French national reference center for hereditary or rare cardiomyopathies and cardiac arrhythmias
The reference center for hereditary or rare cardiomyopathies and cardiac arrhythmias brings together the expert sites within the Assistance Publique-Hôpitaux de Paris. Thanks to the complementarity and multidisciplinarity of the skills brought together (heart failure, rhythmology, genetics, imaging, psychologists and specialized nurses), the site of the Georges-Pompidou European hospital (HEGP), coordinated by Pr Albert Hagege, director of the cardiovascular medical university department of the hospital group and head of the cardiology department, has been providing comprehensive care for arrythmias and cardiomyopathies of genetic and/or rare origin since 2004.
The reference center ensures the diagnosis and the care of complex cases, including explorations and medicinal and invasive treatments (septal alcohol, cardiac defibrillators, ablation of rhythm disorders, cardiac surgery and transplantation); It participates in the implementation of treatment protocols and international care recommendations in this field and coordinates research activities, in close collaboration with the genetics department headed by Pr Xavier Jeunemaître, the HEGP/PARCC sudden death expertise center, and the patient therapeutic education unit. The HEGP site ensures the transition from children to adults within the « Paris-Centre Cardiomyopathies » structure, accredited by the university of Paris and specialized in the care of familial cardiomyopathy, hypertrophic cardiomyopathy (it coordinates a national registry of nearly 2000 patients), cardiac amyloidosis (within the « Paris-Centre Amyloidosis » structure), Fabry’s disease (in connection with the Garches reference center) and dilated cardiomyopathies. The reference center is the leader in France in innovative therapeutics in this field.
This reference center is affiliated with the CARDIOGEN rare diseases healthcare network and the GUARD-HEART european reference networks (ERN).
Keywords: Rare diseases, cardiomyopathies, hypertrophic cardiomyopathies, dilated cardiomyopathies, cardiac rhythm disorders, sudden death, genetics, hereditary diseases, Fabry disease, cardiac amyloidosis, cell therapy, therapeutic trials.
Medical team
Pr Albert Hagège
MD, PhD
In case of emergency
The HEGP center takes care of heart muscle diseases and hereditary arrhythmias as well as rare and non-hereditary heart muscle diseases.
The expertise is multidisciplinary, cardiological (explorations and therapeutics), including imaging (ultrasound, scanner and MRI), genetic (diagnosis, genetic counseling and family investigations), while ensuring the continuity of care from the child to the adult and care organized in regional and national networks.
It provides information to patients and their families and to the general public, teaching (university and post-university), drafting care protocols and treatment recommendations within learned societies (French and European Cardiology Societies).
It participates in numerous national (INSERM, ANR, APHP, etc.) and international (Leducq Foundation, European registries, etc.) research programs, including trials of innovative drug and molecular treatments within the « Cardiomyopathies Paris centre » structure, accredited by the University of Paris and involving the HEGP, Cochin and Necker-Enfants malades sites.
- Pr Albert Hagège – Head of the cardiology department (HEGP) & director of the reference center
- Pr Xavier Jeunemaître – Head of the genetics department (HEGP) & dean of the health faculty
- Pr Eloi Marijon – Head of rhythmology, cardiology department (HEGP)
- Dr Clarisse Billon – Geneticist (UHA), genetics department (HEPG)
- Pr Karim Wahbi – Cardiologist (Cochin Hospital) & genetics department (HEGP)
- Mr Jean-Michael Mazzella – Genetic counselor
- Dr Tania Puscas – Cardiology department (HEGP)
- Anne Bachet – Clinical Research Associate, cardiology department (HEGP)
- Dalila Hakem – Clinical Research Associate, cardiology department (HEGP)
- Dali Stojanovic – Secretary
- Therapeutic education programs set up by the reference center and validated by the regional health agency (ARS)
- Heart failure (including treatments and diets, anticoagulants, circulatory support, coaguchek).
- Adult congenital heart disease (including the transition from children to adults)
- Therapeutic education programs under development
- CECICS (Expertise and coordination unit for severe heart failure)
RESEARCH
- PARADIGM-HF (Sacubitril/Valsartan), Novartis – Finalized
- SOCRATES (Vericiguat), Bayer – Finalized
- LIBERTY-HCM (Eleclazine), Gilead – Finalized
- EXPLORER-HCM (Mavacamten), Myokardia – Finalized
- MAVA-LTE (Mavacamten), Myokardia – In progress
Cohorts
- REMY ( National Registry of Hypertrophic Cardiomyopathies)
- GE-REMY (GEnetics of the National Registry of Hypertrophic Cardiomyopathies)
EDUCATION
- IUD heart failure management
University of Paris
2021
– Cardiac sarcoidosis: A multimodal approach to reach the diagnosis.
