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1. Improving genetic testing pathways for transthyretin amyloidosis in France: challenges and strategies

Author

Hebrard, Bérénice, Babonneau, Marie-Lise, Charron, Philippe, Consolino, Emilie, Dauriat, Benjamin, Dupin-Deguine, Delphine, Fargeaud, Dominique, Farrugia, Agnès, Giguet-Valard, Anna-Gaëlle, Guijarro, Damien, Inamo, Jocelyn, Jeanneteau, Julien, Mazzella, Jean-Michaël, Michon, Claire-Cécile, Millat, Gilles, Mouquet, Frédéric, Oghina, Silvia, Pereon, Yann, Poinsignon, Vianney, Pompougnac, Julie, Proukhnitzky, Julie, Schaefer, Elise, Sturtz, Franck, Trosdorf, Mathilde, Auguste, Anne, Canali, Giorgia, Combes, Alexandre, Funalot, Benoît, and Damy, Thibaud

Published
2024
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5. Incidence and survival of transthyretin amyloid cardiomyopathy from a French nationwide study of in- and out-patient databases

Author

Damy, Thibaud, Bourel, Guillaume, Slama, Michel, Algalarrondo, Vincent, Lairez, Olivier, Fournier, Pauline, Costa, Jérôme, Pelcot, Françoise, Farrugia, Agnès, Zaleski, Isabelle Durand, Lilliu, Hervé, Rault, Caroline, Bartoli, Mathilde, Fievez, Stéphane, Granghaud, Anna, Rudant, Jeremie, Coste, Agathe, Cosson, Charlotte Noirot, Squara, Pierre-Alexandre, Narbeburu, Marion, De Neuville, Bertrand, and Charron, Philippe

Published
2023
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6. Rationale and design of the PACIFIC-PRESERVED (PhenomApping, ClassIFication and Innovation for Cardiac dysfunction in patients with heart failure and PRESERVED left ventricular ejection fraction) study

Author

Hulot, Jean-Sébastien, Janiak, Philip, Boutinaud, Philippe, Boutouyrie, Pierre, Chézalviel-Guilbert, Frédérique, Christophe, Jean-Joseph, Cohen, Ariel, Damy, Thibaud, Djadi-Prat, Juliette, Firat, Hüseyin, Hervé, Pierre-Yves, Isnard, Richard, Jondeau, Guillaume, Mousseaux, Elie, Pernot, Mathieu, Prot, Pierre, Tyl, Benoit, Soulat, Gilles, and Logeart, Damien

Published
2024
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7. Clinical outcomes for 2788 patients with transthyretin amyloidosis: Tafamidis meglumine early access program in France

Author

Lairez, Olivier, Réant, Patricia, Inamo, Jocelyn, Jeanneteau, Julien, Bauer, Fabrice, Habib, Gilbert, Eicher, Jean-Christophe, Lequeux, Benoit, Legallois, Damien, Josse, Constant, Hippocrate, Aurelie, Bartoli, Mathilde, Dubois, Margaux, Noirot Cosson, Charlotte, Squara, Pierre-Alexandre, Fievez, Stephane, Quinault, Aurore, Rudant, Jeremie, Kharoubi, Mounira, and Damy, Thibaud

Published
2024
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8. Transthyretin amyloid cardiomyopathy in France: A cross-sectional multi-centre study (333 patients)

Author

Damy, Thibaud, Donal, Erwan, Lairez, Olivier, Eicher, Jean-Christophe, Karoubi, Mounira, Trochu, Jean-Noël, Inamo, Jocelyn, Habib, Gilbert, Roubille, François, Hagège, Albert, Morio, Flore, Cariou, Eve, Adda, Jérôme, Algalarrondo, Vincent, Coste, Agathe, Bartoli, Mathilde, Rudant, Jérémie, Salvi, Lara, Francou, Bruno, Guiochon-Mantel, Anne, Adams, David, Antoine, Jean-Christophe, Attarian, Shahram, Cintas, Pascal, Morales, Raul Juntas, Lagrange, Emmeline, Magy, Laurent, Mallaret, Martial, Péréon, Yann, Petiot, Philippe, Cauquil, Cécile, Labeyrie, Céline, Echaniz-Laguna, Andoni, Sole, Guilhem, Tard, Céline, Oghina, Silvia, Charron, Philippe, and Slama, Michel

