Type A Aortic Dissection in Marfan Syndrome: Extent of Initial Surgery Determines Long-Term Outcome

Study Questions:

What is the primary surgery and what are the long-term results in Marfan’s Syndrome (MFS) patients who suffered aortic dissection?


Among 1,324 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. Survival and freedom from re-interventions were analyzed using the Kaplan-Meier method.


Aortic re-intervention with re-sternotomy rate was 24% (18/74) and descending aorta (thoracic + abdominal) intervention was 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%, and 79 ± 5% at 5, 10, and 20 years, and 83 ± 9%, 60 ± 13%, and 20 ± 16% in patients who underwent supracoronary ascending replacement. Secondary aortic arch surgery was necessary only in patients with initial hemi-arch replacement.


The authors concluded that emergency surgery for Type A dissection in MFS patients is associated with low in-hospital mortality.


This study reports that the initial surgical intervention on MFS patients who present with acute Stanford Type A dissection is a low-risk procedure when performed in a cardiac center with extensive aortic surgical experience. Since supracoronary ascending replacement is associated with a high root re-intervention rate, aortic root replacement or repair during the initial surgery is highly recommended. Total aortic arch replacement reduces the number of aortic arch re-interventions. It appears that an effective strategy to improve long-term outcome in MFS patients with acute Stanford Type A dissection should include the David reimplantation procedure whenever it does not raise the overall operative risk, and that arch replacement is not necessary during the initial operation unless there are primary tears in the arch itself.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Vascular Medicine, Aortic Surgery, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Quality Improvement

Keywords: Sternotomy, Follow-Up Studies, Reoperation, Hospital Mortality, Aortic Aneurysm, Thoracic, Marfan Syndrome, Europe, United States

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