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3. A Systematic Analysis of the Clinical Outcome Associated with Multiple Reclassified Desmosomal Gene Variants in Arrhythmogenic Right Ventricular Cardiomyopathy Patients

Author

Nagyova, Emilia, Hoorntje, Edgar T., te Rijdt, Wouter P., Bosman, Laurens P., Syrris, Petros, Protonotarios, Alexandros, Elliott, Perry M., Tsatsopoulou, Adalena, Mestroni, Luisa, Taylor, Matthew R. G., Sinagra, Gianfranco, Merlo, Marco, Wada, Yuko, Horie, Minoru, Mogensen, Jens, Christensen, Alex H., Gerull, Brenda, Song, Lei, Yao, Yan, Fan, Siyang, Saguner, Ardan M., Duru, Firat, Koskenvuo, Juha W., Cruz Marino, Tania, Tichnell, Crystal, Judge, Daniel P., Dooijes, Dennis, Lekanne Deprez, Ronald H., Basso, Cristina, Pilichou, Kalliopi, Bauce, Barbara, Wilde, Arthur A. M., Charron, Philippe, Fressart, Véronique, van der Heijden, Jeroen F., van den Berg, Maarten P., Asselbergs, Folkert W., James, Cynthia A., Jongbloed, Jan D. H., Harakalova, Magdalena, and van Tintelen, J. Peter

Published
2023
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4. Phenotype and Clinical Outcomes in Desmin-Related Arrhythmogenic Cardiomyopathy

Author

Bermudez-Jimenez, Francisco J., Protonotarios, Alexandros, García-Hernández, Soledad, Pérez Asensio, Ana, Rampazzo, Alessandra, Zorio, Esther, Brodehl, Andreas, Arias, Miguel A., Macías-Ruiz, Rosa, Fernández-Armenta, Juan, Remior Perez, Paloma, Muñoz-Esparza, Carmen, Pilichou, Kalliopi, Bauce, Barbara, Merino, Jose L., Moliner-Abós, Carlos, Ochoa, Juan P., Barriales-Villa, Roberto, Garcia-Pavia, Pablo, Lopes, Luis R., Syrris, Petros, Corrado, Domenico, Elliott, Perry M., McKenna, William J., and Jimenez-Jaimez, Juan

Published
2024
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5. Alpha kinase 3 signaling at the M-band maintains sarcomere integrity and proteostasis in striated muscle

Author

McNamara, James W., Parker, Benjamin L., Voges, Holly K., Mehdiabadi, Neda R., Bolk, Francesca, Ahmad, Feroz, Chung, Jin D., Charitakis, Natalie, Molendijk, Jeffrey, Zech, Antonia T. L., Lal, Sean, Ramialison, Mirana, Karavendzas, Kathy, Pointer, Hayley L., Syrris, Petros, Lopes, Luis R., Elliott, Perry M., Lynch, Gordon S., Mills, Richard J., Hudson, James E., Watt, Kevin I., Porrello, Enzo R., and Elliott, David A.

Published
2023
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7. Abstract 17689: Distal Ventricular Pacing for Drug-Refractory Mid-Cavity Obstructive Hypertrophic Cardiomyopathy: A Randomized, Placebo-Controlled Trial of Personalized Pacing

Author

Malcolmson, James, Hughes, Rebecca, Husselbury, Tim, Khan, Kamran, Learoyd, Annastazia E, Lees, Martin, Wicks, Eleanor, Smith, Jamie, Simms, Alexander, Moon, James, Lopes, Luis, O’Mahony, Constantinos, Sekhri, Neha, Elliott, Perry M, Petersen, Steffen E, Dhinoja, Mehul B, and Mohiddin, Saidi A

Published
2023
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11. Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy

Author

Norrish, Gabrielle, Cleary, Aoife, Field, Ella, Cervi, Elena, Boleti, Olga, Ziółkowska, Lidia, Olivotto, Iacopo, Khraiche, Diala, Limongelli, Giuseppe, Anastasakis, Aris, Weintraub, Robert, Biagini, Elena, Ragni, Luca, Prendiville, Terence, Duignan, Sophie, McLeod, Karen, Ilina, Maria, Fernandez, Adrian, Marrone, Chiara, Bökenkamp, Regina, Baban, Anwar, Kubus, Peter, Daubeney, Piers E.F., Sarquella-Brugada, Georgia, Cesar, Sergi, Klaassen, Sabine, Ojala, Tiina H., Bhole, Vinay, Medrano, Constancio, Uzun, Orhan, Brown, Elspeth, Gran, Ferran, Sinagra, Gianfranco, Castro, Francisco J., Stuart, Graham, Yamazawa, Hirokuni, Barriales-Villa, Roberto, Garcia-Guereta, Luis, Adwani, Satish, Linter, Katie, Bharucha, Tara, Gonzales-Lopez, Esther, Siles, Ana, Rasmussen, Torsten B., Calcagnino, Margherita, Jones, Caroline B., De Wilde, Hans, Kubo, Toru, Felice, Tiziana, Popoiu, Anca, Mogensen, Jens, Mathur, Sujeev, Centeno, Fernando, Reinhardt, Zdenka, Schouvey, Sylvie, Elliott, Perry M., and Kaski, Juan Pablo

