There is considerable controversy surrounding the appropriate survellience, timing and method to repair an aortic root aneurysm in the Marfan syndrome. The purpose of this case report is to describe our experience with the valve-sparing aortic root replacement. This approach allows replacement of the degenerative Marfan aortic root while sparing the native valve apparatus.
Twenty-five year old male with Marfan syndrome presented with severe chest pain. Computed tomographic scan demonstrated a 5.5 cm aortic root aneurysm. Repair was accomplished by resuspending the native aortic commissures inside a 32 mm hemashield tube graft, realigning the valve leaflets. The coronary arteries were reimpplanted into the left and right neo-coronary ostia. Cross-clamp time was 149 minutes and no blood transfusions were required. The post-opperative course was uneventful. The patient was extubated 3 hours after surgery and discharged to home on the 4th post-operative day. Echocardiography demonstrated normal valve leaflet function and no evidence of aortic insufficiency.DISCUSSION: Annuloaortic ectasia is common in patients with connective tissue disorders. Pathologic weakening of the aortic wall can be detected in patients with marfan syndrome, Ehlers-Danlos, osteogenesis imperfecta and pseudoxanthoma elasticum.Evolution in the repair of the degenerative aortic root has been significant. Historically, the majority of repairs required complete excision of the valvular apparatus, aortic root, and ascending aorta with placement of a prosthetic “composite” valved-aortic graft.CONCLUSIONS: While several options exist for repair of the Marfan aortic root, remodeling of the native valvular apparatus using this resuspension technique is an excellent alternative. Although the long-term results are unknown, this repair reestablishes native aortic valvular competency thereby avoiding the need for long-standing anticoagulation.
D.G. Affleck, None.