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2. POS1187 CATASTROPHIC ADULT ONSET STILL DISEASE: FREQUENCY, PHENOTYPE, AND VALUE OF HLH/MAS VS SOFA SCORE: INSIGHT FROM AN INTERDISCIPLINARY SINGLE CENTER STUDY

Author

Dietrich, T., primary, Bretonniere, C., additional, Darrieutort Laffite, C., additional, Piriou, N., additional, Pattier, S., additional, Graveleau, J., additional, Espitia-Thibault, A., additional, Pottier, P., additional, Agard, C., additional, Canet, E., additional, and Neel, A., additional

Published
2024
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4. Evaluating the current physicians’ knowledge and patients’ pathways for diagnosing transthyretin cardiac amyloidosis (ATTR-CM) in France: An extensive survey of diverse medical specialists

Author

Oghina, S., primary, Legallois, D., additional, Fournier, P., additional, Amara, W., additional, Sabouret, P., additional, Piriou, N., additional, Tresorier, R., additional, Andres, E., additional, Bardin, T., additional, Guignard, S., additional, Hyafil, F., additional, Labeyrie, C., additional, Toulza, O., additional, Walle, M., additional, Bouquillon, B., additional, Sauvage, C., additional, Canali, G., additional, Dubois, M., additional, Charron, P., additional, and Damy, T., additional

Published
2024
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11. P408 PREVALENCE CHARACTERISTICS AND OUTCOMES OF COVID 19 ASSOCIATED ACUTE MYOCARDITIS

Author

Palazzini, M, primary, Ammirati, E, additional, Lupi, L, additional, Giannattasio, C, additional, Soriano, F, additional, Pedrotti, P, additional, Briguglia, D, additional, Mapelli, M, additional, Campodonico, J, additional, Agostoni, P, additional, Leonardi, S, additional, Turco, A, additional, Guida, S, additional, Peretto, G, additional, Sala, S, additional, Camici, P, additional, Marzo, F, additional, Grosu, A, additional, Senni, M, additional, Turrini, F, additional, Bramerio, M, additional, Marini, M, additional, Matassini, M, additional, Rizzo, S, additional, Basso, C, additional, De Gaspari, M, additional, Hendren, N, additional, Schmidt, M, additional, Bochaton, T, additional, Piriou, N, additional, Ubarri, A, additional, Van De Heyning, C, additional, Ariza Sole, A, additional, Cannatà, A, additional, Salamanca, J, additional, Lehtonen, J, additional, Huang, F, additional, Adler, E, additional, and Metra, M, additional

Published
2023
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12. C84 OUTCOME AND MORPHO–FUNCTIONAL CHANGES ON CARDIAC MAGNETIC RESONANCE IN PATIENT WITH ACUTE MYOCARDITIS FOLLOWING MRNA COVID 19 VACCINATION

Author

Palazzini, M, primary, Ammirati, E, additional, Lupi, L, additional, Garascia, A, additional, Gentile, P, additional, Pedrotti, P, additional, Giannattasio, C, additional, Ciabatti, M, additional, Rossi, V, additional, Ruschitzka, F, additional, Uribarri, A, additional, Vecchio, C, additional, Nassiacos, D, additional, Cereda, A, additional, Tumminiello, G, additional, Piriou, N, additional, Stucchi, M, additional, Peretto, G, additional, Galasso, M, additional, Sala, S, additional, Camici, P, additional, Huang, F, additional, Ianni, U, additional, Procopio, A, additional, Saponara, G, additional, Cimaglia, P, additional, Tomasoni, D, additional, Moroni, F, additional, Turco, A, additional, Di Tano, G, additional, Bollano, E, additional, Moro, C, additional, Abbate, A, additional, Dalla Bona, R, additional, Porto, I, additional, Carugo, S, additional, Campodonico, J, additional, Pontone, G, additional, Grosu, A, additional, Adamo, M, additional, Salamanca, J, additional, Ozieransky, K, additional, Sardo Infirri, L, additional, Cannatà, A, additional, Adler, E, additional, Sinagra, G, additional, Potena, L, additional, Foà, A, additional, Metra, M, additional, and Pieroni, M, additional

Published
2023
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13. Intramyocardial transplantation of mesenchymal stromal cells for chronic myocardial ischemia and impaired left ventricular function: Results of the MESAMI 1 pilot trial

Author

Guijarro, D., Lebrin, M., Lairez, O., Bourin, P., Piriou, N., Pozzo, J., Lande, G., Berry, M., Le Tourneau, T., Cussac, D., Sensebe, L., Gross, F., Lamirault, G., Huynh, A., Manrique, A., Ruidavet, J.B., Elbaz, M., Trochu, J.N., Parini, A., Kramer, S., Galinier, M., Lemarchand, P., and Roncalli, J.

