title: Distensibility and diameter of ascending aorta assessed by cardiac magnetic resonance imaging in adults with tetralogy of fallot or complete transposition.
abstract: Structural abnormalities of the medial aorta have been described for conotruncal defects (e.g., tetralogy of Fallot [TOF] and complete transposition of the great arteries (dextrotransposition [d]-TGA). In TOF, progressive aortic dilation is a frequent finding. In patients with d-TGA with an atrial switch, this problem is less often described. The aim of the present study was to compare the extent of dilative aortopathy and aortic distensibility in adults with an atrial switch procedure (n = 39) to that in adults with repaired TOF (n = 39) and controls (n = 39), using cardiac magnetic resonance imaging. The groups were matched for age and gender. Diameters of the aorta indexed to the body surface area were significantly increased in the patients with d-TGA and TOF compared to that of the controls at the aortic sinus up to the level of the right pulmonary artery. On multivariate testing, the diagnosis of a conotruncal defect (β = 0.260; p = 0.003) and aortic regurgitant fraction (β = 0.405; p <0.001) were independent predictors of an increased aortic sinus diameter. Ascending aorta distensibility was significantly reduced in those with d-TGA and TOF compared to controls: 3.6 (interquartile range 1.5 to 4.4) versus 2.8 (interquartile range 2.0 to 3.7) versus 5.5 (interquartile range 4.8 to 6.9) ×10(-3) mm Hg(-1) (p <0.001). The independent predictors of ascending aorta distensibility were the diagnosis of a conotruncal defect (p <0.001) and age (p = 0.028). In conclusion, intrinsic aortopathy, manifested as increased ascending aortic diameters and reduced ascending aortic distensibility, is not only evident in adults with TOF, but also in adults with d-TGA and an atrial switch procedure. Long-term follow-up is needed to monitor the aortic size in both patient groups.
authors.affiliation: Department of Cardiology and Pediatric Cardiology, University Hospital Inselspital, Bern, Switzerland.
authors.forename: Tobias
authors.initials: T
authors.lastname: Rutz
authors.forename: Friedrich
authors.initials: F
authors.lastname: Max
authors.forename: Andreas
authors.initials: A
authors.lastname: Wahl
authors.forename: Kerstin
authors.initials: K
authors.lastname: Wustmann
authors.forename: Kerstin
authors.initials: K
authors.lastname: Khattab
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Pfammatter
authors.forename: Alexander
authors.initials: A
authors.lastname: Kadner
authors.forename: Markus
authors.initials: M
authors.lastname: Schwerzmann
mesh_terms: D000293:Adolescent; D000328:Adult; D001013:Aorta, Thoracic / Q000473:pathology / Q000503:physiopathology*; D004548:Elasticity; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D008297:Male; D008875:Middle Aged; D012720:Severity of Illness Index; D013771:Tetralogy of Fallot / Q000175:diagnosis* / Q000503:physiopathology; D014188:Transposition of Great Vessels / Q000175:diagnosis* / Q000503:physiopathology; D059289:Vascular Stiffness / Q000502:physiology*; D055815:Young Adult