less than one-third of patients enrolled in the registry underwent CMR and the use varied greatly between cardiomyopathy subtypes, clinical profiles of patients, and European tertiary referral centres. This gap with current guidelines needs to be considered carefully by scientific societies to promote wider availability and use of CMR in patients with cardiomyopathies.
Published: 2021

31. Cardiovascular Involvement in mtDNA Disease: Diagnosis, Management, and Therapeutic Options

Author: Lioncino M., Monda E., Caiazza M., Fusco A., Cirillo A., Dongiglio F., Simonelli V., Sampaolo S., Ruggiero L., Scarano G., Pota V., Frisso G., Mazzaccara C., D'Amati G., Nigro G., Russo M. G., Wahbi K., Limongelli G., Lioncino, M., Monda, E., Caiazza, M., Fusco, A., Cirillo, A., Dongiglio, F., Simonelli, V., Sampaolo, S., Ruggiero, L., Scarano, G., Pota, V., Frisso, G., Mazzaccara, C., D'Amati, G., Nigro, G., Russo, M. G., Wahbi, K., and Limongelli, G.
Subjects: Cardiomyopathy, Dilated, Mitochondrial Diseases, mtDNA, hypertrophic, DNA, hypertrophic cardiomyopathy, MELAS syndrome, mitochondrial diseases, DNA, mitochondrial, humans, cardiomyopathies, cardiomyopathy, dilated, cardiomyopathy, hypertrophic, Cardiomyopathy, Hypertrophic, mitochondrial, DNA, Mitochondrial, Mitochondrial disease, Hypertrophic cardiomyopathy, Humans, Cardiomyopathies, cardiomyopathy, dilated, Human, Cardiomyopathie
Abstract: Mitochondrial diseases (MD) include an heterogenous group of systemic disorders caused by sporadic or inherited mutations in nuclear or mitochondrial DNA (mtDNA), causing impairment of oxidative phosphorylation system. Hypertrophic cardiomyopathy is the dominant pattern of cardiomyopathy in all forms of mtDNA disease, being observed in almost 40% of the patients. Dilated cardiomyopathy, left ventricular noncompaction, and conduction system disturbances have been also reported. In this article, the authors discuss the current clinical knowledge on MD, focusing on diagnosis and management of mitochondrial diseases caused by mtDNA mutations.
Published: 2021

32. Diagnostic and prognostic implications of myocardial work in cardiac amyloidosis and in genetic and non-genetic cardiomyopathies with hypertrophic phenotype

Author: Palmiero, G, primary, Rubino, M, additional, Monda, E, additional, Lioncino, M, additional, Verrillo, F, additional, Vetrano, E, additional, Caiazza, M, additional, Fusco, A, additional, Cirillo, A, additional, Dongiglio, F, additional, Ascione, L, additional, Caso, P, additional, and Limongelli, G, additional
Published: 2022
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33. Pathophysiological, haemodynamic and prognostic implications of left atrial dysfunction in cardiac amyloidosis and other cardiomyopathies with hypertrophic phenotype

Author: Palmiero, G, primary, Rubino, M, additional, Lioncino, M, additional, Monda, E, additional, Vetrano, E, additional, Verrillo, F, additional, Dongiglio, F, additional, Fusco, A, additional, Cirillo, A, additional, Caiazza, M, additional, Ascione, L, additional, Caso, P, additional, and Limongelli, G, additional
Published: 2022
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34. Diagnostic and prognostic implications of right ventricular-arterial coupling in cardiac amyloidosis and in genetic and non-genetic cardiomyopathies with hypertrophic phenotype

Author: Palmiero, G, primary, Rubino, M, additional, Monda, E, additional, Lioncino, M, additional, Verrillo, F, additional, Dongiglio, F, additional, Caiazza, M, additional, Vetrano, E, additional, Cirillo, A, additional, Fusco, A, additional, Ascione, L, additional, Caso, P, additional, and Limongelli, G, additional
Published: 2022
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35. new insights into cardiac functional status after MitraClip: the role of cardiopulmonary exercise test: P973

Author: Salerno, G, Di Maio, M, Bigazzi, Cappelli M, Bianchi, R, Palladino, M T, Calabro, P, Valente, F, Maiello, C, Limongelli, G, and Pacileo, G
Published: 2016

36. The metabolic exercise cardiac and kidney indexes (mecki) score: a new prognostic tool in patients with heart failure. a multicenter comparison with seattle and hfss in a large cohort of patients: 679

