Type A Aortic Dissection in Marfan Syndrome: Extent of Initial Surgery Determines Long-term Outcome
Background—Data on outcomes after Stanford Type A aortic dissection in Marfan's Syndrome (MFS) patients are limited. We investigated the primary surgery and long-term results in MFS patients who suffered aortic dissection.
Methods and Results—Among 1324 consecutive patients with aortic dissection Type A, 74 with MFS (58% males, median age 37 (29; 48) years) underwent surgical repair (85% acute dissections; 68% DeBakey I; 55% composite valved graft (CVG), 30% supracoronary ascending replacement, 15% valve sparing aortic root replacement (V-SARR); 12% total arch replacement; in-hospital mortality 3%) at two tertiary centers in the United States and Europe over the last 25 years. Aortic re-intervention with re-sternotomy rate was 24% (18/74) and descending aorta (thoracic+abdominal) intervention - 30% (22/74) at median follow-up of 8.4 (2.2; 12.7) years. Freedom from need for aortic root reoperation in patients who underwent primarily a CVG or V-SARR procedure was 95±3%, 88±5%, 79±5% at 5, 10 and 20 years, and 83±9%, 60±13%, 20±16% in patients who underwent supracoronary ascending replacement. Secondary aortic arch surgery was necessary only in patients with initial hemi-arch replacement.
Conclusions—Emergency surgery for Type A dissection in Marfan patients is associated with low in-hospital mortality. Failure to extend the primary surgery to aortic root and/or arch repair leads to a highly complex clinical course. Aortic root replacement or repair is highly recommended, since supracoronary ascending replacement is associated with a high need (>40%) for root re-intervention.
- Received August 22, 2013.
- Revision received November 26, 2013.
- Accepted January 8, 2014.