Nicolas Piriou, Patrick Bruneval
Int J Cardiol, 2021 Jan 15, PMID: 33096145 DOI: 10.1016/j.ijcard.2020.10.047
– Management and outcomes of hypertrophic cardiomyopathy in young adults.
Émilie Baron, Nicole Karam, Erwan Donal, Tania Puscas, Mariana Mirabel, Anne Bacher, Karim Wahbi, Jean-Michael Mazzella, Xavier Jeunemaitre, Patricia Reant, Albert Hagège, REMY, GEREMY working groups of the French Society of Cardiology
Arch Cardiovasc Dis, 2021 Jun-Jul, PMID: 33744178 DOI: 10.1016/j.acvd.2020.12.006
– Association between prophylactic angiotensin-converting enzyme inhibitors and overall survival in Duchenne muscular dystrophy-analysis of registry data.
Raphaël Porcher, Isabelle Desguerre, Helge Amthor, Brigitte Chabrol, Frédérique Audic, François Rivier, Arnaud Isapof, Vincent Tiffreau, Emmanuelle Campana-Salort, France Leturcq, Sylvie Tuffery-Giraud, Rabah Ben Yaou, Djillali Annane
Eur Heart J, 2021 May 21, PMID: 33748842 DOI: 10.1093/eurheartj/ehab054
– Improved Cardiac Outcomes by Early Treatment with Angiotensin-Converting Enzyme Inhibitors in Becker Muscular Dystrophy.
Caroline Stalens, Leslie Motté, Anthony Béhin, Rabah Ben Yaou, France Leturcq, Guillaume Bassez, Pascal Laforêt, Bertrand Fontaine, Stéphane Ederhy, Marion Masingue, Malika Saadi, Sarah Leonard Louis, Nawal Berber, Tanya Stojkovic, Denis Duboc, Karim Wahbi
J Neuromuscul Dis, 2021, PMID: 33814458 PMCID: PMC8385526 DOI: 10.3233/JND-200620
– Prevalence and clinical outcomes of dystrophin-associated dilated cardiomyopathy without severe skeletal myopathy.
Maria A Restrepo-Cordoba, Karim Wahbi, Anca R Florian, Juan Jiménez-Jáimez, Luisa Politano, Michael Arad, Vicente Climent-Paya, Ana Garcia-Alvarez, Rasmus B Hansen et al.
Eur J Heart Fail, 2021 Aug, PMID: 34050592 DOI: 10.1002/ejhf.2250
– Narrative review of glycogen storage disorder type III with a focus on neuromuscular, cardiac and therapeutic aspects.
Édouard Berling, Pascal Laforêt, Karim Wahbi, Philippe Labrune, François Petit, Giuseppe Ronzitti, Alan O’Brien
J Inherit Metab Dis, 2021 May, PMID: 33368379 DOI: 10.1002/jimd.12355
– Genome-Wide Association Meta-Analysis Supports Genes Involved in Valve and Cardiac Development to Associate With Mitral Valve Prolapse.
Mengyao Yu, Sergiy Kyryachenko, Stephanie Debette, Philippe Amouyel, Jean-Jacques Schott, Thierry Le Tourneau, Christian Dina, Russell A Norris, Albert A Hagège, Xavier Jeunemaitre, Nabila Bouatia-Naji
Circ Genom Precis Med, 2021 Oct, PMID: 34461747 PMCID: PMC8530910 DOI: 10.1161/CIRCGEN.120.003148
– Sudden Cardiac Arrest in Young Women.
Orianne Weizman, Ardalan Sharifzadehgan, Wulfran Bougouin, Kumar Narayanan, Noémie Tence, Florence Dumas, Victor Waldmann, Lionel Lamhaut
Circulation, 2021 Feb 16, PMID: 33587656 DOI: 10.1161/CIRCULATIONAHA.120.052219
– Cardiac Involvement in Fabry Disease: JACC Review Topic of the Week.