Published
2024
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9. FACE study: 2-year follow-up of adaptive servo-ventilation for sleep-disordered breathing in a chronic heart failure cohort

Author

Attali, Valérie, Maria-Anna, Balice, Laurent, Barthel, Rosa, Buendia, Bertien, Buyse, Laurent, Boyer, Marie-Pierre, Cadars, Pascal, Cornec, Thibaud, Damy, Jean-Marc, Davy, Geoffroy, De Faverges, Toufik, Didi, Marie-Pia, d'ortho, Frédéric, Gagnadoux, Thibaud, Gentina, François, Goupil, Frédéric, Goutorbe, Jean-Maurice, Guillemot, Carmen, Iamandi, Richard, Isnard, François, Jounieaux, Christian, Koltes, Alain, Le Coz, Benoit, Lequeux, René, Lerest, Lionel, Lerousseau, Jean-Pierre, Mallet, Francis, Martin, Jean-Claude, Meurice, Ala, Noroc, Frédéric, Ortuno, Alain, Palot, Philippe, Papola, Audrey, Paris, Hélène, Pastinelli, Jean-Louis, Pepin, Christophe, Perrin, Carole, Philippe, Sandrine, Pontier, Arnaud, Prigent, Pascaline, Priou, Vincent, Puel, Claudio, Rabec, Benjamin, Richard, Claude, Richard, Marijke, Rutten, Manuel, Sastry, Murielle, Salvat, Kamila, Sedkaoui, Bharati, Shivalkar, Renaud, Tamisier, Dries, Testelmans, Johan, Verbraecken, Tamisier, Renaud, Damy, Thibaud, Bailly, Sébastien, Goutorbe, Frédéric, Davy, Jean-Marc, Lavergne, Florent, Palot, Alain, Verbraecken, Johan A., d’Ortho, Marie-Pia, and Pépin, Jean-Louis

Published
2024
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12. Changes in amyloidosis phenotype over 11 years in a cardiac amyloidosis referral centre cohort in France

Author

Damy, Thibaud, Zaroui, Amira, de Tournemire, Marie, Kharoubi, Mounira, Gounot, Romain, Galat, Arnault, Guendouz, Soulef, Funalot, Benoit, Itti, Emmanuel, Roulin, Louise, Audard, Vincent, Fanen, Pascale, Leroy, Vincent, Poulot, Elsa, Belhadj, Karim, Mallet, Sophie, Deep Singh Chadah, Gagan, Planté-Bordeneuve, Violaine, Gendre, Thierry, Chevalier, Xavier, Guignard, Sandra, Bequignon, Emilie, Bartier, Sophie, Folliguet, Thierry, Lemonier, François, Audureau, Etienne, Tixier, Denis, Canoui-Poitrine, Florence, Lefaucheur, Jean-Pascal, Souvannanorath, Sarah, Authier, Francois-Jerome, Maupou, Steven, Hittinger, Luc, Molinier-Frenkel, Valérie, David, Jean-Philippe, Broussier, Amaury, Oghina, Silvia, and Teiger, Emmanuel

Published
2023
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14. Real-Life Evaluation of an Algorithm for the Diagnosis of Cardiac Amyloidosis

Author

Bézard, Mélanie, Kharoubi, Mounira, Galat, Arnault, Le Bras, Fabien, Poullot, Elsa, Molinier-Frenkel, Valérie, Fanen, Pascale, Funalot, Benoit, Moktefi, Anissa, Abulizi, Mukedaisi, Deux, Jean-François, Lemonnier, François, Guendouz, Soulef, Chalard, Coraline, Zaroui, Amira, Itti, Emmanuel, Hittinger, Luc, Teiger, Emmanuel, Oghina, Silvia, and Damy, Thibaud

Published
2023
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15. Prognosis of Transthyretin Cardiac Amyloidosis Without Heart Failure Symptoms