Author: Mapelli, Massimo M, La Gioia, R L, Emdin, M E, Raimondo, R R, Limongelli, G L, Scardovi, A S, Lombardi, C L, Sinagra, G S, Corra, U C, and Agostoni, P A
Published: 2016

37. Exercise oscillatory ventilation and prognosis in heart failure patients with reduced and mid-range ejection fraction

Author: Rovai, S, Corra, U, Piepoli, M, Vignati, C, Salvioni, E, Bonomi, A, Mattavelli, I, Arcari, L, Scardovi, A, Perrone Filardi, P, Lagioia, R, Paolillo, S, Magri, D, Limongelli, G, Metra, M, Senni, M, Scrutinio, D, Raimondo, R, Emdin, M, Lombardi, C, Cattadori, G, Parati, G, Re, F, Cicoira, M, Villani, G, Mina, C, Correale, M, Frigerio, M, Perna, E, Mapelli, M, Magini, A, Clemenza, F, Bussotti, M, Battaia, E, Guazzi, M, Bandera, F, Badagliacca, R, Di Lenarda, A, Pacileo, G, Maggioni, A, Passino, C, Sciomer, S, Sinagra, G, Agostoni, P, Rovai S., Corra U., Piepoli M., Vignati C., Salvioni E., Bonomi A., Mattavelli I., Arcari L., Scardovi A. B., Perrone Filardi P., Lagioia R., Paolillo S., Magri D., Limongelli G., Metra M., Senni M., Scrutinio D., Raimondo R., Emdin M., Lombardi C., Cattadori G., Parati G., Re F., Cicoira M., Villani G. Q., Mina C., Correale M., Frigerio M., Perna E., Mapelli M., Magini A., Clemenza F., Bussotti M., Battaia E., Guazzi M., Bandera F., Badagliacca R., Di Lenarda A., Pacileo G., Maggioni A., Passino C., Sciomer S., Sinagra G., Agostoni P., Rovai, S, Corra, U, Piepoli, M, Vignati, C, Salvioni, E, Bonomi, A, Mattavelli, I, Arcari, L, Scardovi, A, Perrone Filardi, P, Lagioia, R, Paolillo, S, Magri, D, Limongelli, G, Metra, M, Senni, M, Scrutinio, D, Raimondo, R, Emdin, M, Lombardi, C, Cattadori, G, Parati, G, Re, F, Cicoira, M, Villani, G, Mina, C, Correale, M, Frigerio, M, Perna, E, Mapelli, M, Magini, A, Clemenza, F, Bussotti, M, Battaia, E, Guazzi, M, Bandera, F, Badagliacca, R, Di Lenarda, A, Pacileo, G, Maggioni, A, Passino, C, Sciomer, S, Sinagra, G, Agostoni, P, Rovai S., Corra U., Piepoli M., Vignati C., Salvioni E., Bonomi A., Mattavelli I., Arcari L., Scardovi A. B., Perrone Filardi P., Lagioia R., Paolillo S., Magri D., Limongelli G., Metra M., Senni M., Scrutinio D., Raimondo R., Emdin M., Lombardi C., Cattadori G., Parati G., Re F., Cicoira M., Villani G. Q., Mina C., Correale M., Frigerio M., Perna E., Mapelli M., Magini A., Clemenza F., Bussotti M., Battaia E., Guazzi M., Bandera F., Badagliacca R., Di Lenarda A., Pacileo G., Maggioni A., Passino C., Sciomer S., Sinagra G., and Agostoni P.
Abstract: Aims: Exercise oscillatory ventilation (EOV) is a pivotal cardiopulmonary exercise test parameter for the prognostic evaluation of patients with chronic heart failure (HF). It has been described in patients with HF with reduced ejection fraction (<40%, HFrEF) and with HF with preserved ejection fraction (>50%, HFpEF), but no data are available for patients with HF with mid-range ejection fraction (40–49%, HFmrEF). The aim of the study was to evaluate the prognostic role of EOV in HFmrEF patients. Methods and results: We analysed 1239 patients with HFmrEF and 4482 patients with HFrEF, enrolled in the MECKI score database, with a 2-year follow-up. The study endpoint was the composite of cardiovascular death, urgent heart transplant, and ventricular assist device implantation. We identified EOV in 968 cases (16% and 17% of cases in HFmrEF and HFrEF,. respectively). HFrEF EOV+ patients were significantly older, and their parameters suggested a more severe HF than HFrEF EOV− patients. A similar behaviour was found in HFmrEF EOV+ vs. EOV− patients. Kaplan–Meier analysis, irrespective of ejection fraction, showed that EOV is associated with a worse survival, and that patients with HFrEF and HFmrEF EOV+ had a significantly worse outcome than the EOV− of the same ejection fraction groups. EOV-associated survival differences in HFmrEF patients started after 18 months of follow-up. Conclusion: Exercise oscillatory ventilation has a similar prevalence and ominous prognostic value in both HFmrEF and HFrEF patients, indicating a group of patients in need of a more intensive follow-up and a more aggressive therapy. In HFmrEF, the survival curves between EOV+ and EOV− patients diverged only after 18 months.
Published: 2019