Published
2016
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14. Acute Myocarditis Associated With Desmosomal Gene Variants

Author

Ammirati, E, Raimondi, F, Piriou, N, Sardo Infirri, L, Mohiddin, S, Mazzanti, A, Shenoy, C, Cavallari, U, Imazio, M, Aquaro, G, Olivotto, I, Pedrotti, P, Sekhri, N, Van de Heyning, C, Broeckx, G, Peretto, G, Guttmann, O, Dellegrottaglie, S, Scatteia, A, Gentile, P, Merlo, M, Goldberg, R, Reyentovich, A, Sciamanna, C, Klaassen, S, Poller, W, Trankle, C, Abbate, A, Keren, A, Horowitz-Cederboim, S, Cadrin-Tourigny, J, Tadros, R, Annoni, G, Bonoldi, E, Toquet, C, Marteau, L, Probst, V, Trochu, J, Kissopoulou, A, Grosu, A, Kukavica, D, Trancuccio, A, Gil, C, Tini, G, Pedrazzini, M, Torchio, M, Sinagra, G, Gimeno, J, Bernasconi, D, Valsecchi, M, Klingel, K, Adler, E, Camici, P, Cooper, L, Ammirati E., Raimondi F., Piriou N., Sardo Infirri L., Mohiddin S. A., Mazzanti A., Shenoy C., Cavallari U. A., Imazio M., Aquaro G. D., Olivotto I., Pedrotti P., Sekhri N., Van de Heyning C. M., Broeckx G., Peretto G., Guttmann O., Dellegrottaglie S., Scatteia A., Gentile P., Merlo M., Goldberg R. I., Reyentovich A., Sciamanna C., Klaassen S., Poller W., Trankle C. R., Abbate A., Keren A., Horowitz-Cederboim S., Cadrin-Tourigny J., Tadros R., Annoni G. A., Bonoldi E., Toquet C., Marteau L., Probst V., Trochu J. N., Kissopoulou A., Grosu A., Kukavica D., Trancuccio A., Gil C., Tini G., Pedrazzini M., Torchio M., Sinagra G., Gimeno J. R., Bernasconi D., Valsecchi M. G., Klingel K., Adler E. D., Camici P. G., Cooper L. T., Ammirati, E, Raimondi, F, Piriou, N, Sardo Infirri, L, Mohiddin, S, Mazzanti, A, Shenoy, C, Cavallari, U, Imazio, M, Aquaro, G, Olivotto, I, Pedrotti, P, Sekhri, N, Van de Heyning, C, Broeckx, G, Peretto, G, Guttmann, O, Dellegrottaglie, S, Scatteia, A, Gentile, P, Merlo, M, Goldberg, R, Reyentovich, A, Sciamanna, C, Klaassen, S, Poller, W, Trankle, C, Abbate, A, Keren, A, Horowitz-Cederboim, S, Cadrin-Tourigny, J, Tadros, R, Annoni, G, Bonoldi, E, Toquet, C, Marteau, L, Probst, V, Trochu, J, Kissopoulou, A, Grosu, A, Kukavica, D, Trancuccio, A, Gil, C, Tini, G, Pedrazzini, M, Torchio, M, Sinagra, G, Gimeno, J, Bernasconi, D, Valsecchi, M, Klingel, K, Adler, E, Camici, P, Cooper, L, Ammirati E., Raimondi F., Piriou N., Sardo Infirri L., Mohiddin S. A., Mazzanti A., Shenoy C., Cavallari U. A., Imazio M., Aquaro G. D., Olivotto I., Pedrotti P., Sekhri N., Van de Heyning C. M., Broeckx G., Peretto G., Guttmann O., Dellegrottaglie S., Scatteia A., Gentile P., Merlo M., Goldberg R. I., Reyentovich A., Sciamanna C., Klaassen S., Poller W., Trankle C. R., Abbate A., Keren A., Horowitz-Cederboim S., Cadrin-Tourigny J., Tadros R., Annoni G. A., Bonoldi E., Toquet C., Marteau L., Probst V., Trochu J. N., Kissopoulou A., Grosu A., Kukavica D., Trancuccio A., Gil C., Tini G., Pedrazzini M., Torchio M., Sinagra G., Gimeno J. R., Bernasconi D., Valsecchi M. G., Klingel K., Adler E. D., Camici P. G., and Cooper L. T.

