Primary aortic root replacement or repair in Marfan syndrome patients with type A dissection resulted in 79% freedom from root reoperation at 20 years, compared to 20% with supracoronary ascending replacement.
Cohort (n=74)
Yes
Does the extent of initial surgical repair reduce the need for long-term aortic reinterventions in patients with Marfan syndrome and type A aortic dissection?
In Marfan syndrome patients with type A aortic dissection, initial aortic root replacement or repair is highly recommended to prevent the high rate of future root reinterventions associated with supracoronary ascending replacement.
Absolute Event Rate: 79% vs 20%
BACKGROUND: Data on outcomes after Stanford type A aortic dissection in patients with Marfan syndrome are limited. We investigated the primary surgery and long-term results in patients with Marfan syndrome who suffered aortic dissection. METHODS AND RESULTS: Among 1324 consecutive patients with aortic dissection type A, 74 with Marfan syndrome (58% men; median age, 37 years first and third quartiles, 29 and 48 years) underwent surgical repair (85% acute dissections; 68% DeBakey I; 55% composite valved graft, 30% supracoronary ascending replacement, 15% valve-sparing aortic root replacement; 12% total arch replacement; 3% in-hospital mortality) at 2 tertiary centers in the United States and Europe over the past 25 years. The rate of aortic reintervention with resternotomy was 24% (18 of 74) and of descending aorta (thoracic+abdominal) intervention was 30% (22 of 74) at a median follow-up of 8.4 years (first and third quartiles, 2.2 and 12.7 years). Freedom from need for aortic root reoperation in patients who underwent primarily a composite valved graft or valve-sparing aortic root replacement procedure was 95±3%, 88±5%, and 79±5% and in patients who underwent supracoronary ascending replacement was 83±9%, 60±13%, 20±16% at 5, 10, and 20 years. Secondary aortic arch surgery was necessary only in patients with initial hemi-arch replacement. CONCLUSIONS: Emergency surgery for type A dissection in patients with Marfan syndrome is associated with low in-hospital mortality. Failure to extend the primary surgery to aortic root or arch repair leads to a highly complex clinical course. Aortic root replacement or repair is highly recommended because supracoronary ascending replacement is associated with a high need (>40%) for root reintervention.
Rylski et al. (Wed,) conducted a cohort in Stanford type A aortic dissection in Marfan syndrome (n=74). Aortic root replacement or repair (composite valved graft or valve-sparing aortic root replacement) vs. Supracoronary ascending replacement was evaluated on Freedom from aortic root reoperation at 20 years. Primary aortic root replacement or repair in Marfan syndrome patients with type A dissection resulted in 79% freedom from root reoperation at 20 years, compared to 20% with supracoronary ascending replacement.