Extensive aortic reconstruction for aortic aneurysms in Marfan syndrome

Background. Marfan syndrome patients frequently develop aneurysms or dissec tions involving multiple segments of the aorta, and occasionally require st aged replacement of the entire aorta. This study reviews the surgical outco me of patients with Marfan syndrome who underwent extensive aortic reconstr uction. Extensive reconstruction is defined as reconstruction of more than two segments of the ascending, arch, descending thoracic, or abdominal aort a. Methods. From March 1973 to December 1997, 101 patients with Marfan syndrom e underwent aortic operation. Twenty-six patients (25.7%) had extensive aor tic reconstruction. All 26 patients suffered from aortic dissection: 13 pat ients had Stanford type A and 13 had type B dissection. Twenty-three patien ts (88.4%) had annuloaortic ectasia and aortic regurgitation. Surgical proc edures included composite valve graft replacement (n = 23, 88.4%), aortic a rch reconstruction (n = 15, 57.7%), graft replacement of the descending tho racic aorta (n = 6, 23.1%), and graft replacement of the thoracoabdominal a orta (n = 16, 61.5%). Five patients (19.2%) had total thoracoabdominal aort ic replacement, and three patients (11.5%) had replacement of the entire ao rta. Twenty-one patients (80.8%) required multiple operations. Results. Follow-up was complete in all patients. The 30-day survival rate w as 88.5%. None of the survivors had paraplegia or paraparesis. The overall long-term survival rate was 88.5 +/- 6% at 1 year, and 81.7 +/- 9% at 9 yea rs. Conclusions. Aortic surgery prolongs survival in patients with Marfan syndr ome, and currently there is a relatively low associated morbidity and morta lity even for aggressive surgical treatment. (C) 1999 by The Society of Tho racic Surgeons.