We aimed at evaluating the results of aortic valve-sparing root replacement (AVSRR) in children with aortic root aneurysm (ARA) due to genetic disorders in terms of mortality, reoperation and recurrent aortic valve regurgitation (AVR).Thirteen patients (mean age 9.7 ± 6.5 years, 10 months-18 years) underwent AVSRR for ARA between 2002 and 2011. Six of the 13 patients had Marfan syndrome, 3 Loeys-Dietz syndrome (LDS), 2 bicuspid aortic valve syndrome and 2 an unspecified connective tissue disorder. AVR was graded as none/trace, mild and severe in 5, 7 and 1 patient, respectively. The mean pre-operative root diameter was 45 ± 10 mm (mean Z-score 10.3 ± 2.0). Remodelling of the aortic root was performed in 4 patients, reimplantation of the aortic valve in 9 and a concomitant cusp repair in 4. The diameter of the prostheses used for root replacement varied from 22 to 30 mm (mean Z-score = 2.3 ± 3). The follow-up was 100% complete with a mean follow-up time of 3.7 years.There was no operative mortality. One patient with LDS died 2.5 years after the operation due to spontaneous rupture of the descending aorta. Root re-replacement with mechanical conduit was necessary in 1 patient for severe recurrent AVR 8 days after remodelling of the aortic root. At final follow-up, AVR was graded as none/trace and mild in all patients. Eleven patients presented in New York Heart Association functional Class I and 1 in Class II.In paediatric patients with ARA, valve-sparing root replacement can be performed with low operative risk and excellent mid-term valve durability. Hence, prosthetic valve-related morbidity may be avoided. Due to the large diameters of the aortic root and the ascending aorta, the size of the implanted root prostheses will not limit later growth of the native aorta.     «

We aimed at evaluating the results of aortic valve-sparing root replacement (AVSRR) in children with aortic root aneurysm (ARA) due to genetic disorders in terms of mortality, reoperation and recurrent aortic valve regurgitation (AVR).Thirteen patients (mean age 9.7 ± 6.5 years, 10 months-18 years) underwent AVSRR for ARA between 2002 and 2011. Six of the 13 patients had Marfan syndrome, 3 Loeys-Dietz syndrome (LDS), 2 bicuspid aortic valve syndrome and 2 an unspecified connective tissue disorde...     »