Published
2022
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14. Maximal Wall Thickness Measurement in Hypertrophic Cardiomyopathy: Biomarker Variability and its Impact on Clinical Care

Author

Captur, Gabriella, Manisty, Charlotte H., Raman, Betty, Marchi, Alberto, Wong, Timothy C., Ariga, Rina, Bhuva, Anish, Ormondroyd, Elizabeth, Lobascio, Ilaria, Camaioni, Claudia, Loizos, Savvas, Bonsu-Ofori, Jenade, Turer, Aslan, Zaha, Vlad G., Augutsto, João B., Davies, Rhodri H., Taylor, Andrew J., Nasis, Arthur, Al-Mallah, Mouaz H., Valentin, Sinitsyn, Perez de Arenaza, Diego, Patel, Vimal, Westwood, Mark, Petersen, Steffen E., Li, Chunming, Tang, Lijun, Nakamori, Shiro, Nezafat, Reza, Kwong, Raymond Y., Ho, Carolyn Y., Fraser, Alan G., Watkins, Hugh, Elliott, Perry M., Neubauer, Stefan, Lloyd, Guy, Olivotto, Iacopo, Nihoyannopoulos, Petros, and Moon, James C.

Published
2021
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15. The genetic architecture of Plakophilin 2 cardiomyopathy

Author

Dries, Annika M., Kirillova, Anna, Reuter, Chloe M., Garcia, John, Zouk, Hana, Hawley, Megan, Murray, Brittney, Tichnell, Crystal, Pilichou, Kalliopi, Protonotarios, Alexandros, Medeiros-Domingo, Argelia, Kelly, Melissa A., Baras, Aris, Ingles, Jodie, Semsarian, Christopher, Bauce, Barbara, Celeghin, Rudy, Basso, Cristina, Jongbloed, Jan D.H., Nussbaum, Robert L., Funke, Birgit, Cerrone, Marina, Mestroni, Luisa, Taylor, Matthew R.G., Sinagra, Gianfranco, Merlo, Marco, Saguner, Ardan M., Elliott, Perry M., Syrris, Petros, van Tintelen, J. Peter, James, Cynthia A., Haggerty, Christopher M., and Parikh, Victoria N.

Published
2021
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17. The Novel Desmin Variant p.Leu115Ile Is Associated With a Unique Form of Biventricular Arrhythmogenic Cardiomyopathy

Author

Protonotarios, Alexandros, Brodehl, Andreas, Asimaki, Angeliki, Jager, Joanna, Quinn, Ellie, Stanasiuk, Caroline, Ratnavadivel, Sandra, Futema, Marta, Akhtar, Mohammed M., Gossios, Thomas D., Ashworth, Michael, Savvatis, Konstantinos, Walhorn, Volker, Anselmetti, Dario, Elliott, Perry M., Syrris, Petros, Milting, Hendrik, and Lopes, Luis R.

Published
2021
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21. The arrhythmic substrate of hypertrophic cardiomyopathy using ECG imaging.

Author

Ji-Jian Chow, Leong, Kevin M. W., Shun-Shin, Matthew, Jones, Sian, Guttmann, Oliver P., Mohiddin, Saidi A., Lambiase, Pier, Elliott, Perry M., Ormerod, Julian O. M., Koa-Wing, Michael, Lefroy, David, Phang Boon Lim, Linton, Nicholas W. F., Fu Siong Ng, Qureshi, Norman A., Whinnett, Zachary I., Peters, Nicholas S., Francis, Darrel P., Varnava, Amanda M., and Kanagaratnam, Prapa

Subjects
VENTRICULAR arrhythmia, VENTRICULAR fibrillation, HYPERTROPHIC cardiomyopathy, VENTRICULAR tachycardia, IMPLANTABLE cardioverter-defibrillators
Abstract

