Primary Non-Aortic Lesions Are Not Rare in Marfan Syndrome and Are Associated with Aortic Dissection Independently of Age

Author:

Sénémaud Jean1,Gaudry Marine2,Jouve Elisabeth3ORCID,Blanchard Arnaud4,Milleron Olivier5,Dulac Yves6,Olivier-Faivre Laurence7,Stephan Dominique8,Odent Sylvie9,Lanéelle Damien10,Dupuis-Girod Sophie11ORCID,Jondeau Guillaume5ORCID,Bal-Theoleyre Laurence4

Affiliation:

1. Service de Chirurgie Vasculaire, Thoracique et de Transplantation Pulmonaire, AP-HP, CHU Bichat, 75018 Paris, France

2. Service de Chirurgie Vasculaire, AP-HM, CHU La Timone, 13385 Marseille, France

3. Service d’Evaluation Médicale, AP-HM, CHU La Conception, 13005 Marseille, France

4. Centre de Référence Constitutif Pour le Syndrome de Marfan et Apparentés, Centre Aorte Timone, AP-HM, CHU La Timone Adultes, 13014 Marseille, France

5. Centre National de Référence Pour le Syndrome de Marfan et Apparentés, VASCERN HTAD European Reference Centre, Service de Cardiologie, AP-HP, CHU Bichat, INSERM U 1148 LVTS, Université de Paris, 75014 Paris, France

6. Centre de Référence Constitutif Pour le Syndrome de Marfan et Apparentés, Hôpital des Enfants, CHU de Toulouse, 31300 Toulouse, France

7. Centre de Compétence Pour le Syndrome de Marfan et Apparentés, Hôpital des Enfants, CHU de Dijon, 21000 Dijon, France

8. Centre de Compétence Pour le Syndrome de Marfan et Apparentés, CHU Nouvel Hôpital Civil, 67000 Strasbourg, France

9. Centre de Compétence Pour le Syndrome de Marfan et Apparentés, Hôpital Sud, CHU de Rennes, 35200 Rennes, France

10. Centre de Compétence Pour le Syndrome de Marfan et Apparentés, CHU de la Côte de Nacre, 14033 Caen, France

11. Centre de Compétence Pour le Syndrome de Marfan et Apparentés, CHU Hôpital Louis Pradel, 69500 Lyon, France

Abstract

Purpose: The study sought to estimate the prevalence of primary non-aortic lesions (PNAL) unrelated to extension of aortic dissection (AD) in a cohort of patients with Marfan syndrome (MFS). Methods: Adult patients presenting with pathogenic FBN1 mutations and an available pan-aortic contrast-enhanced CTA in eight French MFS clinics from April to October 2018 were included. Clinical and radiological data, particularly the presence of aortic lesions and PNAL (including aneurysm and ectasia), were retrospectively analyzed. Results: Out of 138 patients, 28 (20.3%) had PNAL. In total, 27 aneurysms in 13 patients and 41 ectasias in 19 patients were reported mainly in the subclavian, iliac, and vertebral segments. Four patients (31%) with aneurysms and none with ectasia required prophylactic intervention during follow-up (median: 46 months). In multivariate analysis, factors associated with PNAL were history of AD (OR = 3.9, 95%CI: 1.3–12.1, p = 0.018), history of previous descending aortic surgery (OR = 10.3, 95%CI: 2.2–48.3, p = 0.003) and age (per 10 years OR = 1.6, 95%CI: 1.1–2.4, p = 0.008). Conclusion: PNAL is not rare in MFS patients with evolutive aortic disease. Natural history may differ between aneurysms and ectasia, emphasizing the need for standardized definitions and systematic screening for PNAL.

Publisher

MDPI AG

Subject

General Medicine

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