Results after thoracic aortic reoperations in Marfan syndrome.

BACKGROUND

Reoperations on the aortic root and distal thoracic aorta late after initial root surgery in patients with Marfan syndrome might carry high periprocedural risks and be associated with adverse early and midterm clinical outcome.

METHODS

Overall clinical follow-up was 13.0±7.6 years and 3.7±3 years after secondary aortic procedures. Cumulative follow-up was a total of 148 patient-years. Kaplan-Meier, log-rank, and multiple logistic regression calculations were performed to identify risk factors for mortality.

RESULTS

Of 122 patients with Marfan syndrome who underwent aortic root surgery from 1998 to 2013, 40 (21 men; age, 33±12 years) underwent subsequent open thoracic aortic or endovascular secondary procedures between 1998 and 2013. Initial aortic root procedures were performed for aneurysmal disease (n=16, 40%) or acute or subacute Stanford type A dissection (n=24, 60%). Secondary interventions were performed on the aortic arch or descending thoracic or thoracoabdominal aorta (n=18, 45%; n=8 stent grafts) and aortic valve, root, or ascending aorta (n=22, 60%) 9.3±6.7 years (range, 0.06 to 24 years) after initial root surgery. Survival at 5 and 10 years after secondary aortic surgery was 80% (range, 65% to 90%) and 66% (range, 35% to 85%), respectively (n=13 and n=5 remaining at risk at 5 and 10 years). No difference between stent graft and conventional procedures was detected (p=0.756). Actuarial freedom from stroke was 89% at 5 and 10 years (range, 69% to 96%; stent graft versus no stent graft log-rank p=0.47). Four patients had tertiary aortic procedures. The presence of a chronic dissection or root or valve disease was not associated with adverse outcomes after secondary procedures. The only strong predictor of mortality after reoperations was acute dissection at the time of the initial treatment.

CONCLUSIONS

Aortic reoperations in patients with Marfan syndrome can be performed with acceptable midterm outcomes. Performed as a bailout procedure, stent grafting was not associated with increased mortality. The only strong predictor of mortality after reoperations is acute dissection at the time of the initial treatment.