38. Heart failure prognosis over time: how the prognostic role of oxygen consumption and ventilatory efficiency during exercise has changed in the last 20 years

Author: Paolillo, S., Veglia, F., Salvioni, E., Corra, U., Piepoli, M., Lagioia, R., Limongelli, G., Sinagra, G., Cattadori, G., Scardovi, A. B., Metra, M., Senni, M., Bonomi, A., Scrutinio, D., Raimondo, R., Emdin, M., Magri, D., Parati, G., Re, F., Cicoira, M., Mina, C., Correale, M., Frigerio, M., Bussotti, M., Battaia, E., Guazzi, M., Badagliacca, R., Di Lenarda, A., Maggioni, A., Passino, C., Sciomer, S., Pacileo, G., Mapelli, M., Vignati, C., Clemenza, F., Binno, S., Lombardi, C., Filardi, P. P., Agostoni, P., Apostolo, A., Palermo, P., Contini, M., Farina, S., Mantegazza, V., Spadafora, E., Lattarulo, M. S., Giordano, A., Mezzani, A., Ricci, R., Ferraironi, A., Carubelli, V., Pietrucci, F., Malfatto, G., Caravita, Sergio, Vigano, E., Valente, F., Vastarella, R., Gravino, R., Roselli, T., Buono, A., De Maria, R., Passantino, A., Santoro, D., Campanale, S., Caputo, D., Bertipaglia, D., Confalonieri, M., Gentile, P., Zambon, E., Morosin, M., Carriere, C., Ferraretti, A., Marchese, G., Iorio, A., Pastormerlo, L., Gargiulo, P., Villani, G. Q., Oliva, F., Perna, E., Paolillo, Stefania, Veglia, Fabrizio, Salvioni, Elisabetta, Corrà, Ugo, Piepoli, Massimo, Lagioia, Rocco, Limongelli, Giuseppe, Sinagra, Gianfranco, Cattadori, Gaia, Scardovi, Angela B., Metra, Marco, Senni, Michele, Bonomi, Alice, Scrutinio, Domenico, Raimondo, Rosa, Emdin, Michele, Magrì, Damiano, Parati, Gianfranco, Re, Federica, Cicoira, Mariantonietta, Minà, Chiara, Correale, Michele, Frigerio, Maria, Bussotti, Maurizio, Battaia, Elisa, Guazzi, Marco, Badagliacca, Roberto, Di Lenarda, Andrea, Maggioni, Aldo, Passino, Claudio, Sciomer, Susanna, Pacileo, Giuseppe, Mapelli, Massimo, Vignati, Carlo, Clemenza, Francesco, Binno, Simone, Lombardi, Carlo, Filardi, Pasquale Perrone, Agostoni, Piergiuseppe, Apostolo, Anna, Palermo, Pietro, Contini, Mauro, Farina, Stefania, Mantegazza, Valentina, Spadafora, Emanuele, Lattarulo, Maria Silvia, Giordano, Andrea, Mezzani, Alessandro, Ricci, Roberto, Ferraironi, Alessandro, Carubelli, Valentina, Pietrucci, Francesca, Malfatto, Gabriella, Caravita, Sergio, Viganò, Elena, Valente, Fabio, Vastarella, Rossella, Gravino, Rita, Roselli, Teo, Buono, Andrea, De Maria, Renata, Passantino, Andrea, Santoro, Daniela, Campanale, Saba, Caputo, Domenica, Bertipaglia, Donatella, Confalonieri, Marco, Gentile, Piero, Zambon, Elena, Morosin, Marco, Carriere, Cosimo, Ferraretti, Armando, Marchese, Giovanni, Iorio, Annamaria, Pastormerlo, Luigi, Gargiulo, Paola, Villani, Giovanni Quinto, Oliva, Fabrizio, Perna, Enrico, Paolillo, S, Veglia, F, Salvioni, E, Corrà, U, Piepoli, M, Lagioia, R, Limongelli, G, Sinagra, G, Cattadori, G, Scardovi, A, Metra, M, Senni, M, Bonomi, A, Scrutinio, D, Raimondo, R, Emdin, M, Magrì, D, Parati, G, Re, F, Cicoira, M, Minà, C, Correale, M, Frigerio, M, Bussotti, M, Battaia, E, Guazzi, M, Badagliacca, R, Di Lenarda, A, Maggioni, A, Passino, C, Sciomer, S, Pacileo, G, Mapelli, M, Vignati, C, Clemenza, F, Binno, S, Lombardi, C, Perrone Filardi, P, Agostoni, P, Apostolo, A, Palermo, P, Contini, M, Farina, S, Mantegazza, V, Spadafora, E, Lattarulo, M, Giordano, A, Mezzani, A, Ricci, R, Ferraironi, A, Carubelli, V, Pietrucci, F, Malfatto, G, Caravita, S, Vigano', E, Valente, F, Vastarella, R, Gravino, R, Roselli, T, Buono, A, De