Maurizio Pieroni, James C Moon, Eloisa Arbustini, Roberto Barriales-Villa, Antonia Camporeale, Andreja Cokan Vujkovac, Perry M Elliott, Albert Hagege, Johanna Kuusisto, Aleš Linhart, Peter Nordbeck, Iacopo Olivotto, Päivi Pietilä-Effati, Mehdi Namdar
J Am Coll Cardiol, 2021 Feb 23, PMID: 33602475 DOI: 10.1016/j.jacc.2020.12.024
2020
– Cardiovascular manifestations of myotonic dystrophy.
Karim Wahbi, Denis Furling
Trends Cardiovasc Med, 2020 May, PMID: 31213350 DOI: 10.1016/j.tcm.2019.06.001
– Prognostic value of the 12-lead surface electrocardiogram in sarcomeric hypertrophic cardiomyopathy: data from the REMY French register.
Nicolas Ledieu, Louis Larnier, Vincent Auffret, Coralie Marie, Dominique Fargeau, Erwan Donal, Mariana Mirabel, Xavier Jeunemaitre, Tania Puscas, Eloi Marijon, Amélie Reynaud, Philippe Ritter, Stephane Lafitte, Philippe Mabo, Patricia Réant, Claude Daubert, Albert A Hagège, REMY Working Group of the French Society of Cardiology
Europace, 2020 Jan 1, PMID: 31603495 DOI: 10.1093/europace/euz272
– A high prevalence of arterial hypertension in patients with mitochondrial diseases.
Caroline Chong-Nguyen, Caroline Stalens, Yves Goursot, Wulfran Bougouin, Tanya Stojkovic, Anthony Béhin, Fanny Mochel, Nawal Berber, Bruno Eymard, Denis Duboc, Pascal Laforêt, Karim Wahbi
J Inherit Metab Dis, 2020 May, PMID: 31762033 DOI: 10.1002/jimd.12195
– Incidence and predictors of total mortality in 267 adults presenting with mitochondrial diseases.
Constantinos Papadopoulos, Karim Wahbi, Anthony Behin, Wulfran Bougouin, Tanya Stojkovic, Sarah Leonard-Louis, Nawal Berber, Anne Lombès, Denis Duboc, Claude Jardel, Bruno Eymard, Pascal Laforêt
J Inherit Metab Dis, 2020 May, PMID: 31652339 DOI: 10.1002/jimd.12185
– Looking at New Unexpected Disease Targets in LMNA-Linked Lipodystrophies in the Light of Complex Cardiovascular Phenotypes: Implications for Clinical Practice.
Héléna Mosbah, Camille Vatier, Franck Boccara, Isabelle Jéru, Olivier Lascols, Marie-Christine Vantyghem, Bruno Fève, Bruno Donadille, Elisabeth Sarrazin, Sophie Benabbou, Jocelyn Inamo, Stéphane Ederhy, Ariel Cohen, Barbara Neraud, Pascale Richard, Fabien Picard, Sophie Christin-Maitre, Alban Redheuil, Karim Wahbi, Corinne Vigouroux
Cells, 2020 Mar 20, PMID: 32245113 PMCID: PMC7140635 DOI: 10.3390/cells9030765
– Prospective follow-up in various subtypes of cardiomyopathies: Insights from the ESC EORP Cardiomyopathy Registry
Gimeno JR, Elliott PM, Tavazzi L, Tendera M, Kaski JP, Laroche C, et al.
Eur Heart J Qual Care Clin Outcomes, 2020 Oct 9, PMID: 33035297 DOI: 10.1093/ehjqcco/qcaa075
– An expert consensus document on the management of cardiovascular manifestations of Fabry disease
Linhart A, Germain DP, Olivotto I, Hagège A, Cecchi F, Gimeno JR, et al.
Eur J Heart Fail, 2020 Jul, PMID: 32640076 DOI: 10.1002/ejhf.1960
– Prognostic value of the 12-lead surface electrocardiogram in sarcomeric hypertrophic cardiomyopathy: data from the REMY French register
Ledieu N, Larnier L, Auffret V, Marie C, Fargeau D, Donal E, et al.
Europace, 2020 Jan 1, PMID: 31603495 DOI: 10.1093/europace/euz272
– Influence of centre expertise on the diagnosis and management of hypertrophic cardiomyopathy: A study from the French register of hypertrophic cardiomyopathy (REMY)
Mirabel M, Damy T, Donal E, Huttin O, Labombarda F, Eicher JCet al.
