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Surgical implication of aortic dissection on long-term outcome in Marfan patients
1Second Department of Surgery, Niigata University School of Medicine, Niigata City, Japan.
Abstract:
We herein review our 17-year surgical experience for the treatment of ascending aortic aneurysm in patients with Marfan syndrome to clarify the risks of increased mortality and reoperation. The subjects consisted of 15 patients who had all undergone surgery for the aortic root and ascending aorta at Niigata University Hospital between July 1978 and January 1995. Aortic valve replacement and ascending aortic wrapping were performed in 5 patients, Bentall or Cabrol operation in 6, and combined aortic arch reconstruction and Cabrol operation in 2, as the initial surgery. Patients who had an aortic dissection (Stanford type A) at initial surgery were assigned to group I (n = 7), while those with an aortic root aneurysm were assigned to group II (n = 8). In group I, 3 patients required a second operation for the remaining aortic arch aneurysm, and 1 died due to a late rupture of the distal aneurysm. In group II, no patient needed a reoperation; however, 1 died due to an intracranial hemorrhage and another due to composite valve graft failure and distal dissection. The results thus indicate that aortic dissection seems to affect long-term outcome, and therefore the combined repair of the aortic root and transverse arch is recommended in Marfan patients with aortic dissection involving the transverse aortic arch.
Insights
For Marfan syndrome patients undergoing aortic aneurysm surgery, aortic dissection significantly increases mortality and reoperation risks. Combined aortic root and transverse arch repair is recommended for dissections involving the transverse arch.
Area of Science:
- Cardiovascular Surgery
- Genetics
- Aortic Diseases
Background:
- Marfan syndrome is a genetic disorder that affects the connective tissue, predisposing individuals to aortic root and ascending aorta aneurysms.
- Ascending aortic aneurysms in Marfan syndrome patients carry significant risks of dissection, rupture, and mortality.
- Long-term outcomes and reoperation rates following surgical repair require further clarification.
Purpose of the Study:
- To review a 17-year surgical experience treating ascending aortic aneurysms in Marfan syndrome patients.
- To clarify the risks of mortality and reoperation associated with these procedures.
- To identify factors influencing long-term outcomes.
Main Methods:
- Retrospective review of 15 Marfan syndrome patients who underwent surgery for aortic root and ascending aorta between 1978 and 1995.
- Classification of patients into two groups: Group I (aortic dissection at initial surgery) and Group II (aortic root aneurysm).
- Analysis of initial surgical procedures (aortic valve replacement, ascending aortic wrapping, Bentall/Cabrol operation, aortic arch reconstruction).
Main Results:
- Group I (aortic dissection) had 3 reoperations for aortic arch aneurysm and 1 late death from distal aneurysm rupture.
- Group II (aortic root aneurysm) had no reoperations, but experienced 2 deaths (intracranial hemorrhage, graft failure with distal dissection).
- Aortic dissection was associated with poorer long-term outcomes and increased reoperation rates.
Conclusions:
- Aortic dissection is a critical factor impacting long-term outcomes in Marfan syndrome patients with ascending aortic aneurysms.
- Combined repair of the aortic root and transverse aortic arch is recommended for patients with aortic dissection involving the transverse arch.
- Early and comprehensive surgical intervention may improve survival in this high-risk population.