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----- Original Message ----- From: Martha Murdock & (gigi*) Lawrence

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Sent: Sunday, February 17, 2002 1:30 AM

Subject: Subject Reference: Fw: Mitral Valve Prolapse

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----- Original Message ----- From: MARTHA-NSIF

Sent: Sunday, February 17, 2002 2:29 AM

Subject: Mitral Valve Prolapse

Mitral Valve Prolapse Authored by C. Plewa, M.D., FAAEM, Research Director, ClinicalAssistant Professor, Department of Surgery, Medical College of Ohio, St. Mercy Medical CenterEdited by McNamara, M.D., Program Director, Professor of EmergencyMedicine, Emergency Medicine, Allegheny University Hospitals, MCP division INTRODUCTION Background: Mitral valve prolapse (MVP) can occur in a multitude of disordersand, in most instances, reflects a variant of normal rather than a singledisease process. Despite years of research, the symptomatology and significanceof MVP remain controversial. What was once termed the disease of the decade inthe 1980s is now considered an interesting finding of dubious importance bysome. Initial studies reporting associated symptoms of chest pain, dyspnea,anxiety and panic were likely flawed by recruitment bias. Recent studies implythat the incidence of MVP has previously been overestimated by inaccurateechocardiographic diagnostic criteria, and that associated symptoms other thanpalpitations are uncommon. Despite this, MVP patients are at risk forendocarditis, cerebrovascular accident, mitral valve surgery and sudden death.Pathophysiology: A myxomatous degeneration from collagen dissolution leads toexcess mucopolysaccharides in the middle spongiosa layer of the mitral valveleaflets, with resultant stretching of the leaflets and chordae tendinae.MVP occurs when the left ventricular (LV) size is small in comparison to anenlarged mitral annulus, leaflets or chordae tendinae and can be induced inhealthy women with typical body habitus following dehydration and reversed withrehydration. MVP resolves during pregnancy and following weight gain inanorexic patients.A constellation of abnormalities including increased sensitivity to adrenergicstimuli, increased catecholamines, abnormal beta receptors, increased atrialnatriuretic factor, renin-aldosterone dysregulation, decreased intravascularvolume and magnesium deficiency have been thought to lead to chest pain,dyspnea, fatigue, dizziness, near-syncope and anxiety in a subset of MVPpatients.Cardiac manifestations include supraventricular arrhythmias, palpitations,mitral regurgitation, bacterial endocarditis and sudden death. Chest pain maynot be more common in MVP than in the general population and may be attributedto myofascial syndromes, hyperventilation, coronary spasm, syndrome X,esophageal dysmotility or gastroesophageal.MVP can result in cerebrovascular ischemia, perhaps related to abnormalplatelet activity or protein C or S deficiencies.Frequency: In the U.S.: MVP is present in 3-4% of the general population by echocardiography and 7% atautopsy.Internationally: The worldwide incidence of MVP is likely similar as in the US.Mortality/Morbidity: In general, MVP is a benign disorder but may account forthe majority of isolated cases of mitral regurgitation (MR), 90% of cases ofruptured chordae tendinae, 40% of cerebrovascular accidents in young patientsand 10-15% of cases of endocarditis. Those with structural abnormalities suchas thickened, deformed or redundant mitral valve leaflets are more likely tosuffer complications, such as progressive MR, endocarditis and sudden death.MVP with murmur (not an isolated click) increases the general mortality rate by15-20%.Sex: The male to female ratio is about 1:3.Beyond the age of 45, men have twice the risk of MR and endocarditis.Age: Age of onset is 10-16 years.Although MVP is considered congenital, echocardiographic findings are typicallyabsent in newborns.CLINICAL History: The majority of patients are asymptomatic.Palpitations (40%): Exclude withdrawal syndromes (e.g. alcohol and sedatives),intoxications (e.g. cocaine, amphetamine and phencyclidine) or medicationexposures (e.g. caffeine, sympathomimetic and anticholinergic).Chest pain, dyspnea and anxiety, previously considered part of the MVPsyndrome, are now felt to be no more common than in the general population.Physical: Thin, aesthenic body habitusSkeletal abnormalities: Pectus excavatum, straight back and kyphoscoliosisResting bradycardia and orthostatic hypotensionCardiac auscultation:Apical single or multiple mid- to late-systolic clicks (from tightening of thechordae tendinae or redundant valve)Apical mid- to late-systolic murmur of crescendo, decrescendo or constantnature continuing to (or sometimes beyond) S2The click and murmur change with position changes: closer to S1 with diminishedLV volume, and closer to S2 with increased LV volume.Supine: the click is late (close to S2) and the murmur brief.Standing (and with Valsalva): the click is earlier (close to S1) and the murmurlonger. This may bring out a murmur not previously noted.Squatting: the click is later (closer to S2) and murmur shorter (the click andmurmur may even disappear).Isometric handgrip exercise increases the intensity (loudness) of the murmurwithout affecting position.The murmur should be distinguished from that of aortic stenosis (earlysystolic, at base); pulmonic flow murmur (short, early systolic, diminisheswith Valsalva); hypertrophic cardiomyopathy (diminished with squatting andlouder with standing and Valsalva) and mitral regurgitation (holosystolic, S3,enlarged and displaced PMI).Marfan's syndrome findings: Scoliosis, straight back, pectus excavatum,arachnodactyly and arm span greater than heightEhlers-Danlos syndrome findings: Joint hypermobility, abnormal striae, bruisingand distensibility of skinOsteogenesis imperfecta findings: Blue scleraeCauses: The majority of cases are primary, idiopathic in nature and expressed withautosomal dominant inheritance. Secondary causes of MVP include:Connective tissue disorders:Marfan's syndrome, Ehlers-Danlos syndrome (types I, II & IV), osteogenesisimperfecta, pseudoxanthoma elasticum, polycystic kidney disease, systemic lupuserythematosis, relapsing polychondritis and polyarteritis nodosaMuscle disorders: Duchenne's muscular dystrophy, fragile X syndrome,mucopolysaccharidoses and myotonic dystrophyCongenital heart disease: Atrial septal defect (ASD), Ebstein's anomalyAcquired heart disease: Papillary muscle dysfunction (ischemia, myocarditis),cardiac trauma, post mitral valve surgery and rheumatic endocarditisMiscellaneous: Wolff-Parkinson-White syndrome and Von Willebrand's disease======== An American Classic ===========================

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