Aortopathy in adults with tetralogy of Fallot has a negative impact on the left ventricle

Shiina, Yumi; Murakami, Tomoaki; Kawamatsu, Naoto; Niwa, Koichiro

doi:10.1016/j.ijcard.2016.11.252

Cited by 12 publications

(8 citation statements)

References 31 publications

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“…Indeed, ACEI 27 and ARB 28 may further benefit our patients through reduction of the aortic stiffness. While we did not find associations between indices of aortic elastic properties and the circulating levels of TGF-β1 and MMPs in patients as a group, the adverse effects of arterial stiffening on aortic root dilation and ventricular dysfunction have been shown in the present and previous studies 29–31 .…”

Section: Discussioncontrasting

confidence: 87%

Circulating Transforming Growth Factor-β and Aortic Dilation in Patients with Repaired Congenital Heart Disease

Cheung

Chan

2019

Sci Rep

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This study determined the circulating levels of TGF-β1 and its association with aortic dilation and elastic properties in congenital heart patients. Forty-six patients after tetralogy of Fallot (TOF) repair, 21 patients post arterial switch and 15 patients post atrial switch for transposition of the great arteries (TGA), 27 patients post Fontan procedure, and 36 controls were studied. Aortic dimensions and elastic properties and ventricular function were assessed by echocardiography. Serum TGF-β1, metalloproteinase (MMP)-2 and MMP-9 levels were quantified. Compared with controls, all groups of patients had significantly larger ascending aortic dimensions and worse elastic properties (all p < 0.05). Aortic stiffness correlated positively with sinus dimension (r = 0.48, p < 0.001) and negatively with indices of ventricular deformation (all p < 0.001). Patients with repaired TOF had significantly higher levels of TGF-β1 (p = 0.005), MMP-2 (p = 0.001) and MMP-9 (p < 0.001) than controls, while patients after atrial switch operation (p = 0.034) and Fontan procedures (p < 0.001) had higher MMP-2 levels. In patients as a group, circulating TGF-β1 levels correlated with MMP-9 (r = 0.44, p < 0.001) and aortic sinus dimension (r = 0.22, p = 0.035). In conclusion, increased circulating TGF-β1, MMP-2, and MMP-9 levels were found in patients with repaired TOF, and increased circulating MMP-2 levels were also evident in patients after atrial switch operation and Fontan procedure.

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Section: Discussioncontrasting

confidence: 87%

Circulating Transforming Growth Factor-β and Aortic Dilation in Patients with Repaired Congenital Heart Disease

Cheung

Chan

2019

Sci Rep

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“…2,14 Excess aortic stiffness also increases afterload negatively affecting left ventricular (LV) function, a phenomenon demonstrated even in young children. 9,10,13 Histopathology in TOF-associated aortopathy closely mirrors Marfan's syndrome and other aortopathies. 2,4,15,16 Various degrees of medial degeneration are found and have been identified in unrepaired neonates and infants.…”

Section: Perspectivementioning

confidence: 86%

“…2,26 From a population vantage point, the most concerning adverse effect of TOF-associated aortopathy results from the obligatory increase in LV afterload that accompanies elevated aortic stiffness. 1,9,10 Increased afterload results in increased LV mass and LV dysfunction in adults late after TOF repair. 9 Increased aortic wall stiffness also negatively affects LV ejection, a phenomenon that has been demonstrated in repaired patients with TOF as young as 6 years of age.…”

Section: Discussionmentioning

confidence: 99%

“…1,9,10 Increased afterload results in increased LV mass and LV dysfunction in adults late after TOF repair. 9 Increased aortic wall stiffness also negatively affects LV ejection, a phenomenon that has been demonstrated in repaired patients with TOF as young as 6 years of age. 10 Progressive aortic enlargement is more common when complete TOF repair is delayed.…”

Section: Discussionmentioning

confidence: 99%

“…8 Previous studies report that 88% of preadolescent and adolescent patients with TOF repaired at a mean age of 3.2 years had significant aortic root dilation (z-score >2), and these patients had increased proximal aortic stiffness compared with controls. 6 By using noninvasive 6,9 and catheterization 8,10,11 methods, other investigators demonstrated increased aortic stiffness in patients with TOF and correlated aortic dilation with increased stiffness. Aortic dilation and elevated wall stiffness are risk factors for life-threatening complications in aortopathies associated with bicuspid aortic valve, coarctation, and Marfan's syndrome.…”

Section: Perspectivementioning

confidence: 99%

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Reduced proximal aortic compliance and elevated wall shear stress after early repair of tetralogy of Fallot

Schäfer

Browne

Morgan

et al. 2018

The Journal of Thoracic and Cardiovascular Surgery

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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.

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Aortic Vorticity, Helicity, and Aortopathy in Adult Patients with Tetralogy of Fallot: Pilot Study Using Four-Dimensional Flow Magnetic Resonance Images

Shiina

Inai

Miyazaki³

et al. 2020

Pediatr Cardiol

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Aortopathy in adults with tetralogy of Fallot has a negative impact on the left ventricle

Cited by 12 publications

References 31 publications

Circulating Transforming Growth Factor-β and Aortic Dilation in Patients with Repaired Congenital Heart Disease

Circulating Transforming Growth Factor-β and Aortic Dilation in Patients with Repaired Congenital Heart Disease

Reduced proximal aortic compliance and elevated wall shear stress after early repair of tetralogy of Fallot

Aortic Vorticity, Helicity, and Aortopathy in Adult Patients with Tetralogy of Fallot: Pilot Study Using Four-Dimensional Flow Magnetic Resonance Images

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