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Analysis of the aortic root in patients with tetralogy of Fallot undergoing early repair: form follows function

Katrien Francois (UGent) , David Creytens (UGent) , Katya De Groote (UGent) , Joseph Panzer (UGent) , Kristof Vandekerckhove (UGent) , Daniël De Wolf (UGent) and Thierry Bové (UGent)
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Abstract
Objective: Aortic root dilatation remains of concern late after repair for tetralogy of Fallot (ToF). We have previously demonstrated that the aortic root size decreases progressively after early correction. To test whether the aortic wall changes are intrinsic or acquired, a histologic study was performed of infants at early repair. Methods: From 24 of 31 infants undergoing ToF repair (mean age, 5.9 +/- 4.9 months), ascending aortic biopsies were obtained. After conventional staining, the specimens were given a histologic score (0-3) for medionecrosis, ground substance accumulation, fibrosis, smooth muscle cell disarray, and apoptosis. The aortic root diameters were monitored using echocardiography for <= 2 years postoperatively. Results: Nearly one half of the specimens showed signs of fibrosis (45%), with increasedmucoid accumulation in 15% and elastin fragmentation in 5%. The aortic root was dilated in all infants at operation (mean indexed diameter-annulus, 38.1 +/- 5.8; sinus, 47.8 +/- 6; sinotubular junction, 38.7 +/- 5.4 mm/m(2)) and had regressed significantly within 2 years (mean indexed diameter-annulus, 27.9 +/- 4.7; sinus, 36.4 +/- 5.1; sinotubular junction, 30.4 +/- 6 mm/m(2); P < .0001). The total histology score showed no correlation with indexed diameter regression at 2 years. Conclusions: Infants with ToF undergoing repair around 6 months old already had limited features of aortic wall degradation at surgery. Echocardiographic follow-up, however, demonstrated significant reductions in the indexed aortic diameters. We presume that early repair of ToF will prevent a disproportionate aortic diameter increase by halting progression of the limited histologic changes potentially predisposing to late aortic dilatation.
Keywords
CHILDREN, STIFFNESS, DILATATION, ASCENDING AORTA, PULMONARY ATRESIA, ABNORMALITIES, DILATION, ADULTS

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MLA
Francois, Katrien et al. “Analysis of the Aortic Root in Patients with Tetralogy of Fallot Undergoing Early Repair: Form Follows Function.” JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY 148.4 (2014): 1555–1559. Print.
APA
Francois, K., Creytens, D., De Groote, K., Panzer, J., Vandekerckhove, K., De Wolf, D., & Bové, T. (2014). Analysis of the aortic root in patients with tetralogy of Fallot undergoing early repair: form follows function. JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY, 148(4), 1555–1559.
Chicago author-date
Francois, Katrien, David Creytens, Katya De Groote, Joseph Panzer, Kristof Vandekerckhove, Daniël De Wolf, and Thierry Bové. 2014. “Analysis of the Aortic Root in Patients with Tetralogy of Fallot Undergoing Early Repair: Form Follows Function.” Journal of Thoracic and Cardiovascular Surgery 148 (4): 1555–1559.
Chicago author-date (all authors)
Francois, Katrien, David Creytens, Katya De Groote, Joseph Panzer, Kristof Vandekerckhove, Daniël De Wolf, and Thierry Bové. 2014. “Analysis of the Aortic Root in Patients with Tetralogy of Fallot Undergoing Early Repair: Form Follows Function.” Journal of Thoracic and Cardiovascular Surgery 148 (4): 1555–1559.
Vancouver
1.
Francois K, Creytens D, De Groote K, Panzer J, Vandekerckhove K, De Wolf D, et al. Analysis of the aortic root in patients with tetralogy of Fallot undergoing early repair: form follows function. JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY. 2014;148(4):1555–9.
IEEE
[1]
K. Francois et al., “Analysis of the aortic root in patients with tetralogy of Fallot undergoing early repair: form follows function,” JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY, vol. 148, no. 4, pp. 1555–1559, 2014.
@article{5935635,
  abstract     = {Objective: Aortic root dilatation remains of concern late after repair for tetralogy of Fallot (ToF). We have previously demonstrated that the aortic root size decreases progressively after early correction. To test whether the aortic wall changes are intrinsic or acquired, a histologic study was performed of infants at early repair. 
Methods: From 24 of 31 infants undergoing ToF repair (mean age, 5.9 +/- 4.9 months), ascending aortic biopsies were obtained. After conventional staining, the specimens were given a histologic score (0-3) for medionecrosis, ground substance accumulation, fibrosis, smooth muscle cell disarray, and apoptosis. The aortic root diameters were monitored using echocardiography for <= 2 years postoperatively. 
Results: Nearly one half of the specimens showed signs of fibrosis (45%), with increasedmucoid accumulation in 15% and elastin fragmentation in 5%. The aortic root was dilated in all infants at operation (mean indexed diameter-annulus, 38.1 +/- 5.8; sinus, 47.8 +/- 6; sinotubular junction, 38.7 +/- 5.4 mm/m(2)) and had regressed significantly within 2 years (mean indexed diameter-annulus, 27.9 +/- 4.7; sinus, 36.4 +/- 5.1; sinotubular junction, 30.4 +/- 6 mm/m(2); P < .0001). The total histology score showed no correlation with indexed diameter regression at 2 years. 
Conclusions: Infants with ToF undergoing repair around 6 months old already had limited features of aortic wall degradation at surgery. Echocardiographic follow-up, however, demonstrated significant reductions in the indexed aortic diameters. We presume that early repair of ToF will prevent a disproportionate aortic diameter increase by halting progression of the limited histologic changes potentially predisposing to late aortic dilatation.},
  author       = {Francois, Katrien and Creytens, David and De Groote, Katya and Panzer, Joseph and Vandekerckhove, Kristof and De Wolf, Daniël and Bové, Thierry},
  issn         = {0022-5223},
  journal      = {JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY},
  keywords     = {CHILDREN,STIFFNESS,DILATATION,ASCENDING AORTA,PULMONARY ATRESIA,ABNORMALITIES,DILATION,ADULTS},
  language     = {eng},
  number       = {4},
  pages        = {1555--1559},
  title        = {Analysis of the aortic root in patients with tetralogy of Fallot undergoing early repair: form follows function},
  url          = {http://dx.doi.org/10.1016/j.jtcvs.2013.11.044},
  volume       = {148},
  year         = {2014},
}

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