The terms mitral valve prolapse syndrome (MVPS), dysautonomia, and postural orthostatic tachycardia syndrome (POTS) all refer to a syndrome in which the patient has problems with palpitations, hypotension, syncope, dyspnea, panic/anxiety disorder, numbness, hyperﬂexible joints, pectus excavatum, and gastric emptying disorders. Initially patients (typically fivefold times more likely to be women) who presented with these symptoms were thought to be somatizing their anxiety disorder. It is now accepted that it is a syndrome that appears to involve the autonomic nervous system (64). This often will present first in early adolescence with gradual worsening of the symptoms. The patients are very slender in build and lose weight to a low body mass index over a number of months as the syndrome evolves. This weight loss is the probable cause of the secondary amenorrhea that prompts these women to seek gynecologic care. The symptoms are not clearly explained by the degree of mitral valve prolapse, so many feel MVPS is a marker for individuals at risk for this complex of symptoms. Increased sympathetic activity is the common pathway for most of the proposed mechanisms for POTS or MVPS. There appears to be a genetic component, with over 10% of patients reporting the diagnosis in family members with orthostatic intolerance (65,66).
A tilt table test is often key to the diagnosis. Treatment focuses on the symptoms and maintaining intravascular volume by encouraging oral intake of water and salts (67). Physical fitness with aerobic exercises to increase muscle mass and reduce dependent pooling of blood; avoidance of smoking, caffeine, and alcohol; and limiting simple carbohydrates will minimize symptoms over time. Additional medications, including adrenoreceptor agonists, acetylcholinesterase inhibitor, mineralocorticoid agonist, beta-blockers, and selective serotonin reuptake inhibitors, may be necessary. Because this is a multifaceted disease, it may be best to refer the patient during the acute phase to a physician who is experienced with this syndrome.
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