Michal Schäfer1, Lorna P Browne2, Gareth J Morgan3, Alex J Barker2, Brian Fonseca3, D Dunbar Ivy3, Max B Mitchell4. 1. Division of Cardiology, Heart Institute, Children's Hospital Colorado, University of Colorado Denver | Anschutz Medical Campus, Aurora, Colo; Department of Bioengineering, College of Engineering and Applied Sciences, University of Colorado Denver | Anschutz Medical Campus, Aurora, Colo. Electronic address: michal.schafer@ucdenver.edu. 2. Department of Radiology, Children's Hospital Colorado, University of Colorado Denver | Anschutz Medical Campus, Aurora, Colo. 3. Division of Cardiology, Heart Institute, Children's Hospital Colorado, University of Colorado Denver | Anschutz Medical Campus, Aurora, Colo. 4. Section of Congenital Heart Surgery, Heart Institute, Children's Hospital Colorado, University of Colorado Denver | Anschutz Medical Campus, Aurora, Colo.
Abstract
OBJECTIVE: Patients with tetralogy of Fallot are at risk for late aortic complications due to progressive aortic root dilation and decreased aortic compliance. Early repair normalizes aortic dimensions by preadolescence. It is not known if early repair normalizes aortic wall histology and compliance or reduces late aortic complications. We used 4-dimensional flow magnetic resonance imaging to determine if children with tetralogy of Fallot repaired in infancy had normal aortic dimensions and to characterize the aortic wall hemodynamic state and luminal flow parameters in these patients. METHODS: Comprehensive aortic analysis with 4-dimensional flow magnetic resonance imaging was performed in 18 patients with tetralogy of Fallot who were repaired in infancy and compared with 18 normal volunteers. Peak systolic and time-averaged wall shear stress, relative area change, and distensibility were evaluated in standardized aortic planes. Qualitative grade scale flow analysis with interactive pathline visualization was used to detect pathologic flow patterns. RESULTS: Thoracic aortic dimensions did not differ between groups, and all tetralogy of Fallot aortas were in normal range. In the tetralogy of Fallot group, ascending and descending aortic relative area change and distensibility were significantly reduced, and both peak systolic and time-averaged wall shear stress were elevated throughout the aorta. Supra-physiologic systolic helical formations occurred in the ascending aorta of 14 patients with tetralogy of Fallot (78%) versus 0 controls. CONCLUSIONS: Despite early repair and normal aortic dimensions, preadolescents and adolescents with tetralogy of Fallot had elevated wall shear stress, increased stiffness, and pathologic systolic flow formations in the proximal aorta. Although early repair normalizes aortic dimensions in childhood, our findings suggest that patients with tetralogy of Fallot remain at risk for late aortic complications.
OBJECTIVE:Patients with tetralogy of Fallot are at risk for late aortic complications due to progressive aortic root dilation and decreased aortic compliance. Early repair normalizes aortic dimensions by preadolescence. It is not known if early repair normalizes aortic wall histology and compliance or reduces late aortic complications. We used 4-dimensional flow magnetic resonance imaging to determine if children with tetralogy of Fallot repaired in infancy had normal aortic dimensions and to characterize the aortic wall hemodynamic state and luminal flow parameters in these patients. METHODS: Comprehensive aortic analysis with 4-dimensional flow magnetic resonance imaging was performed in 18 patients with tetralogy of Fallot who were repaired in infancy and compared with 18 normal volunteers. Peak systolic and time-averaged wall shear stress, relative area change, and distensibility were evaluated in standardized aortic planes. Qualitative grade scale flow analysis with interactive pathline visualization was used to detect pathologic flow patterns. RESULTS: Thoracic aortic dimensions did not differ between groups, and all tetralogy of Fallot aortas were in normal range. In the tetralogy of Fallot group, ascending and descending aortic relative area change and distensibility were significantly reduced, and both peak systolic and time-averaged wall shear stress were elevated throughout the aorta. Supra-physiologic systolic helical formations occurred in the ascending aorta of 14 patients with tetralogy of Fallot (78%) versus 0 controls. CONCLUSIONS: Despite early repair and normal aortic dimensions, preadolescents and adolescents with tetralogy of Fallot had elevated wall shear stress, increased stiffness, and pathologic systolic flow formations in the proximal aorta. Although early repair normalizes aortic dimensions in childhood, our findings suggest that patients with tetralogy of Fallot remain at risk for late aortic complications.
Authors: Michal Schäfer; Alex J Barker; James Jaggers; Gareth J Morgan; Matthew L Stone; Uyen Truong; Lorna P Browne; Ladonna Malone; D Dunbar Ivy; Max B Mitchell Journal: Eur J Cardiothorac Surg Date: 2020-03-01 Impact factor: 4.191
Authors: Daniel McLennan; Michal Schäfer; Alex J Barker; Max B Mitchell; Richard J Ing; Lorna P Browne; D Dunbar Ivy; Gareth J Morgan Journal: Eur Radiol Date: 2022-07-19 Impact factor: 7.034
Authors: Michal Schäfer; Alex J Barker; Gareth J Morgan; James Jaggers; Matthew L Stone; Lorna P Browne; D Dunbar Ivy; Max B Mitchell Journal: Int J Cardiovasc Imaging Date: 2020-01-06 Impact factor: 2.357
Authors: Maria Victoria Ordonez; Sandra Neumann; Massimo Caputo; Stephanie Curtis; Giovanni Biglino Journal: Front Pediatr Date: 2021-03-04 Impact factor: 3.418
Authors: Niky Ghorbani; Vivek Muthurangu; Abbas Khushnood; Leonid Goubergrits; Sarah Nordmeyer; Joao Filipe Fernandes; Chong-Bin Lee; Kilian Runte; Sophie Roth; Stephan Schubert; Sebastian Kelle; Felix Berger; Titus Kuehne; Marcus Kelm Journal: BMJ Open Date: 2020-03-25 Impact factor: 2.692