Introduction: Patients with hypertrophic cardiomyopathy (HCM) are at risk for lethal ventricular arrhythmia, but the electrophysiological substrate behind this is not well-understood. We used non-invasive electrocardiographic imaging to characterize patients with HCM, including cardiac arrest survivors. Methods: HCM patients surviving ventricular fibrillation or hemodynamically unstable ventricular tachycardia (n = 17) were compared to HCM patients without a personal history of potentially lethal arrhythmia (n = 20) and a pooled control group with structurally normal hearts. Subjects underwent exercise testing by non-invasive electrocardiographic imaging to estimate epicardial electrophysiology. Results: Visual inspection of reconstructed epicardial HCM maps revealed isolated patches of late activation time (AT), prolonged activation-recovery intervals (ARIs), as well as reversal of apico-basal trends in T-wave inversion and ARI compared to controls (p < 0.005 for all). AT and ARI were compared between groups. The pooled HCM group had longer mean AT (60.1 ms vs. 52.2 ms, p < 0.001), activation dispersion (55.2 ms vs. 48.6 ms, p = 0.026), and mean ARI (227 ms vs. 217 ms, p = 0.016) than structurally normal heart controls. HCM ventricular arrhythmia survivors could be differentiated from HCM patients without a personal history of life-threatening arrhythmia by longer mean AT (63.2 ms vs. 57.4 ms, p = 0.007), steeper activation gradients (0.45 ms/mm vs. 0.36 ms/mm, p = 0.011), and longer mean ARI (234.0 ms vs. 221.4 ms, p = 0.026). A logistic regression model including whole heart mean activation time and activation recovery interval could identify ventricular arrhythmia survivors from the HCM cohort, producing a C statistic of 0.76 (95% confidence interval 0.72-0.81), with an optimal sensitivity of 78.6% and a specificity of 79.8%. Discussion: The HCM epicardial electrotype is characterized by delayed, dispersed conduction and prolonged, dispersed activation-recovery intervals. Combination of electrophysiologic measures with logistic regression can improve differentiation over single variables. Future studies could test such models prospectively for risk stratification of sudden death due to HCM. [ABSTRACT FROM AUTHOR]

Published
2024
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22. Genetics of hypertrophic cardiomyopathy: established and emerging implications for clinical practice.

Author

Lopes, Luis R, Ho, Carolyn Y, and Elliott, Perry M

Subjects
HYPERTROPHIC cardiomyopathy, GENETIC testing, GENETIC variation, MEDICAL screening, CLINICAL medicine
Abstract

Pathogenic variation in genes encoding proteins of the cardiac sarcomere is responsible for 30%–40% of cases of hypertrophic cardiomyopathy. The main clinical utility of genetic testing is to provide diagnostic confirmation and facilitation of family screening. It also assists in the detection of aetiologies, which require distinct monitoring and treatment approaches. Other clinical applications, including the use of genetic information to inform risk prediction models, have been limited by the challenge of establishing robust genotype–phenotype correlations with actionable consequences, but new data on the interaction between rare and common genetic variation, as well as the emergence of therapies targeting disease-specific pathogenic mechanisms, herald a new era for genetic testing in routine practice. [ABSTRACT FROM AUTHOR]

Published
2024
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23. A Proof of Principle 2D Spatial Proteome Mapping Analysis Reveals Distinct Regional Differences in the Cardiac Proteome.

Author

Heywood, Wendy E., Searle, Jon, Collis, Richard, Doykov, Ivan, Ashworth, Michael, Sebire, Neil, Bamber, Andrew, Gautel, Mathias, Eaton, Simon, Coats, Caroline J., Elliott, Perry M., and Mills, Kevin

Subjects
ARRHYTHMOGENIC right ventricular dysplasia, MITRAL valve, PROTEOMICS, MITOCHONDRIAL proteins, TRICUSPID valve
Abstract

Proteomics studies often explore phenotypic differences between whole organs and systems. Within the heart, more subtle variation exists. To date, differences in the underlying proteome are only described between whole cardiac chambers. This study, using the bovine heart as a model, investigates inter-regional differences and assesses the feasibility of measuring detailed, cross-tissue variance in the cardiac proteome. Using a bovine heart, we created a two-dimensional section through a plane going through two chambers. This plane was further sectioned into 4 × 4 mm cubes and analysed using label-free proteomics. We identified three distinct proteomes. When mapped to the extracted sections, the proteomes corresponded largely to the outer wall of the right ventricle and secondly to the outer wall of the left ventricle, right atrial appendage, tricuspid and mitral valves, modulator band, and parts of the left atrium. The third separate proteome corresponded to the inner walls of the left and right ventricles, septum, and left atrial appendage. Differential protein abundancies indicated differences in energy metabolism between regions. Data analyses of the mitochondrial proteins revealed a variable pattern of abundances of complexes I–V between the proteomes, indicating differences in the bioenergetics of the different cardiac sub-proteomes. Mapping of disease-associated proteins interestingly showed desmoglein-2, for which defects in this protein are known to cause Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy, which was present predominantly in the outer wall of the left ventricle. This study highlights that organs can have variable proteomes that do not necessarily correspond to anatomical features. [ABSTRACT FROM AUTHOR]

Published
2024
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24. Endomyocardial biopsy: safety and prognostic utility in paediatric and adult myocarditis in the European Society of Cardiology EURObservational Research Programme Cardiomyopathy and Myocarditis Long-Term Registry.