Maria, R, Passantino, A, Santoro, D, Campanale, S, Caputo, D, Bertipaglia, D, Confalonieri, M, Gentile, P, Zambon, E, Morosin, M, Carriere, C, Ferraretti, A, Marchese, G, Iorio, A, Pastormerlo, L, Gargiulo, P, Villani, G, Oliva, F, Perna, E, Paolillo, S., Veglia, F., Salvioni, E., Corra, U., Piepoli, M., Lagioia, R., Limongelli, G., Sinagra, G., Cattadori, G., Scardovi, A. B., Metra, M., Senni, M., Bonomi, A., Scrutinio, D., Raimondo, R., Emdin, M., Magri, D., Parati, G., Re, F., Cicoira, M., Mina, C., Correale, M., Frigerio, M., Bussotti, M., Battaia, E., Guazzi, M., Badagliacca, R., Di Lenarda, A., Maggioni, A., Passino, C., Sciomer, S., Pacileo, G., Mapelli, M., Vignati, C., Clemenza, F., Binno, S., Lombardi, C., Filardi, P. P., Agostoni, P., Apostolo, A., Palermo, P., Contini, M., Farina, S., Mantegazza, V., Spadafora, E., Lattarulo, M. S., Giordano, A., Mezzani, A., Ricci, R., Ferraironi, A., Carubelli, V., Pietrucci, F., Malfatto, G., Caravita, S., Vigano, E., Valente, F., Vastarella, R., Gravino, R., Roselli, T., Buono, A., De Maria, R., Passantino, A., Santoro, D., Campanale, S., Caputo, D., Bertipaglia, D., Confalonieri, M., Gentile, P., Zambon, E., Morosin, M., Carriere, C., Ferraretti, A., Marchese, G., Iorio, A., Pastormerlo, L., Gargiulo, P., Villani, G. Q., Oliva, F., and Perna, E.
Subjects: Male, Prognosi, Cardiopulmonary exercise test, Heart failure, Peak oxygen uptake, Prognosis, VE/VCO2 slope, cardiopulmonary exercise test, heart failure, peak oxygen uptake, prognosis, ve/vco, 2, slope, disease progression, exercise test, female, follow-up studies, humans, male, middle aged, oxygen consumption, pulmonary ventilation, roc curve, respiratory function tests, retrospective studies, forecasting, Settore MED/11 - Malattie dell'Apparato Cardiovascolare, Follow-Up Studie, VE/VCO, Oxygen Consumption, Cardiology and Cardiovascular Medicine, Retrospective Studie, Humans, Respiratory Function Test, Retrospective Studies, VE/VCO 2 slope, Middle Aged, Respiratory Function Tests, ROC Curve, Disease Progression, Exercise Test, Female, Pulmonary Ventilation, Human, Follow-Up Studies, Forecasting
Abstract: Aims: Exercise-derived parameters, specifically peak exercise oxygen uptake (peak VO 2 ) and minute ventilation/carbon dioxide relationship slope (VE/VCO 2 slope), have a pivotal prognostic value in heart failure (HF). It is unknown how the prognostic threshold of peak VO 2 and VE/VCO 2 slope has changed over the last 20 years in parallel with HF prognosis improvement. Methods and results: Data from 6083 HF patients (81% male, age 61 ± 13 years), enrolled in the MECKI score database between 1993 and 2015, were retrospectively analysed. By enrolment year, four groups were generated: group 1 1993–2000 (n = 440), group 2 2001–2005 (n = 1288), group 3 2006–2010 (n = 2368), and group 4 2011–2015 (n = 1987). We compared the 10-year survival of groups and analysed how the overall risk (cardiovascular death, urgent heart transplantation, or left ventricular assist device implantation) changed over time according to peak VO 2 and VE/VCO 2 slope and to major clinical and therapeutic variables. At 10 years, a progressively higher survival from group 1 to group 3 was observed, with no further improvement afterwards. A 20% risk for peak VO 2 15 mL/min/kg (95% confidence interval 16–13), 9 (11–8), 4 (4–2) and 5 (7–4) was observed in group 1, 2, 3, and 4, respectively, while the VE/VCO 2 slope value for a 20% risk was 32 (37–29), 47 (51–43), 59 (64–55), and 57 (63–52), respectively. Conclusions: Heart failure prognosis improved over time up to 2010 in a HF population followed by experienced centres. The peak VO 2 and VE/VCO 2 slope cut-offs identifying a definite risk progressively decreased and increased over time, respectively. The prognostic threshold of peak VO 2 and VE/VCO 2 slope must be updated whenever HF prognosis improves.
Published: 2019