Int J Cardiol, 2019 Jan 15, PMID: 30316646 DOI: 10.1016/j.ijcard.2018.09.083
2019
– Influence of centre expertise on the diagnosis and management of hypertrophic cardiomyopathy: A study from the French register of hypertrophic cardiomyopathy (REMY).
Mirabel, M ; Damy, T ; Donal, E ; Huttin, O ; Labombarda, F ; Eicher, JC & al.
Int J Cardiol, 2019 Jan 15, PMID: 30316646 DOI: 10.1016/j.ijcard.2018.09.083
– Fabry disease in cardiology practice: Literature review and expert point of view.
Hagège, A ; Réant, P ; Habib, G ; Damy, T ; Barone-Rochette, G ; Soulat, G & al
Arch Cardiovasc Dis, 2019 Apr, PMID: 30826269 DOI: 10.1016/j.acvd.2019.01.002
– Comprehensive evaluation of structural and functional myocardial impairments in Becker muscular dystrophy using quantitative cardiac magnetic resonance imaging.
Marty, B ; Gilles, R ; Toussaint, M ; Béhin, A ; Stojkovic, T ; Eymard, B & al
Eur Heart J Cardiovasc Imaging, 2019 Aug 1, PMID: 30590561 DOI: 10.1093/ehjci/jey209
– Myocardial Stiffness Evaluation Using Noninvasive Shear Wave Imaging in Healthy and Hypertrophic Cardiomyopathic Adults.
Villemain, O ; Correia, M ; Mousseaux, E ; Baranger, J ; Zarka, S ; Podetti, I & al
JACC Cardiovasc Imaging, 2019 Jul 12, PMID: 29550319 PMCID: PMC6603249 DOI: 10.1016/j.jcmg.2018.02.002
– Development and Validation of a New Risk Prediction Score for Life-Threatening Ventricular Tachyarrhythmias in Laminopathies.
Wahbi, K ; Ben Yaou, R ; Gandjbakhch, E ; Anselme, F ; Gossios, T ; Lakdawala, NK & al
Circulation, 2019 Jul 23, PMID: 31155932 DOI: 10.1161/CIRCULATIONAHA.118.039410
– Primary cilia defects causing mitral valve prolapse.
Toomer, KA ; Yu, M ; Fulmer, D ; Guo, L ; Moore, KS ; Moore, R & al
Sci Transl Med, 2019 May 22, PMID: 31118289 PMCID: PMC7331025 DOI: 10.1126/scitranslmed.aax0290
– Acute heart failure congestion and perfusion status - impact of the clinical classification on in-hospital and long-term outcomes; insights from the ESC-EORP-HFA Heart Failure Long-Term Registry.
Chioncel, O ; Mebazaa, A ; Maggioni, AP ; Harjola, VP ; Rosano, G ; Laroche, C & al
Eur J Heart Fail, 2019 Nov, PMID: 31127678 DOI: 10.1002/ejhf.1492
– Does the Flow Know? Mitral Regurgitant Jet Direction and Need for Valve Repair in Hypertrophic Obstructive Cardiomyopathy.
Schwammenthal, E ; Hagège, AA ; Levine, RA
J Am Soc Echocardiogr, 2019 Mar, PMID: 30827370 DOI: 10.1016/j.echo.2019.01.005
– Genome-Wide Association Study-Driven Gene-Set Analyses, Genetic, and Functional Follow-Up Suggest GLIS1 as a Susceptibility Gene for Mitral Valve Prolapse.
Yu, M ; Georges, A ; Tucker, NR ; Kyryachenko, S ; Toomer, K ; Schott, JJ & al
Circ Genom Precis Med, 2019 May, PMID: 31112420 PMCID: PMC6532425 DOI: 10.1161/CIRCGEN.119.002497
– FLNC pathogenic variants in patients with cardiomyopathies: Prevalence and genotype-phenotype correlations.
Ader, F ; De Groote, P ; Réant, P ; Rooryck-Thambo, C ; Dupin-Deguine, D ; Rambaud, C & al
Clin Genet, 2019 Oct, PMID: 31245841 DOI: 10.1111/cge.13594
– Treatment needs and expectations for Fabry disease in France: development of a new Patient Needs Questionnaire.