Abstract

Background: The risk of adverse cardiovascular events in patients with acute myocarditis (AM) and desmosomal gene variants (DGV) remains unknown. Objectives: The purpose of this study was to ascertain the risk of death, ventricular arrhythmias, recurrent myocarditis, and heart failure (main endpoint) in patients with AM and pathogenic or likely pathogenetic DGV. Methods: In a retrospective international study from 23 hospitals, 97 patients were included: 36 with AM and DGV (DGV[+]), 25 with AM and negative gene testing (DGV[−]), and 36 with AM without genetics testing. All patients had troponin elevation plus findings consistent with AM on histology or at cardiac magnetic resonance (CMR). In 86 patients, CMR changes in function and structure were re-assessed at follow-up. Results: In the DGV(+) AM group (88.9% DSP variants), median age was 24 years, 91.7% presented with chest pain, and median left ventricular ejection fraction (LVEF) was 56% on CMR (P = NS vs the other 2 groups). Kaplan-Meier curves demonstrated a higher risk of the main endpoint in DGV(+) AM compared with DGV(−) and without genetics testing patients (62.3% vs 17.5% vs 5.3% at 5 years, respectively; P < 0.0001), driven by myocarditis recurrence and ventricular arrhythmias. At follow-up CMR, a higher number of late gadolinium enhanced segments was found in DGV(+) AM. Conclusions: Patients with AM and evidence of DGV have a higher incidence of adverse cardiovascular events compared with patients with AM without DGV. Further prospective studies are needed to ascertain if genetic testing might improve risk stratification of patients with AM who are considered at low risk.

Published
2022

15. Prevalence, Characteristics, and Outcomes of COVID-19-Associated Acute Myocarditis

Author

Ammirati, E, Lupi, L, Palazzini, M, Hendren, N, Grodin, J, Cannistraci, C, Schmidt, M, Hekimian, G, Peretto, G, Bochaton, T, Hayek, A, Piriou, N, Leonardi, S, Guida, S, Turco, A, Sala, S, Uribarri, A, Van De Heyning, C, Mapelli, M, Campodonico, J, Pedrotti, P, Barrionuevo Sanchez, M, Ariza Sole, A, Marini, M, Matassini, M, Vourc'H, M, Cannata, A, Bromage, D, Briguglia, D, Salamanca, J, Diez-Villanueva, P, Lehtonen, J, Huang, F, Russel, S, Soriano, F, Turrini, F, Cipriani, M, Bramerio, M, Di Pasquale, M, Grosu, A, Senni, M, Farina, D, Agostoni, P, Rizzo, S, De Gaspari, M, Marzo, F, Duran, J, Adler, E, Giannattasio, C, Basso, C, Mcdonagh, T, Kerneis, M, Combes, A, Camici, P, De Lemos, J, Metra, M, Ammirati E., Lupi L., Palazzini M., Hendren N. S., Grodin J. L., Cannistraci C. V., Schmidt M., Hekimian G., Peretto G., Bochaton T., Hayek A., Piriou N., Leonardi S., Guida S., Turco A., Sala S., Uribarri A., Van De Heyning C. M., Mapelli M., Campodonico J., Pedrotti P., Barrionuevo Sanchez M. I., Ariza Sole A., Marini M., Matassini M. V., Vourc'H M., Cannata A., Bromage D. I., Briguglia D., Salamanca J., Diez-Villanueva P., Lehtonen J., Huang F., Russel S., Soriano F., Turrini F., Cipriani M., Bramerio M., Di Pasquale M., Grosu A., Senni M., Farina D., Agostoni P., Rizzo S., De Gaspari M., Marzo F., Duran J. M., Adler E. D., Giannattasio C., Basso C., McDonagh T., Kerneis M., Combes A., Camici P. G., De Lemos J. A., Metra M., Ammirati, E, Lupi, L, Palazzini, M, Hendren, N, Grodin, J, Cannistraci, C, Schmidt, M, Hekimian, G, Peretto, G, Bochaton, T, Hayek, A, Piriou, N, Leonardi, S, Guida, S, Turco, A, Sala, S, Uribarri, A, Van De Heyning, C, Mapelli, M, Campodonico, J, Pedrotti, P, Barrionuevo Sanchez, M, Ariza Sole, A, Marini, M, Matassini, M, Vourc'H, M, Cannata, A, Bromage, D, Briguglia, D, Salamanca, J, Diez-Villanueva, P, Lehtonen, J, Huang, F, Russel, S, Soriano, F, Turrini, F, Cipriani, M, Bramerio, M, Di Pasquale, M, Grosu, A, Senni, M, Farina, D, Agostoni, P, Rizzo, S, De Gaspari, M, Marzo, F, Duran, J, Adler, E, Giannattasio, C, Basso, C, Mcdonagh, T, Kerneis, M, Combes, A, Camici, P, De Lemos, J, Metra, M, Ammirati E., Lupi L., Palazzini M., Hendren N. S., Grodin J. L., Cannistraci C. V., Schmidt M., Hekimian G., Peretto G., Bochaton T., Hayek A., Piriou N., Leonardi S., Guida S., Turco A., Sala S., Uribarri A., Van De Heyning C. M., Mapelli M., Campodonico J., Pedrotti P., Barrionuevo Sanchez M. I., Ariza Sole A., Marini M., Matassini M. V., Vourc'H M., Cannata A., Bromage D. I., Briguglia D., Salamanca J., Diez-Villanueva P., Lehtonen J., Huang F., Russel S., Soriano F., Turrini F., Cipriani M., Bramerio M., Di Pasquale M., Grosu A., Senni M., Farina D., Agostoni P., Rizzo S., De Gaspari M., Marzo F., Duran J. M., Adler E. D., Giannattasio C., Basso C., McDonagh T., Kerneis M., Combes A., Camici P. G., De Lemos J. A., and Metra M.