39. Gender and age normalization and ventilation efficiency during exercise in heart failure with reduced ejection fraction

Author: Salvioni, E, Corrà, U, Piepoli, M, Rovai, S, Correale, M, Paolillo, S, Pasquali, M, Magrì, Damiano, Vitale, G, Fusini, L, Mapelli, M, Vignati, C, Lagioia, R, Raimondo, R, Sinagra, G, Boggio, F, Cangiano, L, Gallo, G, Magini, A, Contini, M, Palermo, P, Apostolo, A, Pezzuto, B, Bonomi, A, Scardovi, Ab, Filardi, Pp, Limongelli, G, Metra, M, Scrutinio, D, Emdin, M, Piccioli, L, Lombardi, C, Cattadori, G, Parati, G, Caravita, S, Re, F, Cicoira, M, Frigerio, M, Clemenza, F, Bussotti, M, Battaia, E, Guazzi, M, Bandera, F, Badagliacca, R, Di Lenarda, A, Pacileo, G, Passino, C, Sciomer, S, Ambrosio, G, Agostoni, P, MECKI score research, Group., Salvioni, E., Corra, U., Piepoli, M., Rovai, S., Correale, M., Paolillo, S., Pasquali, M., Magri, D., Vitale, G., Fusini, L., Mapelli, M., Vignati, C., Lagioia, R., Raimondo, R., Sinagra, G., Boggio, F., Cangiano, L., Gallo, G., Magini, A., Contini, M., Palermo, P., Apostolo, A., Pezzuto, B., Bonomi, A., Scardovi, A. B., Filardi, P. P., Limongelli, G., Metra, M., Scrutinio, D., Emdin, M., Piccioli, L., Lombardi, C., Cattadori, G., Parati, G., Caravita, S., Re, F., Cicoira, M., Frigerio, M., Clemenza, F., Bussotti, M., Battaia, E., Guazzi, M., Bandera, F., Badagliacca, R., Di Lenarda, A., Pacileo, G., Passino, C., Sciomer, S., Ambrosio, G., Agostoni, P., and Guarino, Raimondo
Subjects: Male, lcsh:Diseases of the circulatory (Cardiovascular) system, cardiopulmonary exercise test, heart failure, prognosis, ventilation efficiency, medicine.medical_treatment, Settore MED/11 - Malattie dell'Apparato Cardiovascolare, 030204 cardiovascular system & hematology, Ventricular Function, Left, 0302 clinical medicine, Original Research Articles, Original Research Article, 030212 general & internal medicine, Lung, Aged, 80 and over, education.field_of_study, Ejection fraction, Age Factors, Middle Aged, Net reclassification improvement, Italy, Cardiology, Female, Cardiology and Cardiovascular Medicine, Cardiopulmonary exercise test, Prognosis, Ventilation efficiency, Heart failure, Adult, medicine.medical_specialty, Adolescent, Prognosi, Population, Age and gender, Young Adult, 03 medical and health sciences, Oxygen Consumption, Sex Factors, Internal medicine, Linear regression, medicine, Humans, education, Exercise, Aged, Retrospective Studies, Cardiovascular mortality, business.industry, Stroke Volume, medicine.disease, lcsh:RC666-701, Ventricular assist device, Exercise Test, Morbidity, business, human activities, Follow-Up Studies, Forecasting
Abstract: Aims Ventilation vs. carbon dioxide production (VE/VCO2) is among the strongest cardiopulmonary exercise testing prognostic parameters in heart failure (HF). It is usually reported as an absolute value. The current definition of normal VE/VCO2 slope values is inadequate, since it was built from small groups of subjects with a particularly limited number of women and elderly. We aimed to define VE/VCO2 slope prediction formulas in a sizable population and to test whether the prognostic power of VE/VCO2 slope in HF was different if expressed as a percentage of the predicted value or as an absolute value. Methods and results We calculated the linear regressions between age and VE/VCO2 slope in 1136 healthy subjects (68% male, age 44.9 ± 14.5, range 13–83 years). We then applied age‐adjusted and sex‐adjusted formulas to predict VE/VCO2 slope to HF patients included in the metabolic exercise test data combined with cardiac and kidney indexes score database, which counts 6112 patients (82% male, age 61.4 ± 12.8, left ventricular ejection fraction 33.2 ± 10.5%, peakVO2 14.8 ± 4.9, mL/min/kg, VE/VCO2 slope 32.7 ± 7.7) from 24 HF centres. Finally, we evaluated whether the use of absolute values vs. percentages of predicted VE/VCO2 affected HF prognosis prediction (composite of cardiovascular mortality + urgent transplant or left ventricular assist device). We did so in the entire cardiac and kidney indexes score population and separately in HF patients with severe (peakVO2 < 14 mL/min/kg, n = 2919, 61.1 events/1000 pts/year) or moderate (peakVO2 ≥ 14 mL/min/kg, n = 3183, 19.9 events/1000 pts/year) HF. In the healthy population, we obtained the following equations: female, VE/VCO2 = 0.052 × Age + 23.808 (r = 0.192); male, VE/VCO2 = 0.095 × Age + 20.227 (r = 0.371) (P = 0.007). We applied these formulas to calculate the percentages of predicted VE/VCO2 values. The 2‐year survival prognostic power of VE/VCO2 slope was strong, and it was similar if expressed as absolute value or as a percentage of predicted value (AUCs 0.686 and 0.690, respectively). In contrast, in severe HF patients, AUCs significantly differed between absolute values (0.637) and percentages of predicted values (0.650, P = 0.0026). Moreover, VE/VCO2 slope expressed as a percentage of predicted value allowed to reclassify 6.6% of peakVO2 < 14 mL/min/kg patients (net reclassification improvement = 0.066, P = 0.0015). Conclusions The percentage of predicted VE/VCO2 slope value strengthens the prognostic power of VE/VCO2 in severe HF patients, and it should be preferred over the absolute value for HF prognostication. Furthermore, the widespread use of VE/VCO2 slope expressed as percentage of predicted value can improve our ability to identify HF patients at high risk, which is a goal of utmost clinical relevance.
Published: 2020

