Marco Pappaccogli1,2,3, Silvia Di Monaco1, Ewa Warchoł-Celińska4, Aurélien Lorthioir5, Laurence Amar5,6, Lucas S Aparicio7, Christophe Beauloye2,3, Rosa Maria Bruno8,9, Patrick Chenu2,3, Peter de Leeuw10, Tine De Backer11, Philippe Delmotte12, Zivka Dika13, Daniel Gordin14,15,16, Hilde Heuten17, Yoshio Iwashima18, Jean-Marie Krzesinski19,20, Abraham A Kroon10, Lucia Mazzolai21, Esteban Poch22, Pantelis Sarafidis23, Christophe Seinturier24, Wilko Spiering25, Laurent Toubiana26, Patricia Van der Niepen27, Daan van Twist28, Adriana Visonà29,30, Jean-Claude Wautrecht31, Helena Witowicz4, Jianzhong Xu32, Aleksander Prejbisz4, Andrzej Januszewicz4, Michel Azizi5,6, Alexandre Persu2,3. 1. Division of Internal Medicine and Hypertension Unit, Department of Medical Sciences, University of Turin, Turin, Italy. 2. Division of Cardiology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, 10 Avenue Hippocrate, 1200 Brussels, Belgium. 3. Pole of Cardiovascular Research, Institut de Recherche Expérimentale et Clinique, Université Catholique de Louvain, 1200 Brussels, Belgium. 4. Department of Hypertension, National Institute of Cardiology, Warsaw, Poland. 5. Hypertension Unit and DMU CARTE, AP-HP, Hôpital Européen Georges Pompidou, Paris, France. 6. Université de Paris, CIC1418, INSERM, Paris, France. 7. Hypertension Section, Internal Medicine Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina. 8. Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. 9. INSERM U970 and Université de Paris, Paris, France. 10. Department of Medicine, Maastricht University Medical Center, Maastricht, The Netherlands. 11. Department of Cardiovascular Diseases, University Hospital Ghent, Ghent, Belgium. 12. Hypertension Excellence Centre, Department of Cardiology, Ambroise Paré University Hospital, Mons, Belgium. 13. Department of Nephrology, Arterial Hypertension, Dialysis and Transplantation, University Hospital Center Zagreb, School of Medicine University of Zagreb, Zagreb, Croatia. 14. Helsinki Hypertension Centre of Excellence, University of Helsinki, Helsinki University Hospital, Helsinki, Finland. 15. Abdominal Center Nephrology, University of Helsinki, Helsinki University Hospital, Helsinki, Finland. 16. Folkhälsan Institute of Genetics, Folkhälsan Research Centre, Biomedicum Helsinki, Helsinki, Finland. 17. Department of Cardiology, University Hospital of Antwerp, Edegem, Belgium. 18. Department of Nephrology and Hypertension, Dokkyo Medical University, Tochigi, Japan. 19. Division of Nephrology, Department of Internal Medicine, University of Liège Hospital, Liège, Belgium. 20. Groupe Interdisciplinaire de Génoprotéomique Appliquée (GIGA), Cardiovascular Sciences, University of Liège, Liège, Belgium. 21. Division of Angiology, Heart and Vessel Department, Lausanne University Hospital-CHUV, Centre of Rare Vascular Diseases and RAVAD Registry, Lausanne, Switzerland. 22. Department of Nephrology and Kidney Transplantation, Hospital Clínic of Barcelona, Institut D'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain. 23. Department of Nephrology, Hippokration Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece. 24. Department of Cardiovascular Disease, Competence Center for Rare Vascular Diseases, European Center of Excellence in Arterial Hypertension, Grenoble-Alpes University Hospital, CS Grenoble Cedex 9, France. 25. Department of Vascular Medicine, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands. 26. Sorbonne Université, Université Paris 13, Sorbonne Paris Cité, INSERM, UMR_S1142, LIMICS, IRSAN, Paris, France. 27. Department of Nephrology & Hypertension, Universitair Ziekenhuis Brussel , Vrije Universiteit Brussel (VUB), Brussels, Belgium. 28. Department of Internal Medicine, Zuyderland Medical Centre, Sittard/Heerlen, The Netherlands. 29. Angiology Unit, Azienda ULSS 2 Marca Trevigiana, Castelfranco Veneto, Italy. 30. ESVM Board Member. 31. Department of Vascular Diseases, University Hospital ULB Erasme, Brussels, Belgium. 32. Shanghai Institute of Hypertension, Department of Hypertension, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.
