title: Aortic biomechanics by magnetic resonance: early markers of aortic disease in Marfan syndrome regardless of aortic dilatation?
abstract: BACKGROUND
  Previous studies demonstrated the usefulness of MRI in the evaluation of aortic biomechanics in Marfan patients with aortic dilatation. However, these parameters have not been well studied in earlier stages of aortic disease. The present work aimed to study aortic biomechanics: aortic distensibility (AD) and pulse wave velocity (PWV), by MRI in Marfan patients without advanced aortic disease.
  
  
  METHODS
  Eighty consecutive Marfan patients were compared with 36 age- and sex-matched controls. MRI images at the level of ascending, descending and abdominal aorta were used to determine AD and PWV.
  
  
  RESULTS
  Marfan patients (27 men; age: 32.0 ± 10.5 years; mean aortic root diameter: 37.2 ± 4.6mm) had lower AD at all levels (ascending 2.6 ± 2.1 vs. 6.2 ± 3.7 mm Hg(-1)·10(-3), p<0.001; descending 3.1 ± 2.0 vs. 8.3 ± 4.2, p<0.001; and abdominal 4.5 ± 2.2 vs. 14.0 ± 5.2, p<0.001), higher aortic arch PWV (8.1 ± 6.5 vs. 4.3 ± 1.8m/s, p<0.01) and ascending-to-abdominal PWV (6.1 ± 3.0 vs. 4.7 ± 1.5m/s, p<0.01) compared with controls. Thirty-five Marfan patients had a non-dilated aortic root (mean aortic root diameter: 34.5 ± 3.8 mm). In multivariable analyses, after adjustment for age, pulse pressure and aortic dimensions, AD remained lower and PWV higher in Marfan patients; even Marfan patients with non-dilated aortic root showed impaired aortic biomechanics compared with controls. Z-score for ascending AD<-3.5 distinguished Marfan patients from controls with 82.5% sensitivity and 86.1% specificity.
  
  
  CONCLUSIONS
  Aortic biomechanics by MRI were abnormal in the entire aorta in Marfan patients. Moreover, Marfan patients without dilated aortic root showed clear impairment of aortic biomechanics, which suggests that they may be used as early markers of aortic involvement in these patients.
authors: Department of Cardiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Spain. Electronic address: gisela.tt@gmail.com.
authors: Gisela
authors: G
authors: Teixido-Tura
authors: University of Paris-Descartes, Georges-Pompidou-European-Hospital, APHP and INSERM U678, Paris, France.
authors: Alban
authors: A
authors: Redheuil
authors: Department of Cardiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Spain.
authors: Jose
authors: J
authors: Rodríguez-Palomares
authors: Department of Cardiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Spain.
authors: Laura
authors: L
authors: Gutiérrez
authors: Department of Cardiology, Hospital Doce de Octubre, Madrid, Spain.
authors: Violeta
authors: V
authors: Sánchez
authors: Department of Cardiovascular Surgery, Hospital Doce de Octubre, Madrid, Spain.
authors: Alberto
authors: A
authors: Forteza
authors: Division of Cardiology, Johns Hopkins University, Baltimore, MD, United States.
authors: Joao A C
authors: JA
authors: Lima
authors: Department of Cardiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Spain.
authors: David
authors: D
authors: García-Dorado
authors: Department of Cardiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Spain.
authors: Artur
authors: A
authors: Evangelista
mesh_terms: D000293:Adolescent; D000328:Adult; D001012:Aorta, Abdominal / Q000473:pathology* / Q000502:physiology*; D001018:Aortic Diseases / Q000175:diagnosis* / Q000503:physiopathology; D001696:Biomechanical Phenomena / Q000502:physiology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D008875:Middle Aged; D014664:Vasodilation / Q000502:physiology*; D055815:Young Adult
keywords: Aortic distensibility; Magnetic resonance imaging; Marfan syndrome; Pulse wave velocity