Abstract

Background: Acute myocarditis (AM) is thought to be a rare cardiovascular complication of COVID-19, although minimal data are available beyond case reports. We aim to report the prevalence, baseline characteristics, in-hospital management, and outcomes for patients with COVID-19-associated AM on the basis of a retrospective cohort from 23 hospitals in the United States and Europe. Methods: A total of 112 patients with suspected AM from 56 963 hospitalized patients with COVID-19 were evaluated between February 1, 2020, and April 30, 2021. Inclusion criteria were hospitalization for COVID-19 and a diagnosis of AM on the basis of endomyocardial biopsy or increased troponin level plus typical signs of AM on cardiac magnetic resonance imaging. We identified 97 patients with possible AM, and among them, 54 patients with definite/probable AM supported by endomyocardial biopsy in 17 (31.5%) patients or magnetic resonance imaging in 50 (92.6%). We analyzed patient characteristics, treatments, and outcomes among all COVID-19-associated AM. Results: AM prevalence among hospitalized patients with COVID-19 was 2.4 per 1000 hospitalizations considering definite/probable and 4.1 per 1000 considering also possible AM. The median age of definite/probable cases was 38 years, and 38.9% were female. On admission, chest pain and dyspnea were the most frequent symptoms (55.5% and 53.7%, respectively). Thirty-one cases (57.4%) occurred in the absence of COVID-19-associated pneumonia. Twenty-one (38.9%) had a fulminant presentation requiring inotropic support or temporary mechanical circulatory support. The composite of in-hospital mortality or temporary mechanical circulatory support occurred in 20.4%. At 120 days, estimated mortality was 6.6%, 15.1% in patients with associated pneumonia versus 0% in patients without pneumonia (P=0.044). During hospitalization, left ventricular ejection fraction, assessed by echocardiography, improved from a median of 40% on admission to 55% at discharge

Published
2022

16. Outcome and Morphofunctional Changes on Cardiac Magnetic Resonance in Patients With Acute Myocarditis Following mRNA COVID-19 Vaccination

