Aortic Valve-Sparing Operations for Aortic Root Aneurysms in Grown-up Patients with Congenital Heart Disease

Objective: Aortic root aneurysms are unusual in children or patients with congenital heart disease and have received little attention to develop a best approach for management. In this study we analyzed our experience with aortic valve-sparing operations in grown-up patients with congenital heart disease.

Methods: From 2006 to 2017, twenty-four patients underwent aortic valve-sparing operations due to aortic root aneurysms in our unit for congenital heart surgery. In this study we included the 8 patients with a history of surgery for congenital heart defects. Patients (n = 16) with connective tissue disorders or another etiology of aortic aneurysms were excluded. The underlying diseases were: tetralogy of Fallot (TOF) n = 4, coarctation of the aorta (CoA) n = 2, transposition of great arteries (TGA) n = 1 and pulmonary atresia with VSD n = 1. One patient with CoA had a bicuspid aortic valve. The age of the patients ranged from 19 to 51 years (mean age: 33 ± 9.3 years), and 7 of them were male. The mean diameter of aortic aneurysm was 50 ± 3.1 mm. Regurgitation of the aortic valve was mild in most cases and not the leading reason for the procedure.

Results: David re-implantation was performed in 7 patients, Yacoub remodeling in 1 patient. 4 patients (50%) obtained simultaneously pulmonary valve replacement (Contegra graft n = 1, Hancock conduit n = 1, decellularized pulmonary homografts n = 2). Mean bypass time was 267.5 ± 84 minute, x-clamp time 123 ± 46 minute. There was no operative or hospital mortality and no reoperations so far. During follow-up aortic valve regurgitation was mild or less. Good biventricular heart function was present in all patients, NYHA functional class was I for all at latest follow up (mean: 60 ± 45 months, range: 1–132 months).

Conclusion: Valve-sparing aortic root replacement is accomplished with promising results and stable aortic valve function during the first decade of observation. This approach can be a viable alternative to root replacement with mechanical or biological prostheses in this cohort of young patients.