Author

Caforio, Alida L P, Kaski, Juan P, Gimeno, Juan R, Elliott, Perry M, Laroche, Cecile, Tavazzi, Luigi, Tendera, Michal, Fu, Michael, Sala, Simone, Seferovic, Petar M, Heliö, Tiina, Calò, Leonardo, Blagova, Olga, Amin, Ahmad, Kindermann, Ingrid, Sinagra, Gianfranco, Frustaci, Andrea, Bonnet, Daniel, Charron, Philippe, and Maggioni, Aldo P

Subjects
VENTRICULAR ejection fraction, IMPLANTABLE cardioverter-defibrillators, CARDIAC magnetic resonance imaging, MYOCARDITIS, CARDIAC research, HEART assist devices, CHILD patients
Abstract

Background and Aims Contemporary multicentre data on clinical and diagnostic spectrum and outcome in myocarditis are limited. Study aims were to describe baseline features, 1-year follow-up, and baseline predictors of outcome in clinically suspected or biopsy-proven myocarditis (2013 European Society of Cardiology criteria) in adult and paediatric patients from the EURObservational Research Programme Cardiomyopathy and Myocarditis Long-Term Registry. Methods Five hundred eighty-one (68.0% male) patients, 493 adults, median age 38 (27–52) years, and 88 children, aged 8 (3–13) years, were divided into 3 groups: Group 1 (n = 233), clinically suspected myocarditis with abnormal cardiac magnetic resonance; Group 2 (n = 222), biopsy-proven myocarditis; and Group 3 (n = 126) clinically suspected myocarditis with normal or inconclusive or no cardiac magnetic resonance. Baseline features were analysed overall, in adults vs. children, and among groups. One-year outcome events included death/heart transplantation, ventricular assist device (VAD) or implantable cardioverter defibrillator (ICD) implantation, and hospitalization for cardiac causes. Results Endomyocardial biopsy, mainly right ventricular, had a similarly low complication rate in children and adults (4.7% vs. 4.9%, P = NS), with no procedure-related death. A classical myocarditis pattern on cardiac magnetic resonance was found in 31.3% of children and in 57.9% of adults with biopsy-proven myocarditis (P <.001). At 1-year follow-up, 11/410 patients (2.7%) died, 7 (1.7%) received a heart transplant, 3 underwent VAD (0.7%), and 16 (3.9%) underwent ICD implantation. Independent predictors at diagnosis of death or heart transplantation or hospitalization or VAD implantation or ICD implantation at 1-year follow-up were lower left ventricular ejection fraction and the need for immunosuppressants for new myocarditis diagnosis refractory to non–aetiology-driven therapy. Conclusions Endomyocardial biopsy was safe, and cardiac magnetic resonance using Lake Louise criteria was less sensitive, particularly in children. Virus-negative lymphocytic myocarditis was predominant both in children and adults, and use of immunosuppressive treatments was low. Lower left ventricular ejection fraction and the need for immunosuppressants at diagnosis were independent predictors of unfavourable outcome events at 1 year. [ABSTRACT FROM AUTHOR]

Published
2024
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25. Distal Ventricular Pacing for Drug-Refractory Mid- Cavity Obstructive Hypertrophic Cardiomyopathy: A Randomized, Placebo-Controlled Trial of Personalized Pacing.

Author

Malcolmson, James W., Hughes, Rebecca K., Husselbury, Tim, Khan, Kamran, Learoyd, Annastazia E., Lees, Martin, Wicks, Eleanor C., Smith, Jamie, Simms, Alexander D., Moon, James C., Lopes, Luis R., O’Mahony, Constantinos, Sekhri, Neha, Elliott, Perry M., Petersen, Steffen E., Dhinoja, Mehul B., and Mohiddin, Saidi A.

