Long-term outcome after surgical intervention and interventional procedures for the management of Takayasu's arteritis in children.

Objective: There is little information available on the results of reconstructive arterial surgery for Takayasu's arteritis in children. This study evaluates midterm to long-term outcome after surgical, as well as interventional, procedures.

Methods: From 1984 through 2004, 10 children (5 boys and 5 girls; age, 12.7 +/- 2.6 years) with Takayasu's arteritis were referred to our center. Baseline cardiovascular assessment included contrast angiography (n = 4), magnetic resonance imaging-angiography (n = 3), and combined contrast angiography plus magnetic resonance imaging-angiography (n = 3). Two patients had disease confined to the thoracic aorta, 4 had disease confined to the abdominal aorta, and 4 had combined thoracoabdominal aortic disease. Steno-occlusive lesions were predominant in 92% of cases. Seven patients were maintained on steroid therapy throughout the follow-up period. Eight children underwent complex surgical procedures, 1 patient had balloon dilatation of the renal and mesenteric arteries, and 1 patient had combined vascular surgery with percutaneous transluminal angioplasty. Overall, 24 grafts (polytetrafluoroethylene, Dacron grafts, and cryopreserved homografts) were implanted in various locations.

Results: There were no perioperative deaths. Arterial hypertension regressed in all patients, and cardiac function normalized in all 4 patients with dilative cardiomyopathy. Over a 20-year period, 1 patient presented with sudden death and 2 showed nonfatal disease progression, one of whom required surgical reintervention. The occlusion rate was higher in Dacron grafts.

Conclusions: Our study shows that despite the extent and severity of vascular lesions, children with Takayasu's arteritis could benefit from reconstructive surgery, with low mortality, morbidity, and satisfactory long-term results.