Author

Gonzalez-Lopez, Esther, Escobar-Lopez, Luis, Obici, Laura, Saturi, Giulia, Bezard, Mélanie, Saith, Sunil E., AbouEzzeddine, Omar F., Mussinelli, Roberta, Gagliardi, Christian, Kharoubi, Mounira, Griffin, Jan M., Dispenzieri, Angela, Vilches, Silvia, Perlini, Stefano, Longhi, Simone, Oghina, Silvia, Rivas, Adrian, Grogan, Martha, Maurer, Mathew S., Damy, Thibaud, Palladini, Giovanni, Rapezzi, Claudio, and Garcia-Pavia, Pablo

Published
2022
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17. Post-capillary pulmonary hypertension in heart failure: impact of current definition in the PH-HF multicentre study.

Author

Fauvel, Charles, Damy, Thibaud, Berthelot, Emmanuelle, Bauer, Fabrice, Eicher, Jean-Christophe, Groote, Pascal de, Trochu, Jean-Noël, Picard, François, Renard, Sébastien, Bouvaist, Hélène, Logeart, Damien, Roubille, François, Sitbon, Olivier, and Lamblin, Nicolas

Subjects
PULMONARY hypertension, PULMONARY artery, HEART failure, VASCULAR resistance, PROGNOSIS
Abstract

Background and Aims Based on retrospective studies, the 2022 European guidelines changed the definition of post-capillary pulmonary hypertension (pcPH) in heart failure (HF) by lowering the level of mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR). However, the impact of this definition and its prognostic value has never been evaluated prospectively. Methods Stable left HF patients with the need for right heart catheterization were enrolled from 2010 to 2018 and prospectively followed up in this multicentre study. The impact of the successive pcPH definitions on pcPH prevalence and subgroup [i.e. isolated (IpcPH) vs. combined pcPH (CpcPH)] was evaluated. Multivariable Cox regression analysis was used to assess the prognostic value of mPAP and PVR on all-cause death or hospitalization for HF (primary outcome). Results Included were 662 HF patients were (median age 63 years, 60% male). Lowering mPAP from 25 to 20 mmHg resulted in +10% increase in pcPH prevalence, whereas lowering PVR from 3 to 2 resulted in +60% increase in CpcPH prevalence (with significant net reclassification improvement for the primary outcome). In multivariable analysis, both mPAP and PVR remained associated with the primary outcome [hazard ratio (HR) 1.02, 95% confidence interval (CI) 1.00–1.03, P =.01; HR 1.07, 95% CI 1.00–1.14, P =.03]. The best PVR threshold associated with the primary outcome was around 2.2 WU. Using the 2022 definition, pcPH patients had worse survival compared with HF patients without pcPH (log-rank, P =.02) as well as CpcPH compared with IpcPH (log-rank, P =.003). Conclusions This study is the first emphasizing the impact of the new pcPH definition on CpcPH prevalence and validating the prognostic value of mPAP > 20 mmHg and PVR > 2 WU among HF patients. [ABSTRACT FROM AUTHOR]

Published
2024
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18. Long‐term tafamidis efficacy in patients with transthyretin amyloid cardiomyopathy by baseline left ventricular ejection fraction.

Author

Drachman, Brian, Damy, Thibaud, Hanna, Mazen, Wang, Ronnie, Angeli, Franca S., and Garcia‐Pavia, Pablo

Subjects
*VENTRICULAR ejection fraction, *HEART transplantation, *TREATMENT delay (Medicine), *HEART failure, *TRANSTHYRETIN, *HEART assist devices
Abstract

