Ascending aorta replacement (AAR) is a rare surgical procedure required in the paediatric population. The classical indication is infantile connective tissue diseases, but AAR is also increasingly performed after neonatal repairs of congenital heart diseases.

Between 1995 and 2017, 32 infants under the age of 10 years underwent AAR procedures at our institution. Twenty-one of them (66%) had a connective tissue disease (Infantile Marfan Syndrome IMS or Loeys-Dietz Syndrome) while the rest had undergone a neonatal surgery including aortic valve or root. Ten of all (31%) associated a bicuspid aortic valve. The median age and the median weight were respectively 5.3 years (range, 7 months to 9.7 y) and 18kg (range, 5.8 to 55kg). Median follow-up for operative survivors was 7.7 years (IQR 4.2–12.8 y, up to 23 y, 100% complete).

Irrespective of the size of the aortic aneurysm at the time of surgery, we performed 11 AAR using a composite graft with mechanical prosthesis (median size 21mm) and 21 valve-sparing procedures (10 Yacoub operations and 11 David operations). Cardiac-related mortality was 6% and was significantly associated with the Z-score of the aortic root (9.3±1 vs. 6.7±1.8, P=0.048). Both the one-year and ten-year patient survivals were 91%. Eleven of survivors (38%) required a reintervention after a median time of 4.2 years. All of these latter had undergone an initial valve-sparing procedure. Interestingly, after multivariate logistic regression analysis, only IMS condition remained correlated with the over-risk of reoperation (P=0.026).

Ascending aorta replacement independently of the surgical procedure is safe in infants, especially when it is performed before giant aortic root dilatation. In IMS, our data suggest that composite graft use with mechanical prosthesis should be preferred to limit the risk of reoperation related to aortic valve regurgitation.