MMV is a pathological weakening of
connective tissue. The term is most often used in the context of
mitral valve prolapse, which is known more technically as "primary form of myxomatous degeneration of the mitral valve."
The degeneration occurs in conjunction with an accumulation of dermatan sulfate, a
glycosaminoglycan, within the connective tissue matrix of the valve.
In many cases, the degeneration is limited to the mitral valve and follows a benign course. However,when associated with systemic diseases, like
Marfan syndrome, the degeneration is more extensive and involves other heart valves. The valves can become sufficiently distorted to cause insufficiency and regurgitation. Myxomatous degeneration is the most common cause of pure mitral valve insufficiency, which belong to complications of MMV, the complications
mitral regurgitation and congestive heart failure may, in turn, cause arrhythmias and atrial fibrillation that may progress and lead to sudden death. However, there is no evidence that a prolapsed valve itself contributes to such arrythmias. Generally, MVP is benign. However, MVP patients with a murmur, not just an isolated click, have an increased mortality rate of 15-20%. The major predictors of mortality are the severity of mitral regurgitation and the
ejection fraction.
Echocardiography is the most useful method of diagnosing a MMV.