Aims: Patients with transthyretin amyloid cardiomyopathy (ATTR‐CM) present with diverse left ventricular ejection fraction (LVEF). This study assessed tafamidis efficacy by baseline LVEF in the phase 3 Tafamidis in Transthyretin Cardiomyopathy Clinical Trial (ATTR‐ACT) and its long‐term extension (LTE) study. Methods and results: Patients were randomized to 30 months of tafamidis or placebo treatment in ATTR‐ACT. On completion, patients could join an LTE study to receive tafamidis. All‐cause mortality (death, heart transplant, or cardiac mechanical assist device implantation) from baseline to the end of follow‐up was assessed in patients continuously treated with tafamidis (80 mg meglumine or 61 mg free acid) or delayed tafamidis treatment (placebo in ATTR‐ACT; tafamidis in the LTE study) according to baseline LVEF (<50% or ≥50%). Supportive outcomes were evaluated over a shorter follow‐up. Patients with baseline LVEF <50% (n = 177: 88 tafamidis‐ and 89 placebo‐treated) had signs of more severe heart failure, a higher proportion were Black, and had variant ATTR‐CM than those with LVEF ≥50% (n = 171: 85 tafamidis‐ and 86 placebo‐treated). At the end of follow‐up (median 60–64 months), all‐cause mortality was numerically higher in patients with baseline LVEF <50%; however, consistent with supportive findings, continuous tafamidis treatment was associated with a 47% reduction in mortality risk compared with delayed tafamidis treatment in patients with LVEF <50% and ≥50% (hazard ratio 0.53 [95% confidence interval 0.367–0.758]; p < 0.001, and 0.53 [0.344–0.818]; p < 0.01, respectively). Conclusions: Early initiation of tafamidis is associated with reduced mortality in patients with ATTR‐CM, irrespective of initial LVEF value. Clinical Trial Registration: ClinicalTrials.gov NCT01994889, NCT02791230. [ABSTRACT FROM AUTHOR]

Published
2024
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19. Internalisation of immunoglobulin light chains by cardiomyocytes in AL amyloidosis: what can biopsies tell us?

Author

Bézard, Mélanie, Zaroui, Amira, Kharoubi, Mounira, Lam, France, Poullot, Elsa, Teiger, Emmanuel, Agbulut, Onnik, Damy, Thibaud, and Kordeli, Ekaterini

Subjects
IMMUNOGLOBULIN light chains, HEART cells, HIGH resolution imaging, IMAGE reconstruction, CONFOCAL microscopy, CARDIAC amyloidosis
Abstract

Background: Cardiac involvement in systemic light chain amyloidosis (AL) leads to chronic heart failure and is a major prognosis factor. Severe cellular defects are provoked in cardiac cells by tissue-deposited amyloid fibrils of misfolded free immunoglobulin light chains (LCs) and their prefibrillar oligomeric precursors. Objective: Understanding the molecular mechanisms behind cardiac cell cytotoxicity is necessary to progress in therapy and to improve patient management. One key question is how extracellularly deposited molecules exert their toxic action inside cardiac cells. Here we searched for direct evidence of amyloid LC uptake by cardiomyocytes in patient biopsies. Methods: We immunolocalized LCs in cardiac biopsies from four AL cardiac amyloidosis patients and analysed histopathological images by high resolution confocal microscopy and 3D image reconstruction. Results: We show, for the first time directly in patient tissue, the presence of LCs inside cardiomyocytes, and report their proximity to nuclei and to caveolin-3-rich areas. Our observations point to macropinocytosis as a probable mechanism of LC uptake. Conclusions: Internalisation of LCs occurs in patient cardiomyocytes. This event could have important consequences for the pathogenesis of the cardiac disease by enabling interactions between amyloid molecules and cellular organelles inducing specific signalling pathways, and might bring new insight regarding treatment. [ABSTRACT FROM AUTHOR]

Published
2024
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27. Current practices and access to cardiac bone scans for the detection of transthyretin cardiac amyloidosis based on the results of a large national electronic survey

Author

Hyafil, Fabien, primary, Oghina, Silvia, primary, Legallois, Damien, primary, Amara, Walid, primary, Andres, Emmanuel, primary, Bardin, Thomas, primary, Fournier, Pauline, primary, Guignard, Sandra, primary, Labeyrie, Céline, primary, Toulza, Olivier, primary, Tresorier, Romain, primary, Piriou, Nicolas, primary, SABOURET, Pierre, primary, Stanbury, Trevor, primary, Canali, Giorgia, primary, DUBOIS, Margaux, primary, Noirot-Cosson, Charlotte, primary, Rudant, Jeremie, primary, Bouquillon, Benoit, primary, Sauvage, Cedric, primary, Charron, Philippe, primary, and Damy, Thibaud, primary