Abstract

BACKGROUND: Patients with refractory, symptomatic left ventricular (LV) mid-cavity obstructive (LVMCO) hypertrophic cardiomyopathy have few therapeutic options. Right ventricular pacing is associated with modest hemodynamic and symptomatic improvement, and LV pacing pilot data suggest therapeutic potential. We hypothesized that site-specific pacing would reduce LVMCO gradients and improve symptoms. METHODS: Patients with symptomatic-drug-refractory LVMCO were recruited for a randomized, blinded trial of personalized prescription of pacing (PPoP). Multiple LV and apical right ventricular pacing sites were assessed during an invasive hemodynamic study of multisite pacing. Patient-specific pacing-site and atrioventricular delays, defining PPoP, were selected on the basis of LVMCO gradient reduction and acceptable pacing parameters. Patients were randomized to 6 months of active PPoP or backup pacing in a crossover design. The primary outcome examined invasive gradient change with best-site pacing. Secondary outcomes assessed quality of life and exercise following randomization to PPoP. RESULTS: A total of 17 patients were recruited; 16 of whom met primary end points. Baseline New York Heart Association was 3±0.6, despite optimal medical therapy. Hemodynamic effects were assessed during pacing at the right ventricular apex and at a mean of 8 LV sites. The gradients in all 16 patients fell with pacing, with maximum gradient reduction achieved via LV pacing in 14 (88%) patients and right ventricular apex in 2. The mean baseline gradient of 80±29 mm Hg fell to 31±21 mm Hg with best-site pacing, a 60% reduction (P<0.0001). One cardiac vein perforation occurred in 1 case, and 15 subjects entered crossover; 2 withdrawals occurred during crossover. Of the 13 completing crossover, 9 (69%) chose active pacing in PPoP configuration as preferred setting. PPoP was associated with improved 6-minute walking test performance (328.5±99.9 versus 285.8±105.5 m; P=0.018); other outcome measures also indicated benefit with PPoP. CONCLUSIONS: In a randomized placebo-controlled trial, PPoP reduces obstruction and improves exercise performance in severely symptomatic patients with LVMCO. [ABSTRACT FROM AUTHOR]

Published
2024
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26. The p.(Cys150Tyr) variant in CSRP3 is associated with late-onset hypertrophic cardiomyopathy in heterozygous individuals

Author

Salazar-Mendiguchía, Joel, Barriales-Villa, Roberto, Lopes, Luis R., Ochoa, Juan P., Rodríguez-Vilela, Alejandro, Palomino-Doza, Julián, Larrañaga-Moreira, José M., Cicerchia, Marcos, Cárdenas-Reyes, Ivonne, García-Giustiniani, Diego, Brögger, Noël, Fernández, Germán, García, Soledad, Santiago, Lisi, Vélez, Paula, Ortiz-Genga, Martín, Elliott, Perry M., and Monserrat, Lorenzo

Published
2020
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27. DPD Quantification in Cardiac Amyloidosis: A Novel Imaging Biomarker

Author

Scully, Paul R., Morris, Elizabeth, Patel, Kush P., Treibel, Thomas A., Burniston, Maria, Klotz, Ernst, Newton, James D., Sabharwal, Nikant, Kelion, Andrew, Manisty, Charlotte, Kennon, Simon, Ozkor, Muhiddin, Mullen, Michael, Hartman, Neil, Elliott, Perry M., Pugliese, Francesca, Hawkins, Philip N., Moon, James C., and Menezes, Leon J.

Published
2020
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28. Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy

Author

Norrish, Gabrielle, Ding, Tao, Field, Ella, Cervi, Elena, Ziółkowska, Lidia, Olivotto, Iacopo, Khraiche, Diala, Limongelli, Giuseppe, Anastasakis, Aris, Weintraub, Robert, Biagini, Elena, Ragni, Luca, Prendiville, Terrence, Duignan, Sophie, McLeod, Karen, Ilina, Maria, Fernández, Adrián, Marrone, Chiara, Bökenkamp, Regina, Baban, Anwar, Kubus, Peter, Daubeney, Piers E.F., Sarquella-Brugada, Georgia, Cesar, Sergi, Klaassen, Sabine, Ojala, Tiina H., Bhole, Vinay, Medrano, Constancio, Uzun, Orhan, Brown, Elspeth, Gran, Ferran, Sinagra, Gianfranco, Castro, Francisco J., Stuart, Graham, Vignati, Gabriele, Yamazawa, Hirokuni, Barriales-Villa, Roberto, Garcia-Guereta, Luis, Adwani, Satish, Linter, Katie, Bharucha, Tara, Garcia-Pavia, Pablo, Siles, Ana, Rasmussen, Torsten B., Calcagnino, Margherita, Jones, Caroline B., De Wilde, Hans, Kubo, Toru, Felice, Tiziana, Popoiu, Anca, Mogensen, Jens, Mathur, Sujeev, Centeno, Fernando, Reinhardt, Zdenka, Schouvey, Sylvie, O’Mahony, Costas, Omar, Rumana Z., Elliott, Perry M., and Kaski, Juan Pablo

Published
2022
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34. Formin Homology 2 Domain Containing 3 (FHOD3) Is a Genetic Basis for Hypertrophic Cardiomyopathy

