Is aortic valve-sparing root replacement effective in pediatric patients with aortic root aneurysm?

Objectives: We aimed at evaluating the results of aortic valve-sparing root replacement (AVSRR) in children with aortic root aneurysm (ARA) and aortic regurgitation (AVR) in terms of mortality, reoperation and recurrent AVR.

Methods: Between 2002 and 2011, twelve patients (mean age 9.7±6.5 years, 10 months-18 years) underwent AVSRR for ARA. Six out of 12 patients had Marfan syndrome, three Loeys-Dietz-Syndrome, and three unspecified connective tissue disorders. AVR was graded as ≤trace, mild and severe in five, six and one patient, respectively. Mean preoperative root diameter was 45±9.6mm (mean Z-score=10.6±2.1). Remodeling of the aortic root was performed in four patients, reimplantation of the aortic valve in eight and a concomitant cusp repair in three. The diameter of the used root prosthesis varied from 22–30mm (mean Z-score=2.9±3). The follow-up (echocardiography, examination) was complete with a mean follow-up time of four years (5months-10years).

Results: There was no operative mortality. One patient died 2.5 years after the operation due to descending aorta rupture. At primary operation this patient was one year old and diagnosed with Loeys-Dietz-Syndrome. Root rereplacement with mechanical conduit was necessary in one patient for severe recurrent AVR eight days after remodelling of the aortic root without annular reinforcement. At final follow-up AVR was graded as ≤mild in all patients. Ten patients presented in NYHA functional class I and one in class II.

Conclusions: AVSRR can be performed without operative mortality and good durability in pediatric patients with ARA and AVR. The used root prostheses are not a limitation for the growing aorta. Thus, reoperation, prosthetic valve implantation and prosthetic valve related morbidity may be avoided.