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5. 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
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6. 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

7. Extracellular volume fraction by computed tomography predicts long-term prognosis among patients with cardiac amyloidosis

Author

Gama, F, primary, Rosmini, S, additional, Bandula, S, additional, Patel, K P, additional, Thornton, G D, additional, Bennett, J B, additional, Wechelakar, A, additional, Gillmore, J D, additional, Whelan, C, additional, Lachmann, H, additional, Taylor, S, additional, Fontana, M, additional, Moon, J, additional, Hawkins, P N, additional, and Treibel, T, additional

Published
2022
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9. Improved diagnostic accuracy for apical hypertrophic cardiomyopathy

Author

Hughes, R K, primary, Shiwani, H, additional, Rosmini, S, additional, Burke, L, additional, Pierce, I, additional, Castelletti, S, additional, Xue, H, additional, Kellman, P, additional, Lopes, L R, additional, Treibel, T, additional, Manisty, C, additional, Captur, G, additional, Davies, R, additional, and Moon, J, additional

Published
2022
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12. Global longitudinal strain by CMR improves prognostic stratification in acute myocarditis presenting with normal LVEF

Author

Porcari, A, primary, Merlo, M, additional, Baggio, C, additional, Gagno, G, additional, Andreis, A, additional, Rosmini, S, additional, Raafs, A, additional, Bromage, D, additional, Cannata', A, additional, Di Bella, G, additional, Nucifora, G, additional, Perazzolo Marra, M, additional, Heymans, S, additional, Imazio, M, additional, and Sinagra, G, additional

Published
2022
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13. Cardiovascular Remodeling Experienced by Real-World, Unsupervised, Young Novice Marathon Runners

Author

D'Silva, A, Bhuva, A, van Zalen, J, Bastiaenen, R, Abdel-Gadir, A, Jones, S, Nadarajan, N, Menacho Medina, K, Ye, Y, Augusto, J, Treibel, T, Rosmini, S, Ramlall, M, Scully, P, Torlasco, C, Willis, J, Finocchiaro, G, Papatheodorou, E, Dhutia, H, Cole, D, Chis Ster, I, Hughes, A, Sharma, R, Manisty, C, Lloyd, G, Moon, J, Sharma, S, D'Silva A., Bhuva A. N., van Zalen J., Bastiaenen R., Abdel-Gadir A., Jones S., Nadarajan N., Menacho Medina K. D., Ye Y., Augusto J., Treibel T. A., Rosmini S., Ramlall M., Scully P. R., Torlasco C., Willis J., Finocchiaro G., Papatheodorou E., Dhutia H., Cole D., Chis Ster I., Hughes A. D., Sharma R., Manisty C., Lloyd G., Moon J. C., Sharma S., D'Silva, A, Bhuva, A, van Zalen, J, Bastiaenen, R, Abdel-Gadir, A, Jones, S, Nadarajan, N, Menacho Medina, K, Ye, Y, Augusto, J, Treibel, T, Rosmini, S, Ramlall, M, Scully, P, Torlasco, C, Willis, J, Finocchiaro, G, Papatheodorou, E, Dhutia, H, Cole, D, Chis Ster, I, Hughes, A, Sharma, R, Manisty, C, Lloyd, G, Moon, J, Sharma, S, D'Silva A., Bhuva A. N., van Zalen J., Bastiaenen R., Abdel-Gadir A., Jones S., Nadarajan N., Menacho Medina K. D., Ye Y., Augusto J., Treibel T. A., Rosmini S., Ramlall M., Scully P. R., Torlasco C., Willis J., Finocchiaro G., Papatheodorou E., Dhutia H., Cole D., Chis Ster I., Hughes A. D., Sharma R., Manisty C., Lloyd G., Moon J. C., and Sharma S.

Abstract

Aims: Marathon running is a popular ambition in modern societies inclusive of non-athletes. Previous studies have highlighted concerning transient myocardial dysfunction and biomarker release immediately after the race. Whether this method of increasing physical activity is beneficial or harmful remains a matter of debate. We examine in detail the real-world cardiovascular remodeling response following competition in a first marathon. Methods: Sixty-eight novice marathon runners (36 men and 32 women) aged 30 ± 3 years were investigated 6 months before and 2 weeks after the 2016 London Marathon race in a prospective observational study. Evaluation included electrocardiography, cardiopulmonary exercise testing, echocardiography, and cardiovascular magnetic resonance imaging. Results: After 17 weeks unsupervised marathon training, runners revealed a symmetrical, eccentric remodeling response with 3–5% increases in left and right ventricular cavity sizes, respectively. Blood pressure (BP) fell by 4/2 mmHg (P < 0.01) with reduction in arterial stiffness, despite only 11% demonstrating a clinically meaningful improvement in peak oxygen consumption with an overall non-significant 0.4 ml/min/kg increase in peak oxygen consumption (P = 0.14). Conclusion: In the absence of supervised training, exercise-induced cardiovascular remodeling in real-world novice marathon runners is more modest than previously described and occurs even without improvement in cardiorespiratory fitness. The responses are similar in men and women, who experience a beneficial BP reduction and no evidence of myocardial fibrosis or persistent edema, when achieving average finishing times.

Published
2020

16. 323Inflammatory cardiomyopathy in Fabry disease

Author

Bicho Augusto, J A, primary, Nordon, S, additional, Kozor, R, additional, Vijapurapu, R, additional, Knott, K, additional, Hughes, R, additional, Rosmini, S, additional, Ramaswami, U, additional, Geberhiwot, T, additional, Steeds, R P, additional, Baig, S, additional, Hughes, D, additional, and Moon, J C, additional

Published
2019
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20. Myocardial native T1 and extracellular volume with healthy ageing and gender

Author

Rosmini, S, Bulluck, H, Captur, G, Treibel, T, Abdel-Gadir, A, Bhuva, A, Culotta, V, Merghani, A, Fontana, M, Maestrini, V, Herrey, A, Chow, K, Thompson, R, Piechnik, S, Kellman, P, Manisty, C, and Moon, J

Subjects
Adult, Male, Aging, Myocardial Infarction, Contrast Media, Magnetic Resonance Imaging, Cine, Healthy Aging, age, extracellular volume, gender, healthy volunteers, myocardial T1, T1 mapping, radiology, nuclear medicine and imaging, cardiology and cardiovascular medicine, Sex Factors, Predictive Value of Tests, Image Interpretation, Computer-Assisted, Confidence Intervals, Humans, Phantoms, Imaging, Myocardium, Body Surface Potential Mapping, Heart, Original Articles, Middle Aged, Editor's Choice, Female
Abstract

Aims To determine how native myocardial T1 and extracellular volume (ECV) change with age, both to understand aging and to inform on normal reference ranges. Methods and results Ninety-four healthy volunteers with no a history or symptoms of cardiovascular disease or diabetes underwent cardiovascular magnetic resonance at 1.5 T. Mid-ventricular short axis native and post-contrast T1 maps by Shortened MOdified Look-Locker Inversion-recovery (ShMOLLI), MOdified Look-Locker Inversion Recovery (MOLLI) [pre-contrast: 5s(3s)3s, post-contrast: 4s(1s)3s(1s)2s] and saturation recovery single-shot acquisition (SASHA) were acquired and ECV by these three techniques were derived for the mid anteroseptum. Mean age was 50 ± 14 years (range 20-76), male 52%, with no age difference between genders (males 51 ± 14 years; females 49 ± 15 years, P = 0.55). Quoting respectively ShMOLLI, MOLLI, SASHA throughout, mean myocardial T1 was 957 ± 30 ms, 1025 ± 38 ms, 1144 ± 45 ms (P Conclusion Gender influences native T1 and ECV with women having a higher native T1 and ECV. Native T1 measured by MOLLI and ShMOLLI was slightly lower with increasing age but not with SASHA and ECV was independent of age for all techniques.

