Coronary artery vasculopathy in pediatric cardiac transplant patients: the therapeutic potential of immunomodulators.

The single largest cause of late graft loss in pediatric cardiac transplantation is transplant coronary artery vasculopathy (CAV). The mechanism of CAV remains unknown; it appears to have both immune and non-immune causes. The final common pathway of these mechanisms is endothelial activation, a prothrombotic environment, and endothelial damage with subsequent diffuse intimal proliferation. The disease process has largely been thought to be progressive and unresponsive to treatment. Re-transplantation has been advocated as the only definitive treatment. The appropriate management is largely unknown; intervention or surgical management has had limited utility, while medical management appears to have the most promise. Improvement in outcome can be achieved by optimizing non-immune factors and aggressive management of the immune mechanisms. Long-term survival of transplant patients after diagnosis with CAV is now being reported.