Noël, E ; Dussol, B ; Lacombe, D ; Bedreddine, N ; Fouilhoux, A ; Ronco, P & al
Orphanet J Rare Dis, 2019 Dec 4, PMID: 31801581 PMCID: PMC6894302 DOI: 10.1186/s13023-019-1254-7
– Congenital myopathies are mainly associated with a mild cardiac phenotype.
Petri, H ; Wahbi, K ; Witting, N ; Køber, L ; Bundgaard, H ; Kamoun, E & al
J Neurol, 2019 Jun, PMID: 30874888 DOI: 10.1007/s00415-019-09267-3
– 238th ENMC International Workshop: Updating management recommendations of cardiac dystrophinopathyHoofddorp, The Netherlands, 30 November – 2 December 2018.
Bourke, JP ; Guglieri, M ; Duboc, D
Neuromuscul Disord, 2019 Aug, PMID: 31402197 DOI: 10.1016/j.nmd.2019.06.598
– X-linked Emery-Dreifuss muscular dystrophy manifesting with adult onset axial weakness, camptocormia, and minimal joint contractures.
Brisset, M ; Ben Yaou, R ; Carlier, RY ; Chanut, A ; Nicolas, G ; Romero, NB & al.
Neuromuscul Disord, 2019 Sep, PMID: 31474437 DOI: 10.1016/j.nmd.2019.06.009
2015
– Mitral valve disease–morphology and mechanisms
Levine RA, Hagége AA, Judge DP, Padala M, Dal-Bianco JP, Aikawa E, et al.
Nat Rev Cardiol, 2015 Dec, PMID: 26483167 PMCID: PMC4804623 DOI: 10.1038/nrcardio.2015.161
2014
– 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC).
Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, Charron P, Hagege AA, Lafont A, Limongelli G, Mahrholdt H, McKenna WJ, Mogensen J, Nihoyannopoulos P, Nistri S, Pieper PG, Pieske B, Rapezzi C, Rutten FH, Tillmanns C, Watkins H.
Eur Heart J, 2014 Oct 14, PMID: 25173338 DOI: 10.1093/eurheartj/ehu284
- Fabry disease: Cardiac involvment and differential diagnosis / 6th European Fabry Summer School – Société Européenne de Cardiologie / Roissy / Hagege / Cardiologues européens / 27/06/2019
- Conduite à tenir devant une cardiomyopathie hypertrophique découverte chez le sujet âgé / Paris / Hagege (Président de séance) / Cardiologues – Journées Européennes de la Société Française de Cardiologie 17/01/2019
- Cardiomyopathie hypertrophique : identifier les facteurs de mauvais pronostic et les évaluer en pratique clinique / Paris / Hagege (Président de séance) / Cardiologues – Journées Européennes de la Société Française de Cardiologie 18/01/2019
- The potential of genetic testing in dilated cardiomyopathy / Paris / K Wahbi / ESC Congress 2019 1/09/2019
- Expert Advice – Risk prediction for sudden cardiac death in inherited cardiac condition / Paris / K Wahbi / ESC Congress 2019 1/09/2019
Health – Repairing the heart without opening
France 2 – Télé matin | 04/25/2017
On the occasion of the presentation of the 2016 Daniele Hermann prize to professor Albert Hagège, head of the cardiology department at the Georges-Pompidou European hospital in Paris, we look back at recent advances in cardiology, in particular « percutaneous » techniques, those that make it possible to repair or replace heart valves without opening the chest and heart.
ATTR-ACT study: efficacy and safety of tafamidis in cardiac amyloidosis
Cardio-online |09/03/2018
Among the major studies at ESC 2018 is the ATTR-ACT study investigating the efficacy of tafamidis, a treatment used in transthyretinal amyloid cardiopathies. In this interview, Professor Hagège summarizes the essential points of this study and of tafamidis, which he considers a true « therapeutic revolution ».
- Recommendations for the care of hypertrophic cardiomyopathy
- Recommendations : Physical activities
- Recommendations: Against heart failure
- Patient information brochure: Hypertrophic cardiomyopathy
- Patient information brochure: Dilated cardiomyopathy
- Patient information brochure: The cardiac defibrillator
- Therapeutic education brochure: Understanding heart failure
- Therapeutic education brochure: Understanding heart failure treatments
Contact information
At the Georges-Pompidou European hospital, the reference center for hereditary or rare cardiomyopathies and cardiac arrhythmias in brief …
* data valid for 2022