Published
2018

21. European Cardiomyopathy Pilot Registry : EURObservational Research Programme of the European Society of Cardiology

Author

Elliott P., Charron P., Blanes J. R. G., Tavazzi L., Tendera M., Konte M., Laroche C., Maggioni A. P., Anastasakis A., Arbustini E., Asselbergs F. W., Axelsson A., Brito D., Caforio A. L. P., Carr-White G., Czekaj A., Damy T., Devoto E., Favalli V., Findlay I., Garcia-Pavia P., Hagege A., Helio T., Iliceto S., Isnard R., Jansweijer J. A., Limongelli G., Linhart A., Cuenca D. L., Mansencal N., McKeown P., Mogensen J., Mohiddin S. A., Monserrat L., Olivotto I., Rapezzi C., Rigopoulos A. G., Rosmini S., Pfeiffer B., Wicks E., Podzimkova J., Kuchynka P., Palecek T., Bundgaard H., Thune J. J., Kumme A., Due Vestergaard L., Hey T., Ollila L., Kaartinen M., Dubourg O., Arslan M., Siam Tsieu M., Guellich A., Tissot C. -M., Guendouz S., Thevenin S., Cheikh Khelifa R., Gandjbakhch E., Komajda M., Neugebauer A., Steriotis A., Ritsatos K., Vlagkouli V., Biagini E., Gentile N., Longhi S., Arretini A., Fornaro A., Cecchi F., Spirito P., Formisano F., Masarone D., Valente F., Pacileo G., Schiavo A., Testolina M., Serio A., Grasso M., Wilde A., Pinto Y., Klopping C., Van Der Heijden J. F., De Jonge N., Sikora-Puz A., Wybraniec M., Francisco A. R., Madeira H., Ortiz-Genga M., Barriales-Villa R., Fernandez X., Lopez-Cuenca D., Gomez-Milanes I., Lopez-Ayala J. M., Guzzo-Merello G., Gallego-Delgado M., Muir A., McOsker J., Jardine T., Iqbal H., Sekhri N., Rajani R., Bueser T., Watkinson O., Cardiology, ACS - Amsterdam Cardiovascular Sciences, ACS - Heart failure & arrhythmias, Perry Elliott, Philippe Charron, Juan Ramon Gimeno Blane, Luigi Tavazzi, Michal Tendera, Marème Konté, Cécile Laroche, Aldo P. Maggioni, the EORP Cardiomyopathy Registry Pilot Investigators: [Aris Anastasaki, Eloisa Arbustini, Folkert W. Asselberg, Anna Axelsson, Dulce Brito, Alida L.P. Caforio, Gerald Carr-White, Agata Czekaj, Thibaud Damy, Emmanuela Devoto, Valentina Favalli, Iain Findlay, Pablo Garcia-Pavia, Albert Hagège, Tiina Heliö, Sabino Iliceto, Richard Isnard, Joeri A. Jansweijer, Giuseppe Limongelli, Ales Linhart, David López Cuenca, Nicolas Mansencal, Pascal McKeown, Jens Mogensen, Saidi A. Mohiddin, Lorenzo Monserrat, Iacopo Olivotto, Claudio Rapezzi, A.G. Rigopoulo, Stefania Rosmini, Barbara Pfeiffer, Eleanor Wicks], Elliott, P., Charron, P., Blanes, J. R. G., Tavazzi, L., Tendera, M., Konte, M., Laroche, C., Maggioni, A. P., Anastasakis, A., Arbustini, E., Asselbergs, F. W., Axelsson, A., Brito, D., Caforio, A. L. P., Carr-White, G., Czekaj, A., Damy, T., Devoto, E., Favalli, V., Findlay, I., Garcia-Pavia, P., Hagege, A., Helio, T., Iliceto, S., Isnard, R., Jansweijer, J. A., Limongelli, G., Linhart, A., Cuenca, D. L., Mansencal, N., Mckeown, P., Mogensen, J., Mohiddin, S. A., Monserrat, L., Olivotto, I., Rapezzi, C., Rigopoulos, A. G., Rosmini, S., Pfeiffer, B., Wicks, E., Podzimkova, J., Kuchynka, P., Palecek, T., Bundgaard, H., Thune, J. J., Kumme, A., Due Vestergaard, L., Hey, T., Ollila, L., Kaartinen, M., Dubourg, O., Arslan, M., Siam Tsieu, M., Guellich, A., Tissot, C. -M., Guendouz, S., Thevenin, S., Cheikh Khelifa, R., Gandjbakhch, E., Komajda, M., Neugebauer, A., Steriotis, A., Ritsatos, K., Vlagkouli, V., Biagini, E., Gentile, N., Longhi, S., Arretini, A., Fornaro, A., Cecchi, F., Spirito, P., Formisano, F., Masarone, D., Valente, F., Pacileo, G., Schiavo, A., Testolina, M., Serio, A., Grasso, M., Wilde, A., Pinto, Y., Klopping, C., Van Der Heijden, J. F., De Jonge, N., Sikora-Puz, A., Wybraniec, M., Francisco, A. R., Madeira, H., Ortiz-Genga, M., Barriales-Villa, R., Fernandez, X., Lopez-Cuenca, D., Gomez-Milanes, I., Lopez-Ayala, J. M., Guzzo-Merello, G., Gallego-Delgado, M., Muir, A., Mcosker, J., Jardine, T., Iqbal, H., Sekhri, N., Rajani, R., Bueser, T., and Watkinson, O.