Author

Ochoa, Juan Pablo, Sabater-Molina, María, García-Pinilla, José Manuel, Mogensen, Jens, Restrepo-Córdoba, Alejandra, Palomino-Doza, Julián, Villacorta, Eduardo, Martinez-Moreno, Marina, Ramos-Maqueda, Javier, Zorio, Esther, Peña-Peña, Maria L., García-Granja, Pablo E., Rodríguez-Palomares, José F., Cárdenas-Reyes, Ivonne J., de la Torre-Carpente, María M., Bautista-Pavés, Alicia, Akhtar, Mohammed M., Cicerchia, Marcos N., Bilbao-Quesada, Raquel, Mogollón-Jimenez, Maria Victoria, Salazar-Mendiguchía, Joel, Mesa Latorre, José M., Arnaez, Blanca, Olavarri-Miguel, Ivan, Fuentes-Cañamero, María E., Lamounier, Arsonval, Jr, Pérez Ruiz, José María, Climent-Payá, Vicente, Pérez-Sanchez, Inmaculada, Trujillo-Quintero, Juan P., Lopes, Luis R., Repáraz-Andrade, Alfredo, Marín-Iglesias, Rosario, Rodriguez-Vilela, Alejandro, Sandín-Fuentes, María, Garrote, Jose A., Cortel-Fuster, Alejandro, Lopez-Garrido, Miguel, Fontalba-Romero, Ana, Ripoll-Vera, Tomás, Llano-Rivas, Isabel, Fernandez-Fernandez, Xusto, Isidoro-García, María, Garcia-Giustiniani, Diego, Barriales-Villa, Roberto, Ortiz-Genga, Martín, García-Pavía, Pablo, Elliott, Perry M., Gimeno, Juan R., and Monserrat, Lorenzo

Published
2018
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35. Dilated Cardiomyopathy Due to BLC2-Associated Athanogene 3 (BAG3) Mutations

Author

Eiskjær, Hans, Barriales, Roberto, Fernández Fernández, Xusto, Cicerchia, Marcos, Monserrat, Lorenzo, Ochoa, Juan Pablo, Salazar-Mendiguchia, Joel, Mogollón, Maria Victoria, Ripoll, Tomás, Charron, Philippe, Richard, Pascale, Villard, Eric, Palomino Doza, Julian, Fontalba, Ana, Alonso-Pulpón, Luis, Cobo-Marcos, Marta, Domínguez, Fernando, Garcia-Pavia, Pablo, Gómez-Bueno, Manuel, González-López, Esther, Hernández-Hernández, Aitor, Hernández-Pérez, Francisco José, López-Sainz, Ángela, Restrepo-Córdoba, Alejandra, Segovia-Cubero, Javier, Toro, Rocio, de Gonzalo-Calvo, David, Rosa Longobardo, Félix, Limeres, Javier, Rodriguez-Palomares, Jose F., Garcia-Pinilla, Jose Manuel, López-Garrido, Miguel A., Jiménez-Jaimez, Juan, Garcia-Medina, Dolores, Rangel Sousa, Diego, Peña, Maria Luisa, Mogensen, Jens, Morris-Hey, Thomas, Barton, Paul J., Cook, Stuart A., Midwinter, William, Roberts, Angharad M., Ware, James S., Walsh, Roddy, Akhtar, Mohammed, Elliott, Perry M., Rocha-Lopes, Luis, Savvatis, Konstantinos, Syrris, Petros, Michalak, Ewa, Ploski, Rafal, Sobieszczanska-Malek, Malgorzata, Bilińska, Zofia, Pankuweit, Sabine, Asselbergs, Folkert, Baas, Annette, Dooijes, Dennis, Sammani, Arjan, Cuenca, Sofía, Toro, Rocío, Barriales-Villa, Roberto, Franaszczyk, Maria, Coronado-Albi, Maria José, Rangel-Sousa, Diego, Jiménez-Jáimez, Juan, Garcia-Pinilla, José Manuel, Ripoll-Vera, Tomás, Mogollón-Jiménez, Maria Victoria, Fontalba-Romero, Ana, Palomino-Doza, Julian, Salas, Clara, Hey, Thomas Morris, and Elliott, Perry

Published
2018
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37. Clinical Phenotypes and Prognosis of Dilated Cardiomyopathy Caused by Truncating Variants in the TTN Gene

Author

Akhtar, Mohammed Majid, Lorenzini, Massimiliano, Cicerchia, Marcos, Ochoa, Juan Pablo, Hey, Thomas Morris, Sabater Molina, Maria, Restrepo-Cordoba, Maria Alejandra, Dal Ferro, Matteo, Stolfo, Davide, Johnson, Renee, Larrañaga-Moreira, José M., Robles-Mezcua, Ainhoa, Rodriguez-Palomares, Jose F., Casas, Guillem, Peña-Peña, Maria Luisa, Lopes, Luis Rocha, Gallego-Delgado, Maria, Franaszczyk, Maria, Laucey, Gemma, Rangel-Sousa, Diego, Basurte, Mayte, Palomino-Doza, Julian, Villacorta, Eduardo, Bilinska, Zofia, Limeres Freire, Javier, Garcia Pinilla, José M., Barriales-Villa, Roberto, Fatkin, Diane, Sinagra, Gianfranco, Garcia-Pavia, Pablo, Gimeno, Juan R., Mogensen, Jens, Monserrat, Lorenzo, and Elliott, Perry M.

