Giant cell aortitis: clinical presentation and outcomes in 40 patients consecutively operated on.

Giant cell arteritis (GCA) may affect mid-size and large-size arteries. Although temporal arteritis is a well-characterized clinical entity, GCA of the thoracic aorta remains ill defined. The aim of the study was to evaluate the clinical presentation, surgical and mid-term outcomes in patients operated for GCA of the thoracic aorta.,A retrospective review of patients operated for GCA of the thoracic aorta was conducted. The diagnosis of GCA was established by the pathology report.,Forty consecutive patients (mean age of 66.6 ± 9.1 years) with a diagnosis of GCA of the thoracic aorta were operated on. A history of polymyalgia rheumatica or temporal arteritis was positive in 22.5% of patients. Emergency surgery was performed in 10% of patients (3 'type A' dissections and 1 'type B'). Mega-aorta syndrome was present in 10% of patients. Involvement of the ascending aorta was present in 100% of patients. One patient had a previous branched thoracic endovascular replacement (TEVAR) with a type I proximal endoleak. In 4 patients, the thoracic aorta was totally replaced. Eighty-five percent of patients had an arch replacement; 79.4% a hemiarch and 20.6% a full arch. The mean circulatory arrest time was 16.3 ± 12.3 min. Eighty percent of patients had an aortic valve procedure; aortic valve replacement was performed in 50% of them and Bentall-De Bono/valve sparing in 50%. Cerebrovascular accident occurred in 2.5% of patients. No patient died during hospitalization. The mean hospital stay was 8.7 ± 3.0 days. The mean postoperative follow-up time was 4.2 ± 2.3 years, with a mean of 4.2 ± 2.2 thoraco-abdominal computed tomographies (CTs)/patient. Four patients had late reinterventions: 2 were valve-related, 1 for a distal type I endoleak treated with a distal TEVAR extension and 1 type II open thoraco-abdominal replacement for disease progression. One distal type I TEVAR endoleak was treated medically. Aortic diameter progressions on CT (mm/year) were 0.7 ± 1.0 mm for the arch, 1.2 ± 2.0 mm for the isthmus, 1.1 ± 1.7 mm for the mid-descending, 0.7 ± 0.9 mm for the aortic hiatus, 0.5 ± 0.5 mm for the supra-renal aorta and 0.6 ± 0.6 mm for the infra-renal aorta. One patient who declined reoperation on the descending aorta died suddenly 3 years after her initial operation. The 5-year overall survival rate was 91%.,GCA of the thoracic aorta may be suspected in less than 25% of patients preoperatively. Clinical presentation may be acute or chronic with localized or diffused aortic involvement but always involved the ascending aorta. Surgery may be performed with excellent outcomes. Follow-up imaging is mandatory to assess aortic progression.