Extensive aortic reconstruction for aortic aneurysms in Marfan syndrome

Marfan syndrome patients frequently develop aneurysms or dissections involving multiple segments of the aorta, and occasionally require staged replacement of the entire aorta. This study reviews the surgical outcome of patients with Marfan syndrome who underwent extensive aortic reconstruction. Extensive reconstruction is defined as reconstruction of more than two segments of the ascending, arch, descending thoracic, or abdominal aorta.

From March 1973 to December 1997, 101 patients with Marfan syndrome underwent aortic operation. Twenty-six patients (25.7%) had extensive aortic reconstruction. All 26 patients suffered from aortic dissection: 13 patients had Stanford type A and 13 had type B dissection. Twenty-three patients (88.4%) had annuloaortic ectasia and aortic regurgitation. Surgical procedures included composite valve graft replacement (n = 23, 88.4%), aortic arch reconstruction (n = 15, 57.7%), graft replacement of the descending thoracic aorta (n = 6, 23.1%), and graft replacement of the thoracoabdominal aorta (n = 16, 61.5%). Five patients (19.2%) had total thoracoabdominal aortic replacement, and three patients (11.5%) had replacement of the entire aorta. Twenty-one patients (80.8%) required multiple operations.

Follow-up was complete in all patients. The 30-day survival rate was 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 years.

Aortic surgery prolongs survival in patients with Marfan syndrome, and currently there is a relatively low associated morbidity and mortality even for aggressive surgical treatment.