Subjects
Registrie, Male, Pacemaker, Artificial, Cardiomyopathy, Pilot Projects, 030204 cardiovascular system & hematology, Defibrillator, 0302 clinical medicine, Interquartile range, Residence Characteristics, Dilated, Medicine, 030212 general & internal medicine, Registries, Age of Onset, Non-U.S. Gov't, Research Support, Non-U.S. Gov't, Hypertrophic cardiomyopathy, Dilated cardiomyopathy, Middle Aged, Arrhythmogenic right ventricular dysplasia, Europe, Multicenter Study, cardiovascular system, Cardiology, Arrhythmogenic right ventricular, Female, Cardiology and Cardiovascular Medicine, Cardiomyopathies, Human, Adult, medicine.medical_specialty, Registry, Cardiotonic Agents, Restrictive, Observational Study, Research Support, Right ventricular cardiomyopathy, NO, 03 medical and health sciences, Age Distribution, Internal medicine, Journal Article, Humans, Cardiotonic Agent, Pilot Project, cardiovascular diseases, Sex Distribution, Cardiomyopathie, business.industry, Restrictive cardiomyopathy, Hypertrophic, medicine.disease, Death, Sudden, Cardiac, Residence Characteristic, Heart failure, business, Defibrillators
Abstract

AIMS: Cardiomyopathies are a heterogeneous group of disorders associated with premature death due to ventricular arrhythmia or heart failure. The purpose of this study was to examine the characteristics of patients enrolled in the pilot phase of the EURObservational Research Programme (EORP) cardiomyopathy registry. METHODS AND RESULTS: Between 1 December 2012 and 30 November 2013, four cardiomyopathy phenotypes were studied: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). Twenty-seven centres in 12 countries participated; 1115 patients were enrolled. The commonest cardiomyopathy was HCM (n = 681), followed by DCM (n = 346), ARVC (n = 59), and RCM (n = 29); 423 patients (46.4% of those reported) had familial disease; and 56 (5.0%) had rare disease phenocopies. Median age at enrolment and diagnosis was 54 [interquartile range (IQR), 42-64] and 46 years (IQR, 32-58), respectively; fewer patients with ARVC and more with RCM were diagnosed in the upper age quartile (P < 0.0001). There was a male predominance for all cardiomyopathies except RCM (P = 0.0023). Most patients were in New York Heart Association functional class I (n = 813) at enrolment; 139 (12.5%) reported syncope, most frequently in ARVC (P = 0.0009). Five hundred and seven (45.5%) patients underwent cardiac magnetic resonance imaging, 117 (10.6%) endomyocardial biopsy, and 462 (41.4%) genetic testing with a causative mutation reported in 236 individuals (51.1%). 1026 patients (92.0%) were receiving drug therapy; 316 (28.3%) had received an implantable cardioverter defibrillator (highest proportion in ARVC, P < 0.0001). CONCLUSION: This pilot study shows that services for patients with cardiomyopathy are complex, requiring access to a large range of invasive and non-invasive investigations and involvement of multidisciplinary teams. Treatment regimens are equally multifaceted and show that patients are likely to need long-term follow-up in close liaison with expert centres.

Published
2016

22. 1 Inflammatory cardiomyopathy in fabry disease

Author

Augusto, JB, primary, Nordin, S, additional, Kozor, R, additional, Vijapurapu, R, additional, Knott, K, additional, Hughes, R, additional, Rosmini, S, additional, Ramaswami, U, additional, Geberhiwot, T, additional, Steeds, RP, additional, Baig, S, additional, Hughes, D, additional, and Moon, JC, additional

Published
2019
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25. European cardiomyopathy pilot registry: EURObservational research programme of the European society of cardiology

Author

Elliott, P. Charron, P. Blanes, J.R.G. Tavazzi, L. Tendera, M. Konté, M. Laroche, C. Maggioni, A.P. Anastasakis, A. Arbustini, E. Asselbergs, F.W. Axelsson, A. Brito, D. Caforio, A.L.P. Carr-White, G. Czekaj, A. Damy, T. Devoto, E. Favalli, V. Findlay, I. Garcia-Pavia, P. Hagège, A. Heliö, T. Iliceto, S. Isnard, R. Jansweijer, J.A. Limongelli, G. Linhart, A. Cuenca, D.L. Mansencal, N. McKeown, P. Mogensen, J. Mohiddin, S.A. Monserrat, L. Olivotto, I. Rapezzi, C. Rigopoulos, A.G. Rosmini, S. Pfeiffer, B. Wicks, E. Podzimkova, J. Kuchynka, P. Palecek, T. Bundgaard, H. Thune, J.J. Kumme, A. Due Vestergaard, L. Hey, T. Ollila, L. Kaartinen, M. Dubourg, O. Arslan, M. Siam Tsieu, M. Guellich, A. Tissot, C.-M. Guendouz, S. Thevenin, S. Cheikh Khelifa, R. Gandjbakhch, E. Komajda, M. Neugebauer, A. Pfeiffer, B. Steriotis, A. Ritsatos, K. Vlagkouli, V. Biagini, E. Gentile, N. Longhi, S. Arretini, A. Fornaro, A. Cecchi, F. Spirito, P. Formisano, F. Masarone, D. Valente, F. Pacileo, G. Schiavo, A. Testolina, M. Serio, A. Grasso, M. Wilde, A. Pinto, Y. Klöpping, C. Van Der Heijden, J.F. De Jonge, N. Sikora-Puz, A. Wybraniec, M. Czekaj, A. Francisco, A.R. Brito, D. Madeira, H. Ortiz-Genga, M. Barriales-Villa, R. Fernandez, X. Lopez-Cuenca, D. Gomez-Milanes, I. Lopez-Ayala, J.M. Guzzo-Merello, G. Gallego-Delgado, M. Muir, A. McOsker, J. Jardine, T. Iqbal, H. Sekhri, N. Rajani, R. Bueser, T. Watkinson, O. on behalf of the EORP Cardiomyopathy Registry Pilot Investigators

Abstract

Aims: Cardiomyopathies are a heterogeneous group of disorders associated with premature death due to ventricular arrhythmia or heart failure. The purpose of this study was to examine the characteristics of patients enrolled in the pilot phase of the EURObservational Research Programme (EORP) cardiomyopathy registry. Methods and results: Between 1 December 2012 and 30 November 2013, four cardiomyopathy phenotypes were studied: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). Twenty-seven centres in 12 countries participated; 1115 patients were enrolled. The commonest cardiomyopathy was HCM (n = 681), followed by DCM (n = 346), ARVC (n = 59), and RCM (n = 29); 423 patients (46.4% of those reported) had familial disease; and 56 (5.0%) had rare disease phenocopies. Median age at enrolment and diagnosis was 54 [interquartile range (IQR), 42-64] and 46 years (IQR, 32-58), respectively; fewer patients with ARVC and more with RCM were diagnosed in the upper age quartile (P, 0.0001). There was a male predominance for all cardiomyopathies except RCM (P = 0.0023). Most patients were in New York Heart Association functional class I (n = 813) at enrolment; 139 (12.5%) reported syncope, most frequently in ARVC (P = 0.0009). Five hundred and seven (45.5%) patients underwent cardiac magnetic resonance imaging, 117 (10.6%) endomyocardial biopsy, and 462 (41.4%) genetic testing with a causative mutation reported in 236 individuals (51.1%). 1026 patients (92.0%) were receiving drug therapy; 316 (28.3%) had received an implantable cardioverter defibrillator (highest proportion in ARVC, P, 0.0001). Conclusion: This pilot study shows that services for patients with cardiomyopathy are complex, requiring access to a large range of invasive and non-invasive investigations and involvement of multidisciplinary teams. Treatment regimens are equally multifaceted and show that patients are likely to need long-term follow-up in close liaison with expert centres. © The Author 2015.

