The Ross procedure is the procedure of choice for congenital aortic valve disease.

Al-Halees Z, Pieters F, Qadoura F, Shahid M, Al-Amri M, Al-Fadley F
Journal: J Thorac Cardiovasc Surg 2002;123:437-442

Reviewers: Teodora O. Nicolescu, MD
Jane C. K. Fitch, MD

The pulmonary autograft replacement of the aortic valve first introduced in 1967 has since revolutionized the lives of many children. Being accomplished originally as a scalloped subcoronary implant it was similar to the operative procedure for implementation of an aortic homograft. Due to its viability and excellent hemodynamics the pulmonary autograft has proven to be uniquely suited for children. Its growth potential has made it even more attractive for use in children adding to its umbrella of other features such as freedom from embolic events and lack of coagulation.

These factors have allowed children with a Ross operation to have a near normal lifestyle except for medical surveillance and a limited need for continuing medical care. Prosthetic replacement incurs the risk of early calcification and lifelong anticoagulation with its drawbacks. Pulmonary homograft degeneration has been proven to possibly represent an immune response. Recently reports delineating the humoral immune response to cryopreserved homografts homovital homografts have been associated with an HLA antibody specific to HLA antigens of the homograft. In patients with a class 2 Dr mismatch with two class 1 B antigen mismatch accelerated homograft degeneration has been demonstrated. Although emerging as a more and more frequently offered procedure the Ross procedure may be complicated in the long run by autograft degeneration (defined as reoperation for autograft failure) , severe autograft valve aortic valve diseases that represent still either contraindications or controversies for the use of Ross procedure, autograft valve replacement remains an excellent choice in children. Almost all aortic valve disease, complex LVOT obstruction and associated ascending aortic pathology can be managed with modifications of the Ross operation. The potential for growth (as demonstrated by Dr Al Halees's study by serial measurements of aortic root and LVOT) will still remain in debate since it is still unclear if it represents growth or dilatation. Some studies show an initial dilation followed by real growth. Still, the decreased need for reoperations as well as excellent hemodynamics of the autograft make the Ross procedure the choice for children with aortic valve disease.

In conclusion despite some of the congenital insuffiency and need for reoperation for other reasons. Its use in case of rheumatic fever children as well as in colagen deficiencies related diseases (such as Marfan syndrome, Ehlers-Danlos syndrome or osteogenesis imperfecta has given place to lots of debates. Also patients with severe juvenile rheumatoid arthritis have been reported with early degeneration of the autograft making the Ross procedure unlikely to be a choice for this subset (unless they already have had the disease and are maintained on good prophylaxis). Diseases such as Reiter's ankylosing spondylitis also represent for a number of surgeons still contraindications for the Ross procedure, due to the reccurence of disease on the pulmonary valve in the aortic position.

Concern has been expressed also regarding use of the Ross procedure in patients with bicuspid aortic disease. It has been suggested that these patients may have a genetic defect that involves the aortic and pulmonary artery wall and may lead to long-term complications if the pulmonary root is used as a valved conduit to replace the aortic wall. An increased incidence of aortic dissection of 5-10% has been associated with bicuspid aortic valve disease and bicuspid aortic valve disease has been associated with sudden death in the absence of aortic dissection in young patients. These findings have been suggested as contraindications to the use of autograft root replacement in patients with bicuspid aortic valve disease. Lastly the Ross procedure is not likely to be offered to the patients with pure aortic regurgitation, a subgroup that behaves differently than those with pure aortic stenosis or mixed aortic stenosis - insufficiency. Patients with pure aortic regurgitation demonstrate progressive dilatation of the aortic root and a higher incidence of early failure.

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