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