Published
2016

27. YOUNG INVESTIGATORS COMPETITION1GENETIC ANALYSIS IN THE EVALUATION OF UNEXPLAINED CARDIAC ARREST: FROM THE CARDIAC ARREST SURVIVORS WITH PRESERVED EJECTION FRACTION REGISTRY (CASPER)2IN-VIVO WHOLE HEART CONTACT MAPPING DATA AND A SIMPLE MATHEMATICAL FRAMEWORK TO UNDERSTAND THE INTERACTIONS BETWEEN ACTIVATION AND REPOLARIZATION RESITUTION DYNAMICS IN THE INTACT HUMAN HEART3THE K(ATP) CHANNEL OPENER DIAZOXIDE REDUCES AUTOMATICITY IN AN IN VITRO ATRIAL CELL MODEL - POTENTIAL FOR K(ATP) CHANNELS AS A DRUG TARGET FOR ATRIAL ARRHYTHMIAS4LONG-TERM OUTCOMES AFTER CATHETER ABLATION OF VENTRICULAR TACHYCARDIA IN PATIENTS WITH STRUCTURAL HEART DISEASE: A MULTICENTRE UK STUDY5THE BURDEN OF ARRHYTHMIAS IN LIFE-LONG ENDURANCE ATHLETES6CARDIAC MAGNETIC RESONANCE IMAGING RISK STRATIFICATION USING MARKERS OF REGIONAL AND DIFFUSE FIBROSIS FOR IMPLANTABLE CARDIOVERTER DEFIBRILLATOR THERAPY: THE VALUE OF T1 MAPPING IN NON-ISCHEMIC PATIENTS

Author

Mellor, G., primary, Orini, M., primary, Specterman, M.J., primary, Sawhney, V.S., primary, Merghani, A., primary, Claridge, S., primary, Laksman, Z., additional, Gerull, B., additional, Simpson, C., additional, Klein, G., additional, Champagne, J., additional, Talajic, M., additional, Gardner, M., additional, Steinberg, C., additional, Janzen, M., additional, Arbour, L., additional, Green, M., additional, Angaran, P., additional, Roberts, J., additional, Leather, R., additional, Sanatani, S., additional, Chauhan, V., additional, Healey, J., additional, Krahn, A., additional, Taggart, P., additional, Srinivasan, N., additional, Hayward, M., additional, Lambiase, P.D., additional, Aziz, Q., additional, Finlay, M.C., additional, Nobles, M., additional, Anderson, N., additional, Ng, K., additional, Schilling, R.J., additional, Tinker, A., additional, Breitenstein, A.B., additional, Ullah, W.U., additional, Honarbakhsh, S.H., additional, Dhinoja, M.D., additional, Schilling, R.J.S., additional, Providencia, R.P., additional, Babu, G.B., additional, Chow, A.C., additional, Lambiase, P.L., additional, Panikker, S.P., additional, Kontogeorgis, A.K., additional, Wong, T.W., additional, Hall, M.H., additional, Temple, I.T., additional, Bartoletti, S.B., additional, Kalla, M.K., additional, Cassar, M.C., additional, Rajappan, K.R., additional, Hunter, R.H., additional, Maestrini, V., additional, Rosmini, S., additional, Cox, A.T., additional, Yeo, T.J., additional, Dhutia, H., additional, Narain, R., additional, Malhotra, A., additional, Behr, E., additional, Tome, M., additional, Alfakih, K., additional, Moon, J.C., additional, Sharma, S., additional, Mennuni, Silvia, additional, Jackson, T., additional, Behar, J., additional, Porter, B., additional, Sieniewicz, B., additional, Webb, J., additional, Bostock, J., additional, O'Neill, M., additional, Murgatroyd, F., additional, Carr-White, G., additional, Chiribiri, A., additional, Razavi, R., additional, Chen, Z., additional, and Rinaldi, C., additional

Published
2016
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29. 29 Synthetic ECV – simplifying ECV quantification by deriving haematocrit from T1 blood

Author

Treibel, TA, primary, Fontana, M, additional, Maestrini, V, additional, Castelletti, S, additional, Rosmini, S, additional, Simpson, J, additional, Nasis, A, additional, Bulluck, H, additional, Abdel-Gadir, A, additional, White, SK, additional, Manisty, C, additional, Kellman, P, additional, Schelbert, EB, additional, Robson, MD, additional, Piechnik, SK, additional, and Moon, JC, additional

Published
2015
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30. MODERATED POSTER SESSION: Imaging in cardiomyopathies: Friday 5 December 2014, 08:30-18:00 * Location: Moderated Poster area

Author

Risum, N., primary, Tayal, B., additional, Fritz Hansen, T., additional, Bruun, N., additional, Saba, S., additional, Kisslo, J., additional, Gorcsan, J., additional, Sogaard, P., additional, Venner, C., additional, Selton-Suty, C., additional, Huttin, O., additional, Voilliot, D., additional, Marie, P., additional, Aliot, E., additional, Juilliere, Y., additional, Tsukishiro, Y., additional, Onishi, T., additional, Matsuyama, S., additional, Chimura, M., additional, Yamada, S., additional, Taniguchi, Y., additional, Yasaka, Y., additional, Kawai, H., additional, Reant, P., additional, Mirabel, M., additional, Dickie, S., additional, Rosmini, S., additional, Demetrescu, C., additional, Tome-Esteban, M., additional, Moon, J., additional, Lafitte, S., additional, Elliott, P., additional, Mckenna, W., additional, Ozawa, K., additional, Funabashi, N., additional, Takaoka, H., additional, Kobayashi, Y., additional, Zegri Reiriz, I., additional, Alcolado, A., additional, Mendez, C., additional, Sanchez, M., additional, Gomez, Y., additional, Climent, V., additional, Ripoll, T., additional, Montserrat, L., additional, Gimeno, J., additional, Garcia-Pavia, P., additional, Hu, K., additional, Liu, D., additional, Cikes, M., additional, Stoerk, S., additional, Kramer, B., additional, Gaudron, P., additional, Ertl, G., additional, Bijnens, B., additional, Weidemann, F., additional, Herrmann, S., additional, Kagiyama, N., additional, Okura, H., additional, Yamada, R., additional, Kume, T., additional, Neishi, Y., additional, Ohara, M., additional, Hayashida, A., additional, Hirohata, A., additional, Yamamoto, K., additional, Yoshida, K., additional, Sade, L. E., additional, Kozan, H., additional, Eroglu, S., additional, Pirat, B., additional, Sezgin, A., additional, Aydinalp, A., additional, Muderrisoglu, H., additional, Agricola, E., additional, Spoladore, R., additional, Ballarotto, M., additional, Fisicaro, A., additional, Marcatti, M., additional, Margonato, A., additional, and Camici, P., additional

Published
2014
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34. 323 Inflammatory cardiomyopathy in Fabry disease.

