Long-term outcomes of surgical procedures for Marfan syndrome: aortic dissection versus aneurysm

Multiple of subsequent procedures may necessary in Marfan syndrome (MFS) patients after initial surgery. The aim of this study was to investigate the full spectrum of secondary distal vascular or valvular interventions encountered after initial surgery.

Retrospective analysis of 201 consecutive MFS patients between January 2000 and March 2019 who underwent 274 distal aortic reinterventions and 5 mitral valve replacements.

Of the enrolled 201 MFS patients (73 female, mean age 37.0±12.8 years), the surgical indication for 93 patients was aortic root aneurysm, and for another 108 patients was dissection. The mean follow-up interval was 8.4±5.5 years. Total arch replacement (TAR) was performed in 68.5% of MFS patients presenting with type A aortic dissection (TAAD) and in 2.2% of patients with aneurysm. Secondary TAR became necessary for 3.4% of patients who failed to receive TAR at initial surgery in aneurysm group during follow-up, while for 33.3% of patients in dissection group (P<0.001). Freedom from distal aortic reoperation in dissection group were 65.4%±5.2%, 49.6%±6.4%, and 38.3%±7.7% and in aneurysm group were 90.5%±3.5%, 84.2%±4.8%, and 84.2%±4.8% at 5, 10, and 15 years, respectively (P<0.001). Survival in dissection group were 94.4%±2.4%, 83.4%±5.7%, 68.4%±10.8% and in aneurysm group were 100%, 97.7%±2.3%, 97.7%±2.3% at 5, 10, and 15 years, respectively (P=0.001). Freedom from mitral valve reoperation in dissection group were 98.8%±1.2%, 98.8%±1.2%, 88.9%±9.4% at 5, 10, and 15 years, respectively. Freedom from mitral valve reoperation in aneurysm group were 97.2%±1.9%, 94.6%±3.2%, 94.6%±3.2% at 5, 10, and 15 years, respectively (P=0.775).

TAAD at initial surgery was an independent predictor of distal aortic reoperation. Limited repair was feasible for MFS patients presenting with aneurysm at initial surgery, extended repair might be better for TAAD for its higher risk of distal reintervention. Concomitant mitral valve procedures may depend on mitral regurgitation grades.