Published
2020
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38. European Heart Journal: a call to action.

Author

Crea, Filippo, Badimon, Lina, Berry, Colin, Caterina, Raffaele De, Elliott, Perry M, Hatala, Robert, Libby, Peter, Linde, Cecilia, and Tybjærg-Hansen, Anne

Subjects
SCIENTIFIC knowledge, DRUG side effects, MEDICAL sciences, GENOME-wide association studies, COVID-19 pandemic, CARDIOVASCULAR diseases
Abstract

The European Heart Journal (EHJ) aims to reduce the global impact of cardiovascular disease (CVD) through the publication of cutting-edge research. The journal disseminates new knowledge through original articles and promotes better clinical practice through reviews, consensus documents, and guidelines. EHJ encourages researchers to submit their best work in translational, population, and clinical science, with a focus on new therapeutic targets, novel discoveries in population science, and high-quality studies examining the cardiovascular system. The journal maintains high standards and collaborates with experts in the field to ensure informative and impactful publications. EHJ also emphasizes the importance of personalized approaches to healthcare and the application of artificial intelligence in cardiovascular research. The journal is committed to transparency, data-sharing, and involving diverse perspectives in research. [Extracted from the article]

Published
2024
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40. Corrigendum to ‘Syncope in hypertrophic cardiomyopathy (part II): An expert consensus statement on the diagnosis and management’ [International Journal of Cardiology, 2023, 41:180–186]

Author

Brignole, Michele, Cecchi, Franco, Anastasakis, Aris, Crotti, Lia, Deharo, Jean Claude, Elliott, Perry M., Fedorowski, Artur, Kaski, Juan Pablo, Limongelli, Giuseppe, Maron, Martin S., Olivotto, Iacopo, Ommen, Steve R., Parati, Gianfranco, Shen, Win, Ungar, Andrea, and Wilde, Arthur

Published
2024
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43. Evidence From Family Studies for Autoimmunity in Arrhythmogenic Right Ventricular Cardiomyopathy: Associations of Circulating Anti-Heart and Anti-Intercalated Disk Autoantibodies With Disease Severity and Family History

Author

Caforio, Alida L.P., Re, Federica, Avella, Andrea, Marcolongo, Renzo, Baratta, Pasquale, Seguso, Mara, Gallo, Nicoletta, Plebani, Mario, Izquierdo-Bajo, Alvaro, Cheng, Chun-Yan, Syrris, Petros, Elliott, Perry M., d’Amati, Giulia, Thiene, Gaetano, Basso, Cristina, Gregori, Dario, Iliceto, Sabino, and Zachara, Elisabetta

Published
2020
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46. Mavacamten: a first-in-class myosin inhibitor for obstructive hypertrophic cardiomyopathy.

Author

Braunwald, Eugene, Saberi, Sara, Abraham, Theodore P, Elliott, Perry M, and Olivotto, Iacopo

Subjects
VENTRICULAR outflow obstruction, HYPERTROPHIC cardiomyopathy, MYOSIN, PATIENT reported outcome measures, AEROBIC capacity
Abstract

Mavacamten is a first-in-class, targeted, cardiac-specific myosin inhibitor approved by the US Food and Drug Administration for the treatment of adults with symptomatic New York Heart Association Classes II and III obstructive hypertrophic cardiomyopathy (oHCM). Mavacamten was developed to target the hyper-contractile phenotype, which plays a critical role in the pathophysiology of the disease. In Phase 2 and 3 clinical trials, mavacamten was well tolerated, reduced left ventricular outflow tract gradients, improved exercise capacity and symptoms, and was associated with improvements in other clinically relevant parameters, such as patient-reported outcomes and circulating biomarkers. In addition, treatment with mavacamten was associated with evidence of favourable cardiac remodelling in multi-modality imaging studies. Mavacamten substantially reduced guideline eligibility for septal reduction therapy candidates with oHCM and drug-refractory symptoms. In this article, the available efficacy and safety data from completed and ongoing clinical studies of mavacamten in patients with symptomatic oHCM are reviewed. Longer term extension studies may help address questions related to the positioning of mavacamten in current oHCM management algorithms, interactions with background therapy, as well as the potential for disease modification beyond symptomatic relief of left ventricular outflow tract obstruction. [ABSTRACT FROM AUTHOR]

Published
2023
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47. Performance of the PRIMaCY sudden death risk prediction model for childhood hypertrophic cardiomyopathy: implications for implantable cardioverter-defibrillator decision-making.