Author

Augusto, J A Bicho, Nordon, S, Kozor, R, Vijapurapu, R, Knott, K, Hughes, R, Rosmini, S, Ramaswami, U, Geberhiwot, T, Steeds, R P, Baig, S, Hughes, D, and Moon, J C

Subjects
CONFERENCES & conventions, INFLAMMATION, CARDIOMYOPATHIES, DILATED cardiomyopathy, ANGIOKERATOMA corporis diffusum
Published
2019
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39. MODERATED POSTER SESSION: Imaging in cardiomyopathies: Friday 5 December 2014, 08:30-18:00 * Location: Moderated Poster area

Author

Risum, N, Tayal, B, Fritz Hansen, T, Bruun, NE, Saba, S, Kisslo, J, Gorcsan, J, Sogaard, P, Venner, C, Selton-Suty, C, Huttin, O, Voilliot, D, Marie, PY, Aliot, E, Juilliere, Y, Tsukishiro, Y, Onishi, T, Matsuyama, S, Chimura, M, Yamada, S, Taniguchi, Y, Yasaka, Y, Kawai, H, Reant, P, Mirabel, M, Dickie, S, Rosmini, S, Demetrescu, C, Tome-Esteban, M, Moon, JC, Lafitte, S, Elliott, PM, Mckenna, W, Ozawa, K, Funabashi, N, Takaoka, H, Kobayashi, Y, Zegri Reiriz, I, Alcolado, A, Mendez, C, Sanchez, MJ, Gomez, Y, Climent, V, Ripoll, T, Montserrat, L, Gimeno, JR, Garcia-Pavia, P, Hu, K, Liu, D, Cikes, M, Stoerk, S, Kramer, B, Gaudron, PD, Ertl, G, Bijnens, B, Weidemann, F, Herrmann, S, Kagiyama, N, Okura, H, Yamada, R, Kume, T, Neishi, Y, Ohara, M, Hayashida, A, Hirohata, A, Yamamoto, K, Yoshida, K, Sade, L E, Kozan, H, Eroglu, S, Pirat, B, Sezgin, A, Aydinalp, A, Muderrisoglu, H, Agricola, E, Spoladore, R, Ballarotto, M, Fisicaro, A, Marcatti, M, Margonato, A, and Camici, PG

Abstract

Background: Recent studies have demonstrated that longitudinal septal deformation in patients with left ventricular (LV) dysfunction and left bundle branch block (LBBB) predict LV remodeling after CRT. However, the importance of septal deformation patterns for prediction of long-term survival is unknown. Methods: From 2 centers a total of 193 CRT candidates with LBBB (NYHA II-IV, EF≤35, and QRS≥120ms) underwent echocardiography before CRT. Longitudinal 2-D strain analysis in the apical four-chamber view identified four patterns based on previously defined criteria: double-peaked systolic pattern (type 1), early pre-ejection shortening peak followed by prominent systolic stretch (type 2), shortening with one systolic peak inside 70% of ejection phase (type 3) and normal septal peak timing outside early 70% (type 4) . Outcome was pre-defined as freedom from death, left ventricular assist device or heart transplantation over 4 years. Results: Thirty-six patients (19%) had early septal deformation pattern type 1, 49(25%) type 2, 44(23%) type 3 and 64(33%) pseudonormal septal contraction type 4. There were 35 deaths, 4 transplantations, and 6 left ventricular assist device implantations over 4 years. The event rate was 0.05% (2/36) for type 1, 14% (7/49) for type 2, 25% (11/44) for type 3, 39% (25/64) for type 4. Patients with type 1 and 2 patterns had a more favourable event-free survival HR 0.26, CI 0.09-0.81 (P=0.005) than type 3 HR 1.17, CI 0.06-2.2 (P=0.65). Patients with type 4 septal contraction showed poor event-free survival HR 2.8, CI 1.6-5.1 (<0.001). (Figure 1) Conclusions: In LBBB-patients, septal deformation strain patterns predict long-term survival after CRT. Pattern 1 and 2 are highly associated with long-term survival while patients with pattern 4 have a poor prognosis. Figure

Published
2014
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41. Cardiovascular Remodeling Experienced by Real-World, Unsupervised, Young Novice Marathon Runners

Author

Charlotte Manisty, James Willis, Thomas A. Treibel, N Nadarajan, João B Augusto, Paul Scully, Della Cole, Manish Ramlall, Alun D. Hughes, Harshil Dhutia, Siana Jones, Efstathios Papatheodorou, Sanjay Sharma, Irina Chis Ster, Anish N Bhuva, Guy Lloyd, Camilla Torlasco, Yang Ye, Stefania Rosmini, James C. Moon, Amna Abdel-Gadir, Rajan Sharma, Gherardo Finocchiaro, Katia D. Menacho Medina, Jet van Zalen, Rachel Bastiaenen, Andrew D'Silva, D'Silva, A, Bhuva, A, van Zalen, J, Bastiaenen, R, Abdel-Gadir, A, Jones, S, Nadarajan, N, Menacho Medina, K, Ye, Y, Augusto, J, Treibel, T, Rosmini, S, Ramlall, M, Scully, P, Torlasco, C, Willis, J, Finocchiaro, G, Papatheodorou, E, Dhutia, H, Cole, D, Chis Ster, I, Hughes, A, Sharma, R, Manisty, C, Lloyd, G, Moon, J, and Sharma, S

Subjects
medicine.medical_specialty, Physiology, endurance exercise, athlete’s heart, cardiorespiratory fitness, cardiovascular remodeling, endurance exercise, marathon, sports cardiology, 030204 cardiovascular system & hematology, lcsh:Physiology, 03 medical and health sciences, 0302 clinical medicine, Endurance training, Physiology (medical), Internal medicine, medicine, Eccentric, 030212 general & internal medicine, Original Research, cardiovascular remodeling, cardiorespiratory fitness, lcsh:QP1-981, medicine.diagnostic_test, business.industry, Cardiorespiratory fitness, cardiorespiratory fitne, medicine.disease, Blood pressure, Arterial stiffness, Cardiology, Biomarker (medicine), Observational study, athlete’s heart, marathon, business, sports cardiology, human activities, Electrocardiography
Abstract

Aims: Marathon running is a popular ambition in modern societies inclusive of non-athletes. Previous studies have highlighted concerning transient myocardial dysfunction and biomarker release immediately after the race. Whether this method of increasing physical activity is beneficial or harmful remains a matter of debate. We examine in detail the real-world cardiovascular remodeling response following competition in a first marathon. Methods: Sixty-eight novice marathon runners (36 men and 32 women) aged 30 ± 3 years were investigated 6 months before and 2 weeks after the 2016 London Marathon race in a prospective observational study. Evaluation included electrocardiography, cardiopulmonary exercise testing, echocardiography, and cardiovascular magnetic resonance imaging. Results: After 17 weeks unsupervised marathon training, runners revealed a symmetrical, eccentric remodeling response with 3-5% increases in left and right ventricular cavity sizes, respectively. Blood pressure (BP) fell by 4/2 mmHg (P < 0.01) with reduction in arterial stiffness, despite only 11% demonstrating a clinically meaningful improvement in peak oxygen consumption with an overall non-significant 0.4 ml/min/kg increase in peak oxygen consumption (P = 0.14). Conclusion: In the absence of supervised training, exercise-induced cardiovascular remodeling in real-world novice marathon runners is more modest than previously described and occurs even without improvement in cardiorespiratory fitness. The responses are similar in men and women, who experience a beneficial BP reduction and no evidence of myocardial fibrosis or persistent edema, when achieving average finishing times.

