The following is the summary of “Long-term outcomes of primary aortic valve repair for isolated congenital aortic stenosis in children” published in the November 2022 issue of Thoracic and Cardiovascular Surgery by Wallace, et al.

Their study’s major objective was to evaluate the long-term results of primary aortic valve replacement in children born with isolated congenital aortic stenosis. Children (n=111) diagnosed with isolated congenital aortic stenosis and treated with primary aortic valve repair between 1980 and 2016 were analyzed. Successful surgery would have resulted in a peak systolic gradient in the left ventricular outflow tract of less than 35 mm Hg and little aortic insufficiency. The average patient was 7.0 kg (14.9 lb) and 0.4 years old (IQR: 1 month-7.9 years) (interquartile range, 3.7-25.0 kg). Patch-based aortic valve repairs were performed on 52 patients (46.8%; 52/111). 

The infant mortality rate was 0.9% (1/111). There was a 0.9% death rate due to complications in the late stages of life (1/110). At 10 years, 52.1% of patients did not require a second operation on their aortic valve (95% CI: 38.7-63.8). At 10 years, the rate of survival without aortic valve replacement was 67.9% (95% CI, 55.4-77.5). About 48 patients (43.2 %; 48/111 total) had a successful outcome. Patients who had a good outcome were less likely to need another aortic valve replacement after 10 years (78.2%; 95% CI; 63.1-87.8) than those who had a poor outcome (39.4%; 95% CI; 22.8-55.6; P=.03). The presence of a bicuspid aortic valve was linked to a worse prognosis (P=.01).

Congenital aortic stenosis can be treated with aortic valve repair, and outcomes are favorable both immediately after birth and in the long run, even for the youngest of patients. Nearly half of the patients needed a second aortic valve replacement procedure after 10 years, but the other two-thirds are still doing well without further surgery. Bicuspid aortic valves were more likely to result in a successful outcome than tricuspid aortic valves.