Thirty years of experience with the Ross operation in children with aortic valve disease and complex LVOT obstruction: Results of a multi-center study

Objective: Progressive autograft and homograft dysfunction with need for later reoperation remain major concerns of the Ross procedure. The aim of the study is to document the outcomes into the third decade after the Ross operation in infants and children. For this purpose, a multi-center study was conducted. Methods: 325 children underwent Ross operation in 10 paediatric units between 1990 and December 2020. Median age at surgery was 10 (IQR 6-15) with 32 patients (10%) under 1 year of life. Indication was aortic stenosis in 114 patients, regurgitation in 116 and mixed lesion in 109. 116 (34%) patients had prior procedures. Root replacement was performed in 223 patients, inclusion cylinder in 33, subcoronary grafting in 20 and Ross-Konno in 63. The primary objective is to analyze the survival rate and re-operation rate on the left and right heart. The secondary objective is to identify risk factors for autograft and homograft failure. Results: There were 7 (2.1%) hospital and 14 late deaths with a median follow-up time of 11 years (IQR: 4,2-17). Survival was 91,6±3,3% and 86%±7% and freedom from any reoperation was 56±10% and 44±13% at 20 and 25 years. 39 (12%) patients had autograft reoperations (10 root replacement, 14 root replacement with valve preservation in 8, ascending aortic replacement in 2 and valve repair in 5). Freedom from autograft reoperation was 75±8% and 70%±11% at 20 and 25 years. 58 children had right heart redo procedures: 6 balloon dilation, 25 trans-catheter pulmonary valve implantation and 26 surgical pulmonary homograft valve replacement (20-year freedom from replacement, 75±5,6%). Prior operation (p=0.031), subcoronary implant (p=0.03) was risk factors for left heart reoperation, while infant age (p=0.001), aortic stenosis as indication to Ross procedure (p=0,049) and use of root technique (p=0.045) were for right heart. The majority (70%) of late survivors were in NYHA class I, 76% free from medication. Conclusions: Ross procedure is a low risk operation for isolated aortic valve or complex left heart obstruction in children, with satisfactory early and late survival. When possible, the Ross operation should be avoided in infants and post-pond after 12 months of age due to the high mortality in this sub-group of patients. In these patients an initial strategy of aortic surgical valvulotomy associated with valve repair should be performed if possible.