Medical management of Marfan syndrome

As recently as 40 years ago, people with Marfan syndrome faced a virtually hopeless situation on account of chronic mitral and aortic regurgitation, heart failure, and acute and chronic aortic dissection. Life span was reduced by at least one third, with many patients succumbing in the second and third decades. Today, cardiovascular manifestations of Marfan syndrome remain among the central issues in diagnosis and management, but it is incumbent on the physicians who encounter these patients to stress the prophylactic monitoring and therapies that now can result in a nearly normal life expectancy. Regular monitoring of valvular function and aortic diameter, early initiation of long-term β-adrenergic blockade and elective repair of a moderately regurgitant mitral valve or of a moderately dilated aortic root while preserving the native aortic valve are standards of care. Additionally, insights into the pathogenesis of clinical problems hold considerable promise for more effective, and even curative, medical therapies. © 2008 American Heart Association, Inc.