Author

Ammirati, E, Lupi, L, Palazzini, M, Ciabatti, M, Rossi, V, Gentile, P, Uribarri, A, Vecchio, C, Nassiacos, D, Cereda, A, Conca, C, Tumminello, G, Piriou, N, Lelarge, C, Pedrotti, P, Stucchi, M, Peretto, G, Galasso, M, Huang, F, Ianni, U, Procopio, A, Saponara, G, Cimaglia, P, Tomasoni, D, Moroni, F, Turco, A, Sala, S, Di Tano, G, Bollano, E, Moro, C, Abbate, A, Della Bona, R, Porto, I, Carugo, S, Campodonico, J, Pontone, G, Grosu, A, Bolognese, L, Salamanca, J, Diez-Villanueva, P, Ozieranski, K, Tyminska, A, Sardo Infirri, L, Bromage, D, Cannatà, A, Hong, K, Adamo, M, Quattrocchi, G, Foà, A, Potena, L, Garascia, A, Giannattasio, C, Adler, E, Sinagra, G, Ruschitzka, F, Camici, P, Metra, M, Pieroni, M, Ammirati, Enrico, Lupi, Laura, Palazzini, Matteo, Ciabatti, Michele, Rossi, Valentina A, Gentile, Piero, Uribarri, Aitor, Vecchio, Chiara R, Nassiacos, Daniele, Cereda, Alberto, Conca, Cristina, Tumminello, Gabriele, Piriou, Nicolas, Lelarge, Coline, Pedrotti, Patrizia, Stucchi, Miriam, Peretto, Giovanni, Galasso, Michele, Huang, Florent, Ianni, Umberto, Procopio, Antonio, Saponara, Gianluigi, Cimaglia, Paolo, Tomasoni, Daniela, Moroni, Francesco, Turco, Annalisa, Sala, Simone, Di Tano, Giuseppe, Bollano, Entela, Moro, Claudio, Abbate, Antonio, Della Bona, Roberta, Porto, Italo, Carugo, Stefano, Campodonico, Jeness, Pontone, Gianluca, Grosu, Aurelia, Bolognese, Leonardo, Salamanca, Jorge, Diez-Villanueva, Pablo, Ozieranski, Krzysztof, Tyminska, Agata, Sardo Infirri, Loren, Bromage, Daniel, Cannatà, Antonio, Hong, Kimberly N, Adamo, Marianna, Quattrocchi, Giuseppina, Foà, Alberto, Potena, Luciano, Garascia, Andrea, Giannattasio, Cristina, Adler, Eric D, Sinagra, Gianfranco, Ruschitzka, Frank, Camici, Paolo G, Metra, Marco, Pieroni, Maurizio, Ammirati, E, Lupi, L, Palazzini, M, Ciabatti, M, Rossi, V, Gentile, P, Uribarri, A, Vecchio, C, Nassiacos, D, Cereda, A, Conca, C, Tumminello, G, Piriou, N, Lelarge, C, Pedrotti, P, Stucchi, M, Peretto, G, Galasso, M, Huang, F, Ianni, U, Procopio, A, Saponara, G, Cimaglia, P, Tomasoni, D, Moroni, F, Turco, A, Sala, S, Di Tano, G, Bollano, E, Moro, C, Abbate, A, Della Bona, R, Porto, I, Carugo, S, Campodonico, J, Pontone, G, Grosu, A, Bolognese, L, Salamanca, J, Diez-Villanueva, P, Ozieranski, K, Tyminska, A, Sardo Infirri, L, Bromage, D, Cannatà, A, Hong, K, Adamo, M, Quattrocchi, G, Foà, A, Potena, L, Garascia, A, Giannattasio, C, Adler, E, Sinagra, G, Ruschitzka, F, Camici, P, Metra, M, Pieroni, M, Ammirati, Enrico, Lupi, Laura, Palazzini, Matteo, Ciabatti, Michele, Rossi, Valentina A, Gentile, Piero, Uribarri, Aitor, Vecchio, Chiara R, Nassiacos, Daniele, Cereda, Alberto, Conca, Cristina, Tumminello, Gabriele, Piriou, Nicolas, Lelarge, Coline, Pedrotti, Patrizia, Stucchi, Miriam, Peretto, Giovanni, Galasso, Michele, Huang, Florent, Ianni, Umberto, Procopio, Antonio, Saponara, Gianluigi, Cimaglia, Paolo, Tomasoni, Daniela, Moroni, Francesco, Turco, Annalisa, Sala, Simone, Di Tano, Giuseppe, Bollano, Entela, Moro, Claudio, Abbate, Antonio, Della Bona, Roberta, Porto, Italo, Carugo, Stefano, Campodonico, Jeness, Pontone, Gianluca, Grosu, Aurelia, Bolognese, Leonardo, Salamanca, Jorge, Diez-Villanueva, Pablo, Ozieranski, Krzysztof, Tyminska, Agata, Sardo Infirri, Loren, Bromage, Daniel, Cannatà, Antonio, Hong, Kimberly N, Adamo, Marianna, Quattrocchi, Giuseppina, Foà, Alberto, Potena, Luciano, Garascia, Andrea, Giannattasio, Cristina, Adler, Eric D, Sinagra, Gianfranco, Ruschitzka, Frank, Camici, Paolo G, Metra, Marco, and Pieroni, Maurizio

Published
2023

19. Acute myocarditis associated with desmosomal gene variants

Author

Ammirati, E, primary, Raimondi, F, additional, Piriou, N, additional, Mohiddin, S A, additional, Imazio, M, additional, Aquaro, G, additional, Olivotto, I, additional, Van De Heyning, C M, additional, Peretto, G, additional, Merlo, M, additional, Klaassen, S, additional, Poller, W, additional, Adler, E D, additional, Camici, P G, additional, and Cooper, L T, additional

Published
2022
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21. Myocardites

Author

Trochu, J.-N., Piriou, N., Toquet, C., Bressollette, C., Valleix, F., Le Tourneau, T., and Gueffet, J.-P.

Published
2012
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22. Prognostic impact of mitral valve geometry in patients with secondary mitral regurgitation: A secondary analysis of the MITRA-FR trial.

Author

Ng, P., Riche, B., Le Ruz, R., Le Carpentier, A., Samson, G., Trochu, J.-N., Mewton, N., Guérin, P., Iung, B., Messika-Zeitoun, D., Obadia, J.-F., Capoulade, R., and Piriou, N.