Author

Norrish, Gabrielle, Protonotarios, Alexandros, Stec, Maria, Boleti, Olga, Field, Ella, Cervi, Elena, Elliott, Perry M, and Kaski, Juan P

Abstract

Aims The validated HCM Risk-Kids model provides accurate individualized estimates of sudden cardiac death risk in children with hypertrophic cardiomyopathy (HCM). A second validated model, PRIMaCY, also provides individualized estimates of risk, but its performance and clinical impact has not been independently investigated. The aim of this study was to investigate the clinical impact of using the PRIMaCY sudden cardiac death (SCD) risk model in childhood HCM. Methods and results The estimated 5-year SCD risk was calculated for children meeting diagnostic criteria for HCM in a large single-centre cohort using PRIMaCY (clinical and genetic) and HCM Risk-Kids model, and model performance was assessed. Three hundred one patients [median age 10 (interquartile range 4–14)] were followed up for an average of 4.9 (±3.8) years, during which 30 (10.0%) reached the SCD or equivalent event endpoint. Harrell's C -statistic for the clinical and genetic models was 0.66 [95% confidence interval (CI) 0.52–0.8] and 0.66 (95% CI 0.54–0.80) with a calibration slope of 0.19 (95% CI 0.04–0.54) and 0.26 (95% CI −0.03–0.62), respectively. The number needed to treat to potentially treat one life-threatening arrhythmia for the PRIMaCY clinical, PRIMaCY genetic, and HCM Risk-Kids models was 13.7, 14.5, and 9.4, respectively. Conclusion Although PRIMaCY has a similar discriminatory ability to that reported for HCM Risk-Kids, estimated risk estimates did not correlate well with observed risk. A higher proportion of patients met implantable cardioverter-defibrillator thresholds using PRIMaCY model compared with HCM Risk-Kids. This has important clinical implications as these patients will be exposed to a lifetime risk of complications and inappropriate therapies. [ABSTRACT FROM AUTHOR]

Published
2023
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48. 2023 ESC Guidelines for the management of cardiomyopathies: Developed by the task force on the management of cardiomyopathies of the European Society of Cardiology (ESC).

Author

Arbelo, Elena, Protonotarios, Alexandros, Gimeno, Juan R, Arbustini, Eloisa, Barriales-Villa, Roberto, Basso, Cristina, Bezzina, Connie R, Biagini, Elena, Blom, Nico A, Boer, Rudolf A de, Winter, Tim De, Elliott, Perry M, Flather, Marcus, Garcia-Pavia, Pablo, Haugaa, Kristina H, Ingles, Jodie, Jurcut, Ruxandra Oana, Klaassen, Sabine, Limongelli, Giuseppe, and Loeys, Bart

Subjects
VENTRICULAR outflow obstruction, MELAS syndrome, CARDIOMYOPATHIES, TAKOTSUBO cardiomyopathy, ARRHYTHMOGENIC right ventricular dysplasia, SARS-CoV-2, TASK forces
Abstract

The aim is to provide healthcare professionals with a practical diagnostic and treatment framework for patients of all ages and, as an increasing number of patients have a known genetic basis for their disease, the guideline also considers the implications of a diagnosis for families and provides advice on reproduction and contraception. Keywords: Guidelines; Arrhythmia; Arrhythmogenic right ventricular cardiomyopathy; Cardiomyopathies; Diagnosis; Dilated cardiomyopathy; Genetics; Genetic counselling; Genetic testing; Hypertrophic cardiomyopathy; Implantable cardioverter defibrillator; Management; Multimodality imaging; Non-dilated left ventricular cardiomyopathy; Pregnancy; Restrictive cardiomyopathy; Risk stratification; Screening; Sports; Sudden cardiac death EN Guidelines Arrhythmia Arrhythmogenic right ventricular cardiomyopathy Cardiomyopathies Diagnosis Dilated cardiomyopathy Genetics Genetic counselling Genetic testing Hypertrophic cardiomyopathy Implantable cardioverter defibrillator Management Multimodality imaging Non-dilated left ventricular cardiomyopathy Pregnancy Restrictive cardiomyopathy Risk stratification Screening Sports Sudden cardiac death 3503 3626 124 10/04/23 20231001 NES 231001 Table of contents 1. ESC Guidelines do not override the individual responsibility of health professionals to make appropriate and accurate decisions in consideration of each patient's health condition and in consultation with that patient or the patient's caregiver where appropriate and/or necessary. The NDLVC phenotype will include individuals that up until now may have variably been described as having DCM (but without LV dilatation), arrhythmogenic left ventricular cardiomyopathy (ALVC), left-dominant ARVC, or arrhythmogenic DCM (but often without fulfilling diagnostic criteria for ARVC) ( I Figure 3 i ). [Extracted from the article]

Published
2023
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