Rapid progress has been made in the treatment of Marfan syndrome. Today, the treatment is relatively established and the results have also improved. Even if surgery is performed, however, vascular lesions may recur late after operation and late prognosis is poor considering the age of patients. Issues such as whether a reoperation should be conducted or how the late results might be improved are subjects of debate. Based on an analysis of recent late data, we have performed operations according to the new treatment policy, and here report the results. A total of 203 consecutive operations were conducted in 141 patients with Marfan syndrome who underwent surgery for aortic aneurysm at our department between February 1973 and August 2001. The mean age of patients was 39 (11 years with a male/female ratio of 95:46. At the first operation, 72 patients were diagnosed with annuloaortic ectasia (AAE), 17 patients with AAE + chronic dissection (DeBakey I), 14 patients with AAE + chronic dissection (DeBakey II), 6 patients with AAE + acute dissection (Stanford A), 11 patients with AAE + dissection (DeBakey III), 9 patients with dissection (DeBakey III) only, 3 patients with AAE + abdominal aortic aneurysm only, and 2 patients with abdominal aortic aneurysm only. The cause of reoperation were a new lesion in 17 patients, dissection in 13 patients and a true aneurysm in 4 patients. In 36 patients, an increase in the remaining lesion occurred or a scheduled stage 2 operation was performed. Reoperation was performed following the Bentall operation in 7 patients, dehiscence of the anastomotic region of the coronary artery in 5 patients, aneurysm of the anastomotic region of the coronary artery in 1 patients, and infection of the artificial valve with aneurysm of the anastomotic region of the coronary artery in 1 patient. Hospital deaths were reported in 8 (6%) patients who underwent composite valve graft replacement (including simultaneous arch replacement) for AAE. Hospital deaths also occurred in 8 (13%) patients who underwent a replacement of the aortic arch, descending thoracic or thoracoabdominal aorta, with no cases of paraplegia reported. In all cases, the replacement was extensive. The observed 10-year survival rate was 70% with a freedom from reoperation of 64%. Although dissection did not reduce the observed survival rate, the 10-year freedom from reoperation was significantly decreased at 49% (p = 0.0007).
Conclusions: (1) Surgery is indicated for AAE when the maximum diameter of the aneurysm prior to onset of dissection is 5 cm. In the case of aortic arch without dissection, the Bentall operation with simultaneous arch replacement should be aggressively performed in order to minimize the future risk of vascular events and to eliminate the need for extensive replacement in a reoperation, a procedure which is associated with a high level of risk. (2) Extensive replacement which is associated with poor results should be avoided where possible and, instead, scheduled staged surgery should be aggressively performed in the early stage when the maximum diameter of the aneurysm in the descending aorta is 5 cm or less. (3) By paying adequate attention to patient education, outpatient follow-up using detailed diagnostic imaging, drug therapy, periodic late surveillance, expedition of scheduled surgery, and to the basic approach as well as endeavoring to improve surgical results by the use of new methods of treatment, it is anticipated that further improvement will be observed in late results.