Abstract

Mitral valve (MV) transcatheter edge-to-edge repair (TEER) has emerged as a second line therapy on top of guidelines-directed medical treatment (GDMT) in heart failure (HF) patients with severe secondary mitral regurgitation (SMR). Following the disparate results of the COAPT and MITRA-FR pivotal trials, echocardiographic markers of LV remodeling and MR severity have been proposed to select patients most likely to benefit from MV-TEER. The characterization of MV geometry in MITRA-FR patients could be an additional tool to predict patients prognosis and may help to improve understanding of patient selection for MV-TEER. To evaluate the association between MV geometry and outcomes of HF patients with SMR in the MITRA-FR trial, as well as the impact of SMR treatment modality. Thirteen MV geometry parameters were assessed from baseline transthoracic echocardiograms in patients from the MITRA-FR trial. The prognostic impact of MV geometry parameters was studied on top of the baseline clinical variables used in the primary analysis of MITRA-FR (i.e. age, atrial fibrillation [AF], ischemic cardiomyopathy, myocardial infarction, left ventricular ejection fraction [LVEF] and effective regurgitant orifice area [EROA]). The primary endpoint was the composite of all-cause mortality or HF hospitalization (HFH) within 2 years. The effect of treatment arms (MV-TEER plus GDMT vs. GDMT alone) on this association was also evaluated. Among the 307 patients included in the pivotal trial, 272 were analyzed in the present study (135 from the GDMT group and 137 from the GDMT + MV-TEER group). Mean age was 70 ± 10, 74% male. 50% had ischemic cardiomyopathy and 34% AF. LVEF was 33 ± 7% and EROA was 31 ± 11 mm2. Among mitral geometry parameters, only MV tenting area remained independently associated with the primary endpoint (hazard ratio (HR) = 1.22 per 1 cm2, confidence interval (CI) = 1.01–1.47; P = 0.041) after multivariate analysis (Fig. 1). These findings remained consistent in both treatment arms (P > 0.20 for interaction). In HF patients with SMR included in the MITRA-FR trial, MV tenting area was independently associated with the risk of HFH or all-cause mortality, without any difference according to treatment arms. These data suggest that MV tethering provides an incremental prognostic value beyond classical echocardiographic variables, including EROA, and then should be considered in the risk stratification of patients with HF and SMR. [ABSTRACT FROM AUTHOR]

Published
2025
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23. Left ventricular myocardial work in degenerative mitral regurgitation and its impact on outcome.

Author

Turgeon, P.Y., Thierry, J., Senage, T., Stevant, D., Le Ruz, R., Bernard, Q., Piriou, N., Roussel, J.-C., and Le Tourneau, T.

Abstract

Few studies have evaluated the utility of quantifying myocardial work (MW) in degenerative mitral regurgitation (DMR) We aimed to investigate MW alteration in a large population of patients with DMR. Patients with none to severe DMR were prospectively enrolled and underwent a complete transthoracic echocardiography. Global left ventricular (LV) longitudinal strain (GLS) was assessed. Myocardial work was measured retrospectively. Patients were divided into two groups according to the severity of DMR at baseline (grade 0–2, n = 331; grade 3–4, n = 394). Major cardiovascular events were recorded and censored at the time of mitral valve surgery. A total of 725 patients (≥ 16 years, 55 ± 16 years, males 69%) were included. Patients with grade 3-4 DMR were older (61 ± 14 vs. 48 ± 17 years, P < 0.0001), more often men (73 vs. 43%, P < 0.0001), had more cardiovascular risk factors, were more symptomatic, and had higher levels of NT-pro-BNP (495 ± 972 vs. 196 ± 366, P < 0.0001). Heart rate (71 ± 12 vs. 68 ± 12, P = 0.003) and systolic BP (135 ± 18 vs. 129 ± 18, P < 0.0001) were slightly higher. Left ventricular and atrial remodeling was worse, PASP was higher (39 ± 15 vs. 27 ± 7 mmHg, P < 0.0001), LV EF (69 ± 7 vs. 66 ± 7%, P < 0.0001) and GLS (21.5 ± 3.0 vs. 20.7 ± 2.4, P = 0.0001) were higher but the forward stroke volume index (P < 0.0001) was lower. Regarding MW, patients with a grade 3–4 DMR had an increase in global work index (GWI, 2291 ± 485 vs. 2022 ± 401, P < 0.0001), global constructive work (GCW, 2626 ± 526 vs. 2533 ± 477, P < 0.0001), global positive work (GPW, 2522 ± 497 vs. 2357 ± 437, P < 0.0001), but a decrease in global wasted work (120 ± 65 vs. 124 ± 88, P < 0.0001) and global negative work (233 ± 114 vs. 330 ± 132, P < 0.0001). During follow-up (5.3 ± 3.3 years), 111 events were recorded (7 cardiovascular deaths, 67 heart failure symptoms, 31 new cases of AF, 8 strokes), and MV/Mitraclip surgery was performed in 318 (81%) patients with DMR grade 3–4, but in only 13 (4%) of patients with grade 0–2. Using the univariable Cox model, GLS was a marginal predictor of events (P = 0.04), whereas MW was a strong predictor of outcome (GWI, P < 0.0001; GCW, P < 0.0001; GPW, P < 0.0001) The application of MW to DMR highlights unique pathophysiological features and appears to be a promising tool for predicting outcome. MW could be used as part of the DMR clinical work-up to guide therapeutic management. [ABSTRACT FROM AUTHOR]