Published
2024
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34. 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, Silvia, primary, Legallois, Damien, primary, Hyafil, Fabien, primary, Amara, Walid, primary, Andres, Emmanuel, primary, Bardin, Thomas, primary, Fournier, Pauline, primary, Guignard, Sandra, primary, Labeyrie, Céline, primary, Piriou, Nicolas, primary, SABOURET, Pierre, primary, Toulza, Olivier, primary, Tresorier, Romain, primary, Stanbury, Trevor, primary, Canali, Giorgia, primary, DUBOIS, Margaux, primary, Noirot-Cosson, Charlotte, primary, Rudant, Jeremie, primary, Bouquillon, Benoit, primary, Sauvage, Cedric, primary, Charron, Philippe, primary, and Damy, Thibaud, primary

Published
2024
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35. Survival of patients with transthyretin amyloid cardiomyopathy (ATTR-CM) according to the dispensed daily dose of loop diuretics, based on SNDS French claims database

Author

Algalarrondo, Vincent, primary, Narbeburu, Marion, primary, Slama, Michel, primary, Lairez, Olivier, primary, Fournier, Pauline, primary, Costa, Jerome, primary, PELCOT, Francoise, primary, FARRUGIA, Agnes, primary, Durand, Isabelle, primary, Lilliu, Herve, primary, Bartoli, Mathilde, primary, FIEVEZ, Stéphane, primary, Rudant, Jeremie, primary, COSTE, Agathe, primary, Canali, Giorgia, primary, Coquebert, Bertrand, primary, Charron, Philippe, primary, and Damy, Thibaud, primary

Published
2024
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36. Incidence of transthyretin amyloid cardiomyopathy from a French nationwide study of in- and out-patient databases

Author

Damy, Thibaud, primary, Narbeburu, Marion, primary, Algalarrondo, Vincent, primary, Lairez, Olivier, primary, Fournier, Pauline, primary, Costa, Jerome, primary, PELCOT, Francoise, primary, FARRUGIA, Agnes, primary, Durand, Isabelle, primary, Lilliu, Herve, primary, Bartoli, Mathilde, primary, FIEVEZ, Stéphane, primary, Rudant, Jeremie, primary, COSTE, Agathe, primary, Canali, Giorgia, primary, Coquebert, Bertrand, primary, Slama, Michel, primary, and Charron, Philippe, primary

Published
2024
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37. Prognostic mortality factors in advanced light chain cardiac amyloidosis: A prospective cohort study

Author

Zaroui, Amira, primary, Kharoubi, Mounira, additional, Gounot, Romain, additional, Oghina, Silvia, additional, Degoutte, Charlotte, additional, Bezard, Melanie, additional, Galat, Arnault, additional, Guendouz, Soulef, additional, Roulin, Louise, additional, Audard, Vincent, additional, Leroy, Vincent, additional, Teiger, Emmanuel, additional, Poullot, Elsa, additional, Molinier‐Frenkel, Valérie, additional, Le Bras, Fabien, additional, Belhadj, Karim, additional, Bastard, Jean‐Philippe, additional, Fellahi, Soraya, additional, Shourick, Jason, additional, Lemonier, Francois, additional, and Damy, Thibaud, additional

Published
2024
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42. Coronavirus disease vaccination in heart failure: No time to waste

Author

Roncalli, Jerome, Roubille, François, Lamblin, Nicolas, Girerd, Nicolas, Mouquet, Frédéric, Chapet, Nicolas, Roubille, Camille, Berthelot, Emmanuelle, Galois, Katie, Battistella, Pascal, Jondeau, Guillaume, Tartiere, Jean-Michel, Aguilhon, Sylvain, Gueffet, Jean-Pierre, Salvat, Muriel, Damy, Thibaud, and Galinier, Michel

Published
2021
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46. Epidemiological characteristics and therapeutic management of patients with chronic heart failure who use smartphones: Potential impact of a dedicated smartphone application (report from the OFICSel study)

Author

Pezel, Théo, Berthelot, Emmanuelle, Gauthier, Jean, Chong-Nguyen, Caroline, Iliou, Marie C., Juillière, Yves, Galinier, Michel C., De Groote, Pascal, Beauvais, Florence, Bauer, Fabrice, Vergeylen, Ugo, Gellen, Barnabas, Raphael, Pierre, Bezard, Mélanie, Ricci, Jean-Etienne, Boiteux, Marie-Claire, Bonnefous, Louis, Bodez, Diane, Audureau, Etienne, and Damy, Thibaud

Published
2021
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47. Cardiovascular disease healthcare trajectories: descriptions, similarities, mortality rates of heart failure in France.