40. Myocardial performance is impaired in cardiac amyloidosis: role of myocardial work-derived parameter in differential diagnosis with phenocopies and prognostic implications

Author: Palmiero, G, primary, Rubino, M, additional, Monda, E, additional, Caiazza, M, additional, Di Fraia, F, additional, Lioncino, M, additional, Vetrano, E, additional, Dongiglio, F, additional, Cerciello, G, additional, Manganelli, F, additional, Ascione, L, additional, Caso, P, additional, and Limongelli, G, additional
Published: 2021
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41. Natural history of left ventricular hypertrophy in infants of diabetic mothers

Author: Monda, E, primary, Verrillo, F, additional, Altobelli, I, additional, Lioncino, M, additional, Caiazza, M, additional, Rubino, M, additional, Cirillo, A, additional, Fusco, A, additional, Di Fraia, F, additional, Pacileo, R, additional, Gragnano, F, additional, Passariello, A, additional, Calabro', P, additional, Russo, M G, additional, and Limongelli, G, additional
Published: 2021
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42. Left atrial function is impaired in cardiac amyloidosis and other cardiomyopathies with hypertrophic phenotype: haemodynamic correlations, pathophysiological consequences and prognostic implications

Author: Palmiero, G, primary, Rubino, M, additional, Monda, E, additional, Caiazza, M, additional, Vetrano, E, additional, Lioncino, M, additional, Di Fraia, F, additional, Dongiglio, F, additional, Cerciello, G, additional, Manganelli, F, additional, Ascione, L, additional, Caso, P, additional, and Limongelli, G, additional
Published: 2021
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43. The role of right ventricular-arterial coupling in cardiac amyloidosis: a comparison between subtypes and with other genetic and non-genetic hypertrophic cardiomyopathies and prognostic consequences

