Results of staged repair of aortic disease in patients with Marfan syndrome

Objective: We present our open surgical strategies for staged replacement of the thoracic and thoracoabdominal aorta in patients with Marfan syndrome. Methods: Between October 1999 and December 2017, 82 patients with Marfan syndrome underwent 118 aortic repairs. We divided the aorta into 4 segments for categorization: (1) the aortic root, (2) aortic arch, (3) descending thoracic, and (4) abdominal aorta. Procedures were categorized according to the types of surgery. Staged repair was defined as a subsequent operation on a different segment of the aorta after initial repair (n = 111, 94.1%), and reoperation was defined as an operation on the same segment (n = 7, 5.9%). Results: The mean age at initial operation was 41.7 +/- 14.9 years. Staged repairs included aortic root replacement (n = 42, 36%), total arch replacement (n = 11, 9.3%), combined aortic root and total arch replacement (n = 13, 11%), descending aorta replacement (n = 4, 3.4%), thoracoabdominal aortic repair (n = 36, 31%), and extensive arch-descending or thoracoabdominal repair (n = 5, 4.2%). Four patients received 3 staged repairs. Operative mortality was 0.8% (1/118). Stroke occurred in 1.7% (2/118), and spinal cord injury occurred in 1.7% (2/117). Overall survival was 95.8 +/- 2.4% at 10-years. Twenty-four patients underwent replacement of the whole aorta after 2.5 +/- 3.8 years following initial repair. Conclusions: Our strategies for staged replacement of the thoracic and thoracoabdominal aorta in patients with Marfan syndrome resulted in excellent early- and long-term outcomes.