Marfan's syndrome medical therapy

Medical Therapy

Drug therapies for Marfan syndrome focus on decreasing the mechanical forces to which the aorta is exposed. Beta blockers are the standard of care for adults. These drugs decrease myocardial contractility and as an extension, decrease blood pressure. In 1994, a study was published comparing the beta blocker propanol to placebo. Patients receiving propanol experienced a significant four-fold reduction in aortic root dilation and significantly decreased mortality in the middle of the study (around 5 years). However, at the end of the study, there was no significant difference between the groups in mortality. Evidence for use of beta blockers in children is less clear.

Recent studies of Losartan in children have proven promising in preventing cardiovascular complication of the syndrome. A 2008 study of 18 children with severe aortic root dilation who failed treatment with other medical therapy were treated with losartan. The rate of aortic root dilation was dramatically reduced after the initiation of therapy (3.54+/- 2.87 mm per year versus 0.46 +/-0.62 mm per year, p<.001). These findings have not yet been confirmed in a randomized trial. Clinical trials are currently underway comparing standard beta blocker therapy (Atenolol, Nebivolol) to therapy with Angiotensin Receptor Blockers (Losartan) , .

Even with the advent of new therapies based on angiotensin receptor blockade, patients are still at risk of aortic dissection It is recommended that most patients do not engage in vigorous physical activity. Contact sports such as football, ice hockey are strongly discouraged in addition to surfing and snorkeling. Activities that increase intrathoracic pressure such as weight lifting are also strongly discouraged. Exercise of low to moderate intensity, four to six metabolic equivalents, is permissible.