Published
2020
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42. Significance of sarcomere gene mutations analysis in the end-stage phase of hypertrophic cardiomyopathy

Author

Francesca Girolami, Attilio Iacovoni, Elena Biagini, Giuseppe Limongelli, Maria Isola Parodi, Camillo Autore, Franco Cecchi, Giulia Frisso, Martin S. Maron, Massimiliano Cecconi, Claudio Rapezzi, Paolo Spirito, Stefania Rosmini, Maria Letizia Bacchi Reggiani, Beatrice Musumeci, Barry J. Maron, Maria Iascone, Iacopo Olivotto, Francesco Formisano, Tammy S. Haas, Paolo Ferrazzi, Francesco Salvatore, Biagini, E, Olivotto, I, Iascone, M, Parodi, Mi, Girolami, F, Frisso, G, Autore, C, Limongelli, Giuseppe, Cecconi, M, Maron, Bj, Maron, M, Rosmini, S, Formisano, F, Musumeci, B, Cecchi, F, Iacovoni, A, Haas, T, Bacchi reggiani, Ml, Ferrazzi, P, Salavtore, F, Spirito, P, Rapezzi, C., Biagini, Elena, Olivotto, Iacopo, Iascone, Maria, Parodi, Maria I, Girolami, Francesca, Frisso, Giulia, Autore, Camillo, Cecconi, Massimiliano, Maron, Barry J, Maron, Martin S, Rosmini, Stefania, Formisano, Francesco, Musumeci, Beatrice, Cecchi, Franco, Iacovoni, Attilio, Haas, Tammy S, Bacchi Reggiani, Maria L, Ferrazzi, Paolo, Salvatore, Francesco, Spirito, Paolo, Rapezzi, Claudio, Parodi, Maria I., Maron, Barry J., Maron, Martin S., Haas, Tammy S., and Bacchi Reggiani, Maria L.

Subjects
Male, Pathology, TNNT2, DNA Mutational Analysis, TPM1, Gene mutation, Severity of Illness Index, Adult, Aged, Cardiomyopathy, Hypertrophic, Familial, Carrier Proteins, Cross-Sectional Studies, DNA, Echocardiography, Female, Follow-Up Studies, Genotype, Humans, Magnetic Resonance Imaging, Cine, Middle Aged, Myosin Heavy Chains, Pedigree, Prevalence, Retrospective Studies, Sarcomeres, Mutation, Cardiology and Cardiovascular Medicine, Retrospective Studie, Cardiomyopathy, Hypertrophic, Familial, Magnetic Resonance Imaging, Cine, Hypertrophic cardiomyopathy, Sarcomere, Cardiology, Human, medicine.medical_specialty, Follow-Up Studie, NO, DNA Mutational Analysi, Internal medicine, medicine, Cross-Sectional Studie, business.industry, ACTC1, Myosin Heavy Chain, medicine.disease, MYL3, MYL2, MYH7, Carrier Protein, business
Abstract

End-stage hypertrophic cardiomyopathy (ES-HC) has an ominous prognosis. Whether genotype can influence ES-HC occurrence is unresolved. We assessed the spectrum and clinical correlates of HC-associated mutations in a large multicenter cohort with end-stage ES-HC. Sequencing analysis of 8 sarcomere genes (MYH7, MYBPC3, TNNI3, TNNT2, TPM1, MYL2, MYL3, and ACTC1) and 2 metabolic genes (PRKAG2 and LAMP2) was performed in 156 ES-HC patients with left ventricular (LV) ejection fraction (EF)

Published
2014

43. Predictors of complicated athero-thrombotic lesions in non-ST segment acute coronary syndrome

Author

Laura Alessi, Laura Cinti, Carolina Moretti, Antonio Marzocchi, Claudio Rapezzi, Michela Montefiori, Stefania Rosmini, Tullio Palmerini, Cinzia Marrozzini, Fabio Vagnarelli, Nevio Taglieri, Gianni Dall'Ara, Angelo Branzi, Francesco Saia, Taglieri N, Dall'Ara G, Rapezzi C, Saia F, Cinti L, Rosmini S, Alessi L, Vagnarelli F, Moretti C, Palmerini T, Marrozzini C, Montefiori M, Branzi A, and Marzocchi A.

Subjects
Male, medicine.medical_treatment, Myocardial Infarction, Infarction, Coronary Artery Disease, Kaplan-Meier Estimate, Coronary Angiography, Angina, Recurrence, Risk Factors, Clinical endpoint, Odds Ratio, ST segment, Coronary lesions, Non-ST segment elevation acute coronary syndrome, Aged, 80 and over, Framingham Risk Score, General Medicine, Middle Aged, Risk stratification, Plaque, Atherosclerotic, Cardiology, Disease Progression, Female, Cardiology and Cardiovascular Medicine, medicine.medical_specialty, Acute coronary syndrome, ACUTE CORONARY SYNDROMES, angina, Global Registry of Acute Coronary Events, NO, Predictive Value of Tests, Internal medicine, medicine, Humans, Angina, Unstable, Acute Coronary Syndrome, Aged, Retrospective Studies, Chi-Square Distribution, Rupture, Spontaneous, business.industry, Coronary Thrombosis, Percutaneous coronary intervention, Odds ratio, medicine.disease, Logistic Models, Multivariate Analysis, business
Abstract

AIMS: Non-ST segment elevation acute coronary syndrome (NSTE-ACS) is a heterogeneous syndrome in terms of patho-physiological mechanisms and prognosis. We sought to investigate the clinical features associated with complicated athero-thrombotic (CAT) coronary lesions and their prognostic relevance in NSTE-ACS. METHODS: We enrolled 701 consecutive NSTE-ACS patients without previous coronary bypass undergoing coronary angiography. The study population was divided into two groups according to the presence/absence of angiographic signs of endoluminal thrombi and/or plaque rupture, defined as CAT lesions. Multivariable analyses were used to identify predictors of CAT lesions. Their relation to composite endpoint of death, re-myocardial infarction, and re-unstable angina was investigated with the use of multivariable logistic regression. RESULTS: Patients with CAT lesions (n = 279, 40%) had a higher incidence of the combined endpoint (11.5 vs. 4.3%; P

Published
2013

44. Baseline white blood cell count is an independent predictor of long-term cardiovascular mortality in patients with non-ST-segment elevation acute coronary syndrome, but it does not improve the risk classification of the GRACE score

Author

Maria Letizia Bacchi Reggiani, Francesco Saia, Carolina Moretti, Fabio Vagnarelli, Michela Montefiori, Antonio Marzocchi, Laura Cinti, Nevio Taglieri, Stefania Rosmini, Laura Alessi, Tullio Palmerini, Claudio Rapezzi, Paolo Guastaroba, Cinzia Marrozzini, Angelo Branzi, Taglieri N, Bacchi Reggiani ML, Palmerini T, Cinti L, Saia F, Guastaroba P, Marrozzini C, Moretti Carolina, Montefiori M, Rosmini S, Alessi L, Vagnarelli F, Branzi A, Rapezzi C, and Marzocchi A

