CARDIOVASCULAR MANIFESTATIONS
Mitral valve prolapse (MVP) and mitral valve regurgitation are common CV complications in patients with MFS. Estimated at 40-68% in adults compared to about 30-38% in children with MFS and 1-2% in the general population 34. Aortic regurgitation occurs following the dilatation of the aortic root and also abnormal valve cusp structure leading to aortic valve prolapse. Moderate to severe AR has been documented in a study to be an independent predictor of CV events including dilatation, dissection, and the need to have surgical intervention 35. Eventually, the patient with MFS dies from aortic complications such as dissection or heart failure following volume overload from long-standing aortic and mitral regurgitation.
In the management of MFS, it has been documented that the impact of prophylactic pharmacotherapy with B Blockers on the risk of complication is not insignificant 36. Randomized controlled trials (RCTs) that compared the impact of ARBs and BB found no statistically significant difference in the rates of clinical outcomes such as aortic regurgitation or the need for surgery. Some studies have found an added benefit of concomitant use of BB and ARB therapies 37, 38