Published
2025
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24. Impact of high intensity and long duration exercise in genetic arrhythmogenic left ventricular cardiomyopathy.

Author

Jean, C., Guyomarch, B., Schmitt, S., Thollet, A., Clero, S., Guedon, A., Laprerie, A.-L., and Piriou, N.

Abstract

Arrhythmogenic left ventricular (LV) cardiomyopathy (ALVC) is a genetic disease associating extensive LV myocardial fibrosis with or without dysfunction and no or mild right ventricle (RV) involvement, at risk of life-threatening ventricular arrhythmias (VA). While high intensity (HI) and long duration (LD) exercise have been identified as risk factors of RV dysfunction and VA in arrhythmogenic RV cardiomyopathy including in asymptomatic genetic variant carriers, the impact of exercise on disease phenotype and risk is not known in ALVC. To study the relationship between practicing HI and LD exercise and the risk of developing an ALVC phenotype and its severity. In a retrospective observational study, patients with a pathogenic variant in common ALVC genes (DSP , FLNC , DES , RBM20), including asymptomatic carriers, were interviewed about their physical practice from age 7 to the time of genetic or disease diagnosis. HI and LD exercise were defined as having practiced at least 1 sport with an intensity ≥ 6 METs and ≥ 2.5 hours/week respectively. We studied the association of HI and LD exercise practice with the risk of ALVC phenotype occurrence and its severity. 114 out of 128 eligible patients were included (85 (74.6%) with a DSP variant, 22 FLNC (19.3%), 6 DES (5.3%), 1 RBM20 (0.9%)). 35 were probands (31%) and 79 relatives (69%) among 46 families. 31 (27%) had no ALVC phenotype at the time of genetic diagnosis. Mean age at diagnosis was 41 ± 18. 62 (54%) patients were women. 97 (85%) had practiced at least one sport, among which 81 (83.5%) at HI and 79 (81.4%) of LD. There were no differences in HI and LD exercise practice between probands and relatives (74.3% vs 69.6%, P = 0.6 and 74.3% vs 67.1%, P = 0.4 respectively), as well as between patients with and patients without ALVC phenotype at the time of diagnosis (74.7% vs 61.3%, P = 0.2 and 72.3% vs 61.3%, P = 0.3 respectively). Phenotypic features at diagnosis did not differ between patients with and without HI and LD practice (Table 1). Multivariate analysis adjusted on age and gender showed that HI and LD exercise were not significantly associated with an increased risk of developing an ALVC phenotype (OR [95% CI] for HI and LD exercise were 2.15 [0.63; 7.27] (P = 0.22) and 1.17 [0.35; 3.87] (P = 0.79) respectively). Practicing HI and LD exercise was not associated with an increased risk of developing an ALVC phenotype and its severity at the time of diagnosis in genetic ALVC variant carriers. [ABSTRACT FROM AUTHOR]

Published
2025
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25. The Healthcare Amyloidosis European Registry (HEAR): Study design and methods.

Author

Réant, P., Kharoubi, M., Delelis, F., Jeanneteau, J., Dagrenat, C., Bauer, F., Eicher, J.C., Bisson, A., Jobbé-Duval, A., Inamo, J., Roubille, F., Gueffet, J.-P., Seronde, M.-F., Piriou, N., Zaroui, A., Lairez, O., and Damy, T.

