Key result
Thoracoabdominal aortic aneurysm repair achieves 96% 30-day survival with low morbidity in Marfan syndrome.
Why the study?
Does thoracoabdominal aortic aneurysm repair using contemporary techniques result in low mortality and morbidity in patients with Marfan syndrome?
Cohort (n=50)
Does thoracoabdominal aortic aneurysm repair using contemporary techniques result in low mortality and morbidity in patients with Marfan syndrome?
Thoracoabdominal aortic aneurysm repair can be performed in Marfan syndrome patients with low 30-day mortality and morbidity using contemporary surgical techniques.
May support repair in selected Marfan patients; leaves open need for prospective validation and long-term data.
BACKGROUND: Patients with Marfan syndrome frequently develop aneurysms or dissections involving multiple segments of the aorta; graft replacement of multiple aortic segments prolongs survival. Indications for graft replacement of the thoracoabdominal aortic segment include impending rupture, ischemic complications from dissection, or an aortic diameter measuring 5.5 cm or more. We retrospectively reviewed our experience with 50 consecutive thoracoabdominal aortic aneurysm repairs in Marfan patients to evaluate the results of surgery using contemporary techniques. PATIENTS AND METHODS: From January 1986 through July 1996, 800 patients underwent graft replacement of thoracoabdominal aortic aneurysms. Fifty patients (6.3%) had Marfan syndrome. Aneurysms without dissection were present in nine (18%) of the Marfan patients. The remaining 41 patients (82%) had chronic dissection. Both chronic dissection and Crawford extent II aneurysms were more prevalent in patients with Marfan syndrome than in non-Marfan patients. Extensive thoracoabdominal aortic aneurysm repairs (extents I and II) were performed in 72% of Marfan patients; atriodistal bypass was used in 22 patients (44%). RESULTS: The 30-day survival rate was 96%. Renal failure requiring hemodialysis occurred in three patients (6%). Two patients (4%) had postoperative lower extremity neurological deficits. No patient receiving atriodistal bypass developed paraplegia or paraparesis, versus two patients (7.1%) among the 28 who did not. CONCLUSION: Patients with Marfan syndrome have a high prevalence of extent II aneurysms and associated dissection. Using contemporary surgical techniques, Marfan patients can undergo thoracoabdominal aortic aneurysm repair with low mortality and morbidity, despite the extensive nature of their disease.
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Anderson et al. (1997) conducted a cohort in Marfan syndrome with thoracoabdominal aortic aneurysms (n=50). Thoracoabdominal aortic aneurysm repair was evaluated on 30-day survival. Thoracoabdominal aortic aneurysm repair in patients with Marfan syndrome achieved a 96% 30-day survival rate with low morbidity.
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