Subjects
medicine.medical_specialty, Acute coronary syndrome, Cardiovascular mortality, business.industry, Follow up studies, White blood cell count, Global Registry of Acute Coronary Events score, Independent predictor, medicine.disease, NO, medicine.anatomical_structure, White blood cell, Internal medicine, Cardiology, Medicine, ST segment, Pharmacology (medical), In patient, Cardiology and Cardiovascular Medicine, business, Risk classification
Abstract

Objectives: To investigate the prognostic significance of baseline white blood cell count (WBCc) in non-ST-segment elevation acute coronary syndrome (NSTE-ACS) and its additive predictive value beyond the Global Registry of Acute Coronary Events (GRACE) score. Methods: We included 1,315 consecutive NSTE-ACS patients. Patients were divided in quartiles according to the WBCc (cells per 1 mm3) i.e. Q1 Results: The median age of the study population was 76 years. Overall, 335 patients (25.5%) died with 211 of these (16%) suffering from CVD. Patients in Q4 showed a higher cumulative probability of CVD compared to patients in Q1–Q3. On multivariable analysis, patients in Q4 were at higher risk of CVD [hazard ratio (HR) = 1.47, 95% confidence interval (CI) 1.09–1.98, p = 0.011]. WBCc as a continuous variable was also independently associated with the study end point (HR = 1.043; 95% CI 1.02–1.07; p = 0.001). However, the incorporation of WBCc into the GRACE score did not improve either prediction of risk (C-index = 0.796 for GRACE score with or without WBCc) or classification of risk [relative integrated discrimination improvement = 0.0154, 95% CI) –0.029 to 0.0618; continuous net reclassification improvement = –0.0676, 95% CI –0.2149–0.0738). Conclusions: WBCc was an independent predictor of 3-year CVD in patients with NSTE-ACS. However, it did not add prognostic information beyond the GRACE score.

Published
2013

45. Effetto donna' nelle cardiomiopatie Gender effect on cardiomyopathy

Author

BIAGINI, ELENA, BERARDINI, ALESSANDRA, GRAZIOSI, MADDALENA, ROSMINI, STEFANIA, PAZZI, CHIARA, RAPEZZI, CLAUDIO, Biagini E, Berardini A, Graziosi M, Rosmini S, Pazzi C, and Rapezzi C

Subjects
GENERE, Donne, CARDIOMYOPATHIES, GENDER, WOMAN, DONNA, CARDIOMIOPATIA, Cardiomiopatie, Genere, NO
Abstract

The role of a gender effect (that means differences in clinical manifestations, access to therapies and response to treatments according to gender) in cardiomyopathies remains a matter of debate. Although recent studies have evaluated the differences in the clinical features and prognosis between the two sexes, many issues remain to be elucidated. At present, the only sex-specific condition that affects females is peripartum cardiomyopathy. Recent evidence suggests a pathogenetic role of a prolactin derivative, and ongoing clinical trials are investigating the possibility of targeted therapies using prolactin secretion inhibitors, such as bromocriptine and carbegoline. Although women were considered so far only carriers of X-linked diseases (Anderson-Fabry disease, Danon disease, Hunter syndrome and dystrophinopathies), clinical experience showed a wide spectrum of clinical manifestations in females due to random X chromosome inactivation. Conversely, in mitochondrial diseases (with matrilineal inheritance), cardiomyopathies may occur in the context of clinical multisystemic involvement without significant gender-related differences. Autosomal inherited cardiomyopathies also show different phenotypes and prognostic impact according to gender. The hypothesis of a premenopausal protective role of female hormones towards myocardial involvement has been raised by recent data on transtiretin-related amyloidosis and hypertrophic cardiomyopathy. Preexisting cardiomyopathies may affect pregnancy, labor and delivery in women, since all these conditions are associated with important hemodynamic changes. Women with low-risk hypertrophic cardiomyopathy (asymptomatic and without left ventricular outflow tract gradient) usually can tolerate pregnancy. Conversely, women who are symptomatic before pregnancy or have severe hypertrophy with important outflow tract gradient are at higher risk and should be referred to a tertiary center to be evaluated on a case by case basis. Pregnancy in women with dilated cardiomyopathy and significant left ventricular systolic dysfunction represents a high-risk condition. In addition, information on the clinical course and potential complications in pregnant women with arrhythmogenic right ventricular cardiomyopathy or restrictive cardiomyopathy is limited to individual reports.

Published
2012

46. Effects of myocardial fibrosis assessed by MRI on dynamic left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy: A retrospective database analysis

Author

Rossella Fattori, Elena Biagini, Luigi Lovato, Chiara Pazzi, Stefania Rosmini, Massimiliano Lorenzini, Ferdinando Pasquale, Francesco Lai, Iacopo Olivotto, Claudio Rapezzi, Maria Letizia Bacchi Reggiani, Guido Rocchi, Biagini E, Lorenzini M, Olivotto I, Rocchi G, Lovato L, Lai F, Rosmini S, Pazzi C, Pasquale F, Reggiani ML, Fattori R, and Rapezzi C.

Subjects
medicine.medical_specialty, myocardial fibrosis, MRI, late-gadolinium enhancement (LGE), LVOT gradient, HCM, GeneralLiterature_INTRODUCTORYANDSURVEY, Population, Ventricular outflow tract obstruction, SYMPTOMATIC PATIENTS, Cardiovascular Medicine, NO, Contractility, Fibrosis, Internal medicine, Medicine, cardiovascular diseases, education, CLINICAL-SIGNIFICANCE, GeneralLiterature_REFERENCE(e.g.,dictionaries,encyclopedias,glossaries), RISK, education.field_of_study, Ejection fraction, business.industry, Research, CARDIOVASCULAR MAGNETIC-RESONANCE, LATE GADOLINIUM ENHANCEMENT, PREVALENCE, Hypertrophic cardiomyopathy, General Medicine, medicine.disease, Pathophysiology, Surgery, cardiovascular system, Cardiology, Myocardial fibrosis, medicine.symptom, business
Abstract

BACKGROUND: While implications of myocardial fibrosis on left ventricular (LV) function at rest have been studied in hypertrophic cardiomyopathy (HCM), the pathophysiological consequences on dynamic LV outflow tract (LVOT) gradient have so far not been investigated in detail. OBJECTIVE: To evaluate the influence of myocardial fibrosis, detected by MRI as late-gadolinium enhancement (LGE), on LVOT gradient in HCM. DESIGN: Retrospective database analysis. SETTING: A single Italian cardiomyopathies referral centre. PATIENTS: Seventy-six HCM patients with normal ejection fraction at rest. INTERVENTIONS: Patients underwent cardiac MR and performed bicycle exercise echocardiogram within a month. RESULTS: LGE was present in 54 patients (71%), ranging from 0.2% to 32.4% of LV mass. There was a weak correlation between the amount of fibrosis and LVOT gradient variation during exercise in the overall population (r=-0.243, p=0.034) and a stronger correlation in patients with obstructive HCM at rest (r=-0.524, p=0.021). Patients with an LVOT gradient increase ≥50 mm Hg during exercise had a significantly lesser extent of fibrosis than those with an increase

