Thoracic aortic endografting in patients with connective tissue diseases.

PURPOSE: To present midterm results after thoracic endovascular aortic repair (TEVAR) in patients with connective tissue diseases focusing on secondary endoleak and reintervention due to disease progression.

METHODS

Between January 1997 and January 2007, 167 patients received 241 thoracic aortic stent-grafts. Eight patients (6 men; median age 48 years, range 32-67) with connective tissue diseases (6 Marfan and 2 Ehlers-Danlos syndrome) treated with stent-graft repair were retrospectively analyzed at a median follow-up of 31 months (range 3-79). Surveillance included postoperative computed tomographic angiography and/or magnetic resonance imaging exams prior to discharge, at 3, 6, and 12 months, and yearly thereafter.

RESULTS

Technical success of endovascular placement was 88% due to 1 primary type I endoleak. There were no perioperative deaths, and there have been no conversions to open surgery so far. Perioperative complications occurred in 2 (25%) of the 8 patients. Endoleaks were observed in 3 patients (primary type I, secondary type I, and type II). The reintervention rate was 38%. Progression of disease resulting in de novo aneurysms or aortic expansion occurred in 4 (50%) patients. Seven (88%) patients are alive. There was no disease- or procedure-related death.

CONCLUSION

TEVAR in patients with connective tissue diseases is feasible but still questionable regarding their young age and the rates of endoleaks and reintervention due to disease progression. Close surveillance is mandatory. Low morbidity and mortality rates may justify TEVAR in emergencies as a "bridging" method.