Endovascular treatment of acute and chronic aortic pathology in patients with Marfan syndrome.

BACKGROUND: In patients with Marfan syndrome, the complications of aortic degeneration, including dissection, aneurysm, and rupture represent the main cause of mortality. Although contemporary management of ascending aortic disease requires open surgical reconstruction, endovascular repair is now available for management of descending thoracic and abdominal aortic pathology (ie, thoracic endovascular aortic repair [TEVAR], endovascular aneurysm repair [EVAR]). The short- and long-term benefit of endovascular repair in Marfan patients remains largely unproven. We examine our outcomes after EVAR in this patient population.

METHODS: All patients with a diagnosis of Marfan syndrome who were treated with TEVAR/EVAR were evaluated in a retrospective review. Perioperative, procedure-specific and patient covariate data were aggregated. Primary endpoints were overall mortality and procedural success as divided into three categories: (1) successful therapy, (2) primary failure, or (3) secondary failure.

RESULTS: Between 2000 and June 2010, 16 patients were identified as having undergone 19 TEVAR/EVAR procedures. These included three emergent operations (two for acute dissection/malperfusion and one for anastomotic disruption early after open repair). All 16 patients had previously undergone at least one (range, 1-5) open operation of the ascending aorta or arch at a time interval from 33 years to 1 week prior to the index endovascular repair. During a median follow-up of 9.3 months (range, 0-46 months), there were four deaths (25%). Six patients (38%) had successful endovascular interventions. Despite early success, there was one death in this group at 1 month postintervention. Seven patients (44%) experienced primary treatment failure with five undergoing open conversion and one undergoing left subclavian coil embolization (the seventh was lost to follow-up and presented 4 months later in cardiac arrest and expired without repair). There were three deaths in the primary treatment failure group. Two patients experienced secondary treatment failure. One underwent the index TEVAR for acute dissection with malperfusion and required a subsequent TEVAR for more distal aortic pathology. He is stable without disease progression. The other patient underwent open conversion after a second EVAR with four-vessel "chimney" stent grafts and is stable with his entire native aorta having been replaced.

CONCLUSIONS: Aortic disease associated with Marfan syndrome is a complex clinical problem and many patients require remedial procedures. Endovascular therapy can provide a useful adjunct or bridge to open surgical treatment in selected patients. However, failure of endovascular therapy is common, and its use should be judicious with close follow-up to avoid delay if open surgical repair is required.