Published
2012

47. SHORT AND LONG-TERM PROGNOSTIC SIGNIFICANCE OF ST-SEGMENT ELEVATION IN LEAD AVR IN PATIENTS WITH NON-ST-SEGMENT ELEVATION ACUTE CORONARY SYNDROME

Author

Giovanni Melandri, Caterina Villani, Pamela Gallo, Cinzia Marrozzini, Paolo Ortolani, Tullio Palmerini, Laura Alessi, Antonio Marzocchi, Claudio Rapezzi, Angelo Branzi, Fabio Vagnarelli, Laura Cinti, Nevio Taglieri, Stefania Rosmini, Francesco Saia, Taglieri, N, Marzocchi A, Saia F, Marrozzini C, Rosmini S, Cinti L, Villani C, Alessi L, Vagnarelli F, Gallo P, Palmerini T, Melandri G, Ortolani P, Branzi A, and Rapezzi C

Subjects
medicine.medical_specialty, Acute coronary syndrome, business.industry, ACUTE CORONARY SYNDROMES, Elevation, medicine.disease, Term (time), Internal medicine, Cardiology, medicine, ST segment, ST-SEGMENT ELEVATION, In patient, PROGNOSTIC SIGNIFICANCE, business, Lead (electronics), Cardiology and Cardiovascular Medicine
Published
2011
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48. Short- and long-term prognostic significance of ST-segment elevation in lead aVR in patients with non-ST-segment elevation acute coronary syndrome

Author

Paolo Ortolani, Laura Cinti, Giuseppe Scaramuzzino, Tullio Palmerini, Cinzia Marrozzini, Antonio Marzocchi, Claudio Rapezzi, Ilaria Gallelli, Laura Alessi, Francesco Saia, Caterina Villani, Giovanni Melandri, Angelo Branzi, Fabio Vagnarelli, Nevio Taglieri, Stefania Rosmini, Taglieri N., Marzocchi A., Saia F., Marrozzini C., Palmerini T., Ortolani P., Cinti L., Rosmini S., Vagnarelli F., Alessi L., Villani C., Scaramuzzino G., Gallelli I., Melandri G., Branzi A., and Rapezzi C.

Subjects
Male, medicine.medical_specialty, Acute coronary syndrome, Time Factors, ELECTROCARDIOGRAM, Coronary Angiography, Culprit, Severity of Illness Index, NO, Diagnosis, Differential, Electrocardiography, Internal medicine, Cause of Death, medicine, ST segment, Humans, Hospital Mortality, Acute Coronary Syndrome, Aged, Retrospective Studies, ST depression, medicine.diagnostic_test, business.industry, ST elevation, ACUTE CORONARY SYNDROMES, CARDIOVASCULAR RISK, Hazard ratio, non ST segment elevation, Odds ratio, Middle Aged, medicine.disease, Prognosis, Survival Rate, Early Diagnosis, Italy, Cardiology, Disease Progression, Female, medicine.symptom, Cardiology and Cardiovascular Medicine, business, Follow-Up Studies
Abstract

We sought to evaluate the prognostic significance of ST-segment elevation (STE) in lead aVR in unselected patients with non-STE acute coronary syndrome (NSTE-ACS). We enrolled 1,042 consecutive patients with NSTE-ACS. Patients were divided into 5 groups according to the following electrocardiographic (ECG) patterns on admission: (1) normal electrocardiogram or no significant ST-T changes, (2) inverted T waves, (3) isolated ST deviation (ST depression [STD] without STE in lead aVR or transient STE), (4) STD plus STE in lead aVR, and (5) ECG confounders (pacing, right or left bundle branch block). The main angiographic end point was left main coronary artery (LM) disease as the culprit artery. Clinical end points were in-hospital and 1-year cardiovascular death defined as the composite of cardiac death, fatal stroke, and fatal bleeding. Prevalence of STD plus STE in lead aVR was 13.4%. Rates of culprit LM disease and in-hospital cardiovascular death were 8.1% and 3.8%, respectively. On multivariable analysis, patients with STD plus STE in lead aVR (group 4) showed an increased risk of culprit LM disease (odds ratio 4.72, 95% confidence interval [CI] 2.31 to 9.64, p

Published
2011

49. Prognostic significance of mean platelet volume on admission in unselected cohort of patients with non ST-segment elevation acute coronary syndrome

Author

TAGLIERI, NEVIO, ROSMINI, STEFANIA, CINTI, LAURA, ALESSI, LAURA, VAGNARELLI, FABIO, GALLO, PAMELA, BRANZI, ANGELO, RAPEZZI, CLAUDIO, MARZOCCHI, ANTONIO, Saia, F, Taglieri, N, Saia, F, Rosmini, S, Cinti, L, Alessi, L, Vagnarelli, F, Gallo, P, Branzi, A, Rapezzi, C, and Marzocchi, A

Subjects
platelet volume,ST-segment elevation, acute coronary syndrome
Published
2011

50. Prognostic significance of mean platelet volume on admission in an unselected cohort of patients with non ST-segment elevation acute coronary syndrome

Author

Fabio Vagnarelli, Nevio Taglieri, Giovanni Melandri, Cinzia Marrozzini, Stefania Rosmini, Paolo Ortolani, Tullio Palmerini, Laura Cinti, Francesco Saia, Claudio Rapezzi, Bacchi Reggiani Ml, Laura Alessi, Antonio Marzocchi, Caterina Villani, Angelo Branzi, Taglieri N., Saia F., Rapezzi C., Marrozzini C., Bacchi Reggiani M.L., Palmerini T., Ortolani P., Melandri G., Rosmini S., Cinti L., Alessi L., Vagnarelli F., Villani C., Branzi A., and Marzocchi A.

Subjects
Erythrocyte Indices, Male, Risk, Acute coronary syndrome, medicine.medical_specialty, Myocardial Infarction, 030204 cardiovascular system & hematology, ACUTE MYOCARDIAL INFARCTION, Platelet physiology, RISK FACTORS, NO, Cohort Studies, 03 medical and health sciences, Electrocardiography, 0302 clinical medicine, Predictive Value of Tests, Internal medicine, medicine, Clinical endpoint, Humans, 030212 general & internal medicine, Myocardial infarction, Mean platelet volume, Risk factor, Acute Coronary Syndrome, Prospective cohort study, Killip class, Aged, Aged, 80 and over, business.industry, Diagnostic Tests, Routine, Platelet Count, ST elevation, Hematology, Middle Aged, medicine.disease, Prognosis, Survival Analysis, Surgery, Italy, Acute Disease, Cardiology, Female, business, Biomarkers
Abstract

SummaryMean platelet volume (MPV) has been proposed as a marker of platelet reactivity and cardiovascular risk. Its prognostic significance has not been thoroughly investigated in patients with non-ST elevation acute coronary syndrome (NSTE-ACS). We included 1,041 consecutive patients with NSTE-ACS. Patients were divided in quartiles according to the MPV value on admission (fl) i.e. Q1

Published
2011

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