Valve-sparing versus valve-replacing aortic root operations in patients with Marfan syndrome

Davut Cekmecelioglu, Joseph S. Coselli

Abstract

Aortic root dissection and rupture is the leading cause of death in patients with Marfan syndrome, a heritable connective tissue disorder that affects 1 in 10,000 individuals. Approximately 80% of those with Marfan syndrome experience annuloaortic ectasia, a distinctive form of aortic root dilation that often leads to aortic or mitral valve regurgitation, congestive heart failure, or aortic dissection or rupture. The cornerstone of care for preventing and managing such complications is replacement of the aortic root. Surgical procedures have evolved since Bentall and De Bono’s first aortic root replacement (ARR) in 1968, largely in response to late complications. Today, ARR is low-risk, systematic, and easily reproducible and has all but normalized life expectancy in Marfan syndrome. Nevertheless, long-term effects and durability remain important concerns. ARR surgical approaches include valve-replacement surgery (VRARR) with either a mechanical or tissue prosthetic aortic valve, and valve-sparing surgery (VSARR). VRARR with a mechanical valve is considered a permanent method of repair but requires lifelong anticoagulation, and the durability of bioprosthetic (tissue) valves is unclear. VSARR carries risk for adverse outcomes as well, including a higher likelihood of aortic valve regurgitation and the need for late reoperation; the method also typically involves repair of the valve leaflet, which requires advanced surgical skills and experience. The choice between VSARR and VRARR is often made intraoperatively; whether VSARR is feasible and durable is ultimately determined on the basis of the native aortic valve’s performance. For patients with leaflet fenestration, excessive tissue, annular dilation, or free margin elongation, VSARR is rarely applicable. Nonetheless, VSARR has been shown to be superior in terms of hemorrhage, thromboembolism, endocarditis, and re- exploration. Today, valve-sparing procedures are more commonly used than valve-replacement approaches in patients with Marfan syndrome.