American Association of Plastic Surgeons

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Long-term Surgical Prognosis of Primary Supravalvular Aortic Stenosis Repair
Fei-Yi Wu, MD, Abhijit Mondal, Ph.D, Pedro J. del Nido, MD, Kimberlee Gauvreau, Sitaram M. Emani, MD, Christopher W. Baird, MD, Aditya K. Kaza, MD.
Boston Children's Hospital, Boston, MA, USA.

Objective(s): Supravalvular aortic stenosis (SVAS) represents a heterogeneous group, including Williams syndrome, familial elastin arteriopathy, sporadic cases and others. In this study we sought to evaluate long-term outcomes of primary SVAS repair.
Methods: A total of 87 patients underwent surgical repair of congenital SVAS at our institution between 1997 and 2017. 41 patients had Williams syndrome and 46 patients were non-Williams syndrome of which 23 sporadic SVAS and 13 familial elastin arteriopathy. Demographic data and outcomes were reviewed and analyzed from medical records.
Results: The median age at operation was 2.9 years. Mean z-score of sinotubular junction (STJ) was -3.29 ± 1.42 and aortic root was -0.09 ± 1.19. 5-year, 10-year and 20-year survival rates were all 94.3%. Freedom from left ventricular outflow tract (LVOT) reoperation at 5 years, 10 years and 20 years was 78.5%, 70.3%, 70.3%, respectively. Freedom from aortic arch reintervention at 5 years, 10 years and 20 years was 98.6%, 94.3%, 89.3%, respectively. 26.3% (n=22) patients had coronary ostium stenosis and 41% (n=9) of them required patch plasty. In univariate analysis, age < 1 year and z-score of aortic root were predictive of the need for any intervention and reintervention for LVOTO/RVOTO. Stepwise multivariable logistic regression confirmed that age and z-score of aortic root were the only two independent risk factors for future arch reintervention or reoperation.
Conclusions: Excellent long-term survival rates can be achieved with surgical repair of SVAS. Age < 1 year and small aortic root z-score were predictors of reintervention.


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