Abstract
AIMS: Since December 2015, the European/International Fibromuscular Dysplasia (FMD) Registry enrolled 1022 patients from 22 countries. We present their characteristics according to disease subtype, age and gender, as well as predictors of widespread disease, aneurysms and dissections. METHODS AND RESULTS: All patients diagnosed with FMD (string-of-beads or focal stenosis in at least one vascular bed) based on computed tomography angiography, magnetic resonance angiography, and/or catheter-based angiography were eligible. Patients were predominantly women (82%) and Caucasians (88%). Age at diagnosis was 46 ± 16 years (12% ≥65 years old), 86% were hypertensive, 72% had multifocal, and 57% multivessel FMD. Compared to patients with multifocal FMD, patients with focal FMD were younger, more often men, had less often multivessel FMD but more revascularizations. Compared to women with FMD, men were younger, had more often focal FMD and arterial dissections. Compared to younger patients with FMD, patients ≥65 years old had more often multifocal FMD, lower estimated glomerular filtration rate and more atherosclerotic lesions. Independent predictors of multivessel FMD were age at FMD diagnosis, stroke, multifocal subtype, presence of aneurysm or dissection, and family history of FMD. Predictors of aneurysms were multivessel and multifocal FMD. Predictors of dissections were age at FMD diagnosis, male gender, stroke, and multivessel FMD. CONCLUSIONS: The European/International FMD Registry allowed large-scale characterization of distinct profiles of patients with FMD and, more importantly, identification of a unique set of independent predictors of widespread disease, aneurysms and dissections, paving the way for targeted screening, management, and follow-up of FMD. Published on behalf of the European Society of Cardiology. All rights reserved.
AIMS: Since December 2015, the European/International Fibromuscular Dysplasia (FMD) Registry enrolled 1022 patients from 22 countries. We present their characteristics according to disease subtype, age and gender, as well as predictors of widespread disease, aneurysms and dissections. METHODS AND RESULTS: All patients diagnosed with FMD (string-of-beads or focal stenosis in at least one vascular bed) based on computed tomography angiography, magnetic resonance angiography, and/or catheter-based angiography were eligible. Patients were predominantly women (82%) and Caucasians (88%). Age at diagnosis was 46 ± 16 years (12% ≥65 years old), 86% were hypertensive, 72% had multifocal, and 57% multivessel FMD. Compared to patients with multifocal FMD, patients with focal FMD were younger, more often men, had less often multivessel FMD but more revascularizations. Compared to women with FMD, men were younger, had more often focal FMD and arterial dissections. Compared to younger patients with FMD, patients ≥65 years old had more often multifocal FMD, lower estimated glomerular filtration rate and more atherosclerotic lesions. Independent predictors of multivessel FMD were age at FMD diagnosis, stroke, multifocal subtype, presence of aneurysm or dissection, and family history of FMD. Predictors of aneurysms were multivessel and multifocal FMD. Predictors of dissections were age at FMD diagnosis, male gender, stroke, and multivessel FMD. CONCLUSIONS: The European/International FMD Registry allowed large-scale characterization of distinct profiles of patients with FMD and, more importantly, identification of a unique set of independent predictors of widespread disease, aneurysms and dissections, paving the way for targeted screening, management, and follow-up of FMD. Published on behalf of the European Society of Cardiology. All rights reserved.
Authors: Alexandre Persu; Piotr Dobrowolski; Heather L Gornik; Jeffrey W Olin; David Adlam; Michel Azizi; Pierre Boutouyrie; Rosa Maria Bruno; Marion Boulanger; Jean-Baptiste Demoulin; Santhi K Ganesh; Tomasz J Guzik; Magdalena Januszewicz; Jason C Kovacic; Mariusz Kruk; Peter de Leeuw; Bart L Loeys; Marco Pappaccogli; Melanie H A M Perik; Emmanuel Touzé; Patricia Van der Niepen; Daan J L Van Twist; Ewa Warchoł-Celińska; Aleksander Prejbisz; Andrzej Januszewicz Journal: Cardiovasc Res Date: 2022-01-07 Impact factor: 10.787
Authors: Adrien Georges; Min-Lee Yang; Takiy-Eddine Berrandou; Mark K Bakker; Ozan Dikilitas; Soto Romuald Kiando; Lijiang Ma; Benjamin A Satterfield; Sebanti Sengupta; Mengyao Yu; Jean-François Deleuze; Delia Dupré; Kristina L Hunker; Sergiy Kyryachenko; Lu Liu; Ines Sayoud-Sadeg; Laurence Amar; Chad M Brummett; Dawn M Coleman; Valentina d'Escamard; Peter de Leeuw; Natalia Fendrikova-Mahlay; Daniella Kadian-Dodov; Jun Z Li; Aurélien Lorthioir; Marco Pappaccogli; Aleksander Prejbisz; Witold Smigielski; James C Stanley; Matthew Zawistowski; Xiang Zhou; Sebastian Zöllner; Philippe Amouyel; Marc L De Buyzere; Stéphanie Debette; Piotr Dobrowolski; Wojciech Drygas; Heather L Gornik; Jeffrey W Olin; Jerzy Piwonski; Ernst R Rietzschel; Ynte M Ruigrok; Miikka Vikkula; Ewa Warchol Celinska; Andrzej Januszewicz; Iftikhar J Kullo; Michel Azizi; Xavier Jeunemaitre; Alexandre Persu; Jason C Kovacic; Santhi K Ganesh; Nabila Bouatia-Naji Journal: Nat Commun Date: 2021-10-15 Impact factor: 17.694
Authors: Andrew B Dicks; Heather L Gornik; Xiaokui Gu; J Michael Bacharach; Natalia Fendrikova Mahlay; James B Froehlich; Kamal Gupta; Bruce H Gray; Esther S H Kim; Redah Mahmood; Aditya M Sharma; Bryan J Wells; Jeffrey W Olin; Ido Weinberg Journal: J Am Heart Assoc Date: 2021-08-28 Impact factor: 5.501