Objectives: Descending thoracic (TAA) and thoracoabdominal aortic aneurysms (TAAA) in patients <50 years are rare and often represent a manifestation of connective tissue disorders (CTD). The aim of this study was to evaluate outcomes of elective open TAA/TAAA repair in young patients to serve as a basis for comparison for potential future endovascular procedures.
Methods: Data from 527 consecutive patients who underwent elective TAA/TAAA repair from 1971 to 2007 were analyzed retrospectively. Forty-eight patients <50 years were identified. Patients with acute dissection were excluded.
Results: There were 32 males and 16 females (median age 40 years; range 18 -50) with 30 TAA, 2 Crawford Type I, 5 Type II, 10 Type III, and 1 Type IV TAAAs. Seventeen (37%) aneurysms were secondary to CTDs: Marfan’s (n=9), Ehlers-Danlos (n=2), Takayasu’s arteritis (n=3), Behcet’s (n=1), and Tuberous sclerosis (n=2); the remainder were atherosclerotic/degenerative (n=17), post-traumatic (n=12), or post-congenital coarctation repair (n=2). Two patients were pregnant at presentation. Mean aneurysm diameter was 6.5 cm. All patients underwent standard open surgical repair through a left thoracic/thoracoabdominal approach. There were no early post-operative deaths. Major complications developed in 13/48 (27%), including cardiac (n=3), renal (n=4), paraplegia/paraparesis (n=3), chylothorax (n=1), and cerebral ischemia (n=2). Median hospital length of stay was 8.5 days (range 6-44). Mean follow-up was 33 months (range 1-372 months). Twelve patients (25%) had prior aortic surgery. Fifteen (31%) patients required aortic re-intervention; paravisceral patch aneurysm repair (n=3), ascending or aortic arch repair (n=8) associated with 20% (3/15) mortality. On univariate analysis, the incidence of re-intervention was not significantly higher in patients with CTD. One, 5 and 10 year cumulative survival survival rates were 98%, 94% and 82% respectively.
Conclusion: Open TAAA repair in young patients is associated with favorable early and long-term outcome. Late occurrence of paravisceral patch aneurysms may contraindicate use of the visceral patch technique. A significant proportion of patients require further aortic intervention and long-term imaging surveillance is advised.