Author: Palmiero, G, primary, Monda, E, additional, Rubino, M, additional, Caiazza, M, additional, Vetrano, E, additional, Di Fraia, F, additional, Lioncino, M, additional, Dongiglio, F, additional, Carciello, G, additional, Manganelli, F, additional, Ascione, L, additional, Caso, P, additional, and Limongelli, G, additional
Published: 2021
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44. Insights from cardiopulmonary exercise testing in pediatric patients with hypertrophic cardiomyopathy

Author: Gallo, G, primary, Mastromarino, V, additional, Limongelli, G, additional, Calcagni, G, additional, Ragno, L, additional, Valente, F, additional, Musumeci, M B, additional, Adorisio, R, additional, Rubino, M, additional, Autore, C, additional, and Magri', D, additional
Published: 2021
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45. Effect of migalastat on cardiac involvement in Fabry disease: preliminary results from MAIORA study

Author: Camporeale, A, primary, Bandera, F, additional, Pieroni, M, additional, Pieruzzi, F, additional, Bersano, A, additional, Econimo, L, additional, Lanzillo, C, additional, Limongelli, G, additional, Mignani, R, additional, Motta, I, additional, Olivotto, I, additional, Milani, V, additional, Guazzi, M, additional, and Lombardi, M, additional
Published: 2021
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46. Corrigendum to “Long-term prognostic role of diabetes mellitus and glycemic control in heart failure patients with reduced ejection fraction: Insights from the MECKI Score database” [Int J Cardiol. 2020 Oct 15; 317: 103–110. PMID: 32360652]

Author: Paolillo, S., primary, Salvioni, E., additional, Perrone Filardi, P., additional, Bonomi, A., additional, Sinagra, G., additional, Gentile, P., additional, Gargiulo, P., additional, Scoccia, A., additional, Cosentino, N., additional, Gugliandolo, P., additional, Badagliacca, R., additional, Lagioia, R., additional, Correale, M., additional, Frigerio, M., additional, Perna, E., additional, Piepoli, M., additional, Re, F., additional, Raimondo, R., additional, Minà, C., additional, Clemenza, F., additional, Bussotti, M., additional, Limongelli, G., additional, Gravino, R., additional, Passantino, A., additional, Magrì, D., additional, Parati, G., additional, Caravita, S., additional, Scardovi, A.B., additional, Arcari, L., additional, Vignati, C., additional, Mapelli, M., additional, Cattadori, G., additional, Cavaliere, C., additional, Corrà, U., additional, and Agostoni, P., additional
Published: 2021
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47. Potential resistance to SARS-CoV-2 infection in lysosomal storage disorders

Author: Pieroni, M., Pieruzzi, F., Mignani, R., Graziani, Francesca, Olivotto, I., Riccio, E., Ciabatti, M., Limongelli, G., Manna, Raffaele, Bolognese, L., Pisani, A., Graziani F. (ORCID:0000-0002-4520-5689), Manna R. (ORCID:0000-0003-1560-3907), Pieroni, M., Pieruzzi, F., Mignani, R., Graziani, Francesca, Olivotto, I., Riccio, E., Ciabatti, M., Limongelli, G., Manna, Raffaele, Bolognese, L., Pisani, A., Graziani F. (ORCID:0000-0002-4520-5689), and Manna R. (ORCID:0000-0003-1560-3907)
Abstract: On 11 March 2020, the World Health Organization declared the coronavirus disease 2019 (COVID-19) outbreak to be a ‘pan- demic’. In the following months, the disease spread in Italy to include >2 million infected patients (3.7% of the population), with >80 000 COVID-19-related deaths. While vaccines have been developed, based on the current knowledge of biology of coronavirus infection, COVID-19 is empirically treated with antivirals, immunomodulatory and antimalarial drugs, but most of these approaches do not specifically target the cellular mechanisms involved in viral entry and spreading. It has been demonstrated that the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infects human and some animal cell lines through the Angiotensin-converting enzyme 2 (ACE2) receptor, but the endocytic pathway represents an alter- native entry route for SARS-CoV-2 and other several viruses, including Ebola virus and African swine fever virus. The endo- somal protein, Niemann–Pick C1, deficiency in which causes the lysosomal disorder Niemann–Pick disease type C (NPC) , has been identified as a necessary entry receptor for Ebola virus [1, 2]. Similarly, it is well established that SARS-CoV-2 infection requires an acidic endosomal environment [3]. Of note, endo- some luminal pH and further vesicle maturation are controlled by ion channels, pumps and membrane proteins, suggesting that drugs targeting these structures may potentially have broad-spectrum antiviral properties. Indeed, recent studies have described the inhibitory effect of experimental and Food and Drug Administration-approved drugs acting on the endoso- mal compartment against viruses of high relevance for human and animal health, including SARS-CoV-2 and Ebola virus (Figure 1A) [4]. In Fabry disease, the genetically determined deficit of alpha- galactosidase A leads to glycosphingolipids accumulation in kidney, heart, vessels and lungs. Glycosphingolipid storage causes impairment of several lysosomal
Published: 2021