Abstract

Cardiac amyloidosis (CA) is a rare disease that can lead to poor quality of life, conduction disorders, arrhythmia, heart failure, and even death. Fortunately, specific treatments that can modify the natural history of the disease and the disease outcomes are now available. However, data on the prevailing patient management procedures and long-term outcomes of CA are scarce. In order to gather more information on the diagnosis and management of CA, we created the Healthcare Amyloidosis European Registry (HEAR). The registry's primary objective is to describe the demographic, clinical, biological and imaging characteristics of patients with CA. The secondary objectives are to (i) describe the different types of CA and their progression, (ii) describe the prevailing disease management procedures and any changes in these procedures, (iii) evaluate tools and quality of life questionnaires, (iv) describe the prognosis for patients with CA; (v) describe the management of CA by cardiologists, and (vi) assess hospital admissions and treatments and any changes in these factors. The HEAR will give us an opportunity to share good practice and to evaluate and optimize the quality of care for patients with CA. The HEAR is non-intervention, longitudinal, multicentre registry initiated in France, but which has been designed with a view to extension to other European countries. It includes prospective, retroprospective and retrospective cohorts of patients referred for suspected CA or with a confirmed diagnosis of CA. Since July 2021, 34 hospitals across France have joined the HEAR project. We expect to include 6500 patients in the HEAR between January 2021 and December 2027. At baseline, we use an electronic case report form to collect data on demographics, clinical, biological and imaging variables, the management of CA by cardiologists, specific treatments, quality of life, and diagnostic data. Lastly, we intend to collect in-hospital data on outcomes (deaths, cause of death, and hospital readmissions) annually. The HEAR is the first nationally representative, internationally extendable registry dedicated to suspected and confirmed cases of CA. It will provide crucial information on the prevailing aetiologies, prevalences, and CA management practices. [ABSTRACT FROM AUTHOR]

Published
2025
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32. The added value of contrast-enhanced cardiac magnetic resonance to predict positive genetic testing in clinically suspected Lamin A/C cardiomyopathy

Author

Delhommeau, P, primary, Marteau, L, additional, Kyndt, F, additional, Constant Dit Beaufils, AL, additional, Warin-Fresse, K, additional, Serfaty, JM, additional, Rousseau, O, additional, Karakachoff, M, additional, Conan, E, additional, Clero, S, additional, Le Tourneau, T, additional, Thollet, A, additional, Trochu, JN, additional, Probst, V, additional, and Piriou, N, additional

Published
2021
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34. Determinants and prognostic significance of myocardial replacement fibrosis in patients with mitral valve prolapse

Author

Constant Dit Beaufils, A., primary, Huttin, O., additional, Jobbe-Duval, A., additional, Piriou, N., additional, Senage, T., additional, Cueff, C., additional, Venner, C., additional, Mandry, D., additional, Sellal, J., additional, Capoulade, R., additional, Thollet, A., additional, Trochu, J., additional, Warin-Fresse, K., additional, Marie, P.Y., additional, Schott, J., additional, Roussel, J., additional, Serfaty, J., additional, Selton-Suty, C., additional, and Le Tourneau, T., additional

Published
2021
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37. Determinants and prognostic significance of focal myocardial fibrosis by cardiac magnetic resonance imaging in patients with mitral valve prolapse

Author

Constant Dit Beaufils, A.L, primary, Huttin, O, additional, Piriou, N, additional, Cueff, C, additional, Senage, T, additional, Marrec, M, additional, Warin-Fresse, K, additional, Banasiak, C, additional, Sellal, J.M, additional, Mandry, D, additional, Venner, C, additional, Filippetti, L, additional, Serfaty, J.M, additional, Selton-Suty, C, additional, and Le Tourneau, T, additional

Published
2020
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39. The added value of contrast-enhanced cardiac magnetic resonance to predict positive genetic testing in clinically suspected Lamin A/C cardiomyopathy

Author

Delhommeau, P, primary, Marteau, L, additional, Kyndt, F, additional, Constant Dit Beaufils, A.L, additional, Warin Fresse, K, additional, Serfaty, J.M, additional, Rousseau, O, additional, Karakachoff, M, additional, Conan, E, additional, Clero, S, additional, Le Tourneau, T, additional, Thollet, A, additional, Trochu, J.N, additional, Probst, V, additional, and Piriou, N, additional

Published
2020
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43. Familial cardiomyopathy in patients affected by acute myocarditis is strongly associated to DSP gene mutations

Author

Marteau, L., primary, Kyndt, F., additional, Toquet, C., additional, Serfaty, J.M., additional, Guijarro, D., additional, Le Gloan, L., additional, Lande, G., additional, Gourraud, J.B., additional, Warin-Fresse, K., additional, Conan, E., additional, Thollet, A., additional, Le Tourneau, T., additional, Trochu, J.N., additional, Probst, V., additional, and Piriou, N., additional

Published
2020
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46. 2423Familial cardiomyopathy in patients affected by acute myocarditis is strongly associated to DSP gene mutations

Author

Marteau, L, primary, Kyndt, F, additional, Toquet, C, additional, Serfaty, J M, additional, Guijarro, D, additional, Le Gloan, L, additional, Lande, G, additional, Gourraud, J B, additional, Warin Fresse, K, additional, Conan, E, additional, Thollet, A, additional, Le Tourneau, T, additional, Trochu, J N, additional, Probst, V, additional, and Piriou, N, additional

Published
2019
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