Abstract 20124: Aortopathy in Repaired Tetralogy of Fallot: Pattern of Enlargement, Continued Growth and Risk of Dissection - Should Surveillance Guidelines be Developed?
Background: Emerging data demonstrates aortopathy with repaired tetralogy of Fallot (rTOF) and recent case reports describe aortic dissection. Pattern of dilation, rate of change and factors leading to, and possibly protecting against progression and risk for dissection have not been reported. Therefore we sought to determine, with CMR, the pattern, progression, and factors of aortic enlargement. We calculated cross-sectional area to height ratios (CSA/Ht) as potential determinant those at risk for dissection and those requiring increase surveillance.
Methods: We reviewed our ACHD database for adults with rTOF and at least 1 MRA of the aorta. We measured in 2 planes the aortic sinuses (AoS) and ascending aorta (AsAo). CSA/Ht ≥ 10 considered at risk for dissection and ≥ 7 < 10 increase surveillance. Rate of change was determined for pts with >1 MRA. Multivariate and univariate analysis tested predictors; differences compared with Student's t test, and Fisher's exact test.
Results: We identified 87 pts (51% male), 78 with pulmonary stenosis (TOF-PS) and 8 with atresia (TOF-PA). TOF-PS 36 +/− 11 yrs were older vs TOF-PA 26 +/− 6 yrs. Overall, AoS were larger 3.6 +/ 0.5 vs AsAo 3.1 +/ 0.6 cm (p < 0.0001). However, AsAo were larger in TOF-PA 3.24 +/− 0.4 vs PS 3.07 +/− 0.4 cm. CSA/ht ratio ≥ 7 at AoS in 26% and AsAo in 13%, and ≥ 10 in 4.6% and 5.7%. Predictors included male, older age, right arch, prior pregnancy, older age at repair, prior shunt. We found 55 pts with > 1 MRA, avg of 3.3 yrs between studies. Ao size increased in 39/55 (71%), avg AoS 2.4mm and AsAo 2.2mm per yr. No differences in growth rate for TOF-PS vs PA. Univariate analysis: prior pregnancy and late age at repair predicted AoS enlarging. AoS CSA/Ht decreased over time with βB therapy.
Conclusion: In rTOF, aortopathy is well described and a risk for dissection. For the first time, the pattern, rate of growth, and predictors of Ao enlargement are reported. Over 70% enlarged over time, 26 % had a CSA/Ht ≥ 7 requiring close surveillance and 5% reached ≥10, which in aortopathy may be high risk for dissection. Currently, there are no guidelines for aortic follow-up with rTOF. Therefore, a more clear understanding of Ao enlargement with rTOF may identify high risk populations for screening and surveillance to prevent aortic complications.
- © 2010 by American Heart Association, Inc.