48. Potential resistance to SARS-CoV-2 infection in lysosomal storage disorders

Author: Pieroni, M, Pieruzzi, F, Mignani, R, Graziani, F, Olivotto, I, Riccio, E, Ciabatti, M, Limongelli, G, Manna, R, Bolognese, L, Pisani, A, Pieroni, Maurizio, Pieruzzi, Federico, Mignani, Renzo, Graziani, Francesca, Olivotto, Iacopo, Riccio, Eleonora, Ciabatti, Michele, Limongelli, Giuseppe, Manna, Raffaele, Bolognese, Leonardo, Pisani, Antonio, Pieroni, M, Pieruzzi, F, Mignani, R, Graziani, F, Olivotto, I, Riccio, E, Ciabatti, M, Limongelli, G, Manna, R, Bolognese, L, Pisani, A, Pieroni, Maurizio, Pieruzzi, Federico, Mignani, Renzo, Graziani, Francesca, Olivotto, Iacopo, Riccio, Eleonora, Ciabatti, Michele, Limongelli, Giuseppe, Manna, Raffaele, Bolognese, Leonardo, and Pisani, Antonio
Published: 2021

49. Masked hypertension in young patients after successful aortic coarctation repair: impact on left ventricular geometry and function

Author: Di Salvo, G, Castaldi, B, Baldini, L, Gala, S, del Gaizo, F, D'Andrea, A, Limongelli, G, D'Aiello, A F, Scognamiglio, G, Sarubbi, B, Pacileo, G, Russo, M G, and Calabrò, R
Published: 2011
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50. Improving coeliac disease risk prediction by testing non-HLA variants additional to HLA variants

Author: Romanos, Jihane, Rosén, Anna, Kumar, Vinod, Trynka, Gosia, Franke, Lude, Szperl, Agata, Gutierrez-Achury, Javier, van Diemen, Cleo C, Kanninga, Roan, Jankipersadsing, Soesma A, Steck, Andrea, Eisenbarth, Georges, van Heel, David A, Cukrowska, Bozena, Bruno, Valentina, Mazzilli, Maria Cristina, Núñez, Concepcion, Bilbao, Jose Ramon, Mearin, M Luisa, Barisani, Donatella, Rewers, Marian, Norris, Jill M, Ivarsson, Anneli, Boezen, H Marieke, Liu, Edwin, Wijmenga, Cisca, Scerri, Cristian, Koltai, Tunde, Kolaček, Sanja, Mišak, Zrinka, Abdović, Slaven, Koletzko, Sibylle, Osiander, Gertraud, Werkstetter, Katharina, Mummert, Eckart, Korponay-Szabo, Ilma R, Gyimesi, Judit, Shamir, Raanan, Hartman, Corina, Bravi, Enzo, Poles, Marco, Auricchio, Renata, Limongelli, G Gianna Giovamma, Greco, Luigi, Troncone, Riccardo, Villanacci, Vincenzo, Bindels, Jacques G, Brand, Ronald, Kupper, Bibi Funke, Esch, Caroline E Hogen, Hopman, Erica G, Koning, Frits, Kooy-Winkelaar, Yvonne, te Marvelde, Chantal, Putter, Hein, Stoopman, Els, Vriezinga, Sabine, Sollid, Ludvig M, Ráki, Melinda, Chmielewska, Ania, Dziechciarz, Piotr, Pieścik-Lech, Małgorzata, Szajewska, Hania, Szaflarska-Szczepanik, Anna, Castillejo, Gemma, Capilla, Amalia, Varea, Vicente, Ribes-Koninckx, Carmen, Lopez, Anna, Crespo, Paula, Martinez, Eva, Polanco, Isabel, Högberg, Lotta, Stenhammar, Lars, Carlsson, Annelie, Webb, Charlotta, Hammarroth, Solveig, Hernell, Olle, Lagerqvist, Carina, Myléus, Anna, Nordyke, Katrina, Norström, Fredrik, Sandström, Olof, Wall, Stig, and Karlsson, Eva
Published: 2014
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