Author

Nevoret, Camille, Tran, Yohann, Guendouz, Soulef, Lavenu, Audrey, Katsahian, Sandrine, Damy, Thibaud, and Tropeano, Anne‐Isabelle

Subjects
PROGNOSIS, ATRIAL fibrillation, DEATH rate, DATA mining, DATABASES, HEART failure
Abstract

Aims: The primary objectives of this study were to analyse the nationwide healthcare trajectories of heart failure (HF) patients in France, 2 years after their first hospitalization, and to measure sequence similarities. Secondary objectives were to identify the association between trajectories and the risk of mortality. Methods and results: A retrospective, observational study was conducted using data extracted from the Echantillon Généraliste des Bénéficiaires database, covering the period from 1 January 2008 to 31 December 2018. Follow‐up concluded upon death or at the end of the study. We developed a methodology specific to healthcare data by extracting frequent healthcare trajectories and measuring their similarity for use in a survival machine learning analysis. In total, 11 488 HF patients were included and followed up for an average of 2.9 ± 1.3 years. The mean age of the patients was 78.0 ± 13.2 years. The first‐year mortality rate was 31.7% and increased to 78.8% at 5 years. Fifty per cent of patients experienced re‐hospitalization for reasons related to cardiovascular diseases. We identified 1707 hospitalization sequences, and 21 sequences were associated with survival, while 15 sequences were linked to mortality. In all our models, age and gender emerged as the most significant predictors of mortality (permutation feature importance: 0.099 ± 0.00078 and 0.0087 ± 0.00018, respectively; weights could be interpreted in relative terms). Specifically, the age at initial hospitalization for HF was positively associated with mortality. Gender (male: 49.5%) was associated with poorer prognoses. Healthcare trajectories, including non‐surgical device treatments, valve replacements, and atrial fibrillation ablation, were associated with a better prognosis (permutation feature importance: 0.0047 ± 0.00011, 0.0014 ± 0.000073, and 0.00095 ± 0.000097, respectively), except in cases where these invasive treatments preceded or followed hospitalization for cardiac decompensation. The predominant negative prognosis sequences were mostly those that included HF‐related hospitalizations before or after other‐related hospitalizations (permutation feature importance: 0.0007 ± 0.000091 and 0.00011 ± 0.000045, respectively). Conclusions: We highlight the value of healthcare trajectories on frequent hospitalization sequences, mortality, and prognosis and indicate the necessary prognostic value of HF re‐hospitalization. Our work may be an essential tool for better identification of at‐risk patients in order to increase and improve personalized care in the future. [ABSTRACT FROM AUTHOR]

Published
2024
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50. Heart Transplantation, Either Alone or Combined With Liver and Kidney, a Viable Treatment Option for Selected Patients With Severe Cardiac Amyloidosis

Author

Guendouz, Soulef, Grimbert, Philippe, Radu, Costin, Cherqui, Daniel, Salloum, Chady, Mongardon, Nicolas, Maghrebi, Sami, Belhadj, Karim, Le Bras, Fabien, Teiger, Emmanuel, Couetil, Jean-Paul, Balan, Adriana, Kharoubi, Mounira, Bézard, Mélanie, Oghina, Silvia, Bodez, Diane, Hittinger, Luc, Audard, Vincent, Planté-Bordeneuve, Violaine, De la Taille, Alexandre, Bergoend, Eric, Frenkel, Valerie, Fanen, Pascale, Leroy, Vincent, Duvoux, Christophe, Carmagnat, Maryvonnick, Folliguet, Thierry, and Damy, Thibaud

Published
2022
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