Mitral valve prolapse

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all about MVP

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This ghost is called MitralValveProlapse:

This ghost is called M itral V alve P rolapse Sayed Syleem , Msc Cardiologist Kafr Saad central hospital 2012

Many names: :

Many names: Systolic click murmur syndrome Barlow syndrome Billowing mitral cusp syndrome Myxomatous mitral valve syndrome Floppy valve syndrome Redundant cusp syndrome

Structure of mitral valve:

Structure of mitral valve Features Description Annulus Saddle Shaped, 4-6cm2 Leaflets Sail shaped AML & C shaped PML Papillary Anterolateral PM at 4 O Clock, Posteromedial PM at 7 O Clock position Chordea Tendinae 120 in number

MVP Pathophysiology:

MVP Pathophysiology

History :

History IN 1916, Sir James MacKenzie described the soldier's heart in spare, thin young men with great vasomotor instability , easy fatigability , breathlessness , and pain over the region of the heart. In 1920, Kerley first described the syndrome In 1928, Lincoln described the syndrome. In 1963, Barlow and colleague s made the first clinical diagnosis of the syndrome as it is known today.

Etiology:

Etiology Primary condition Familial – Autosomal trait Non familial Secondary conditions Heritable disorders of connective tissue The most common cause is idiopathic degeneration Several reports suggest magnesium deficiency underlies the disease in some patients

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Autosomal dominant X-linked inheritance. Three different loci on chromosomes 11, 13, 16 are linked to MVP, but No specific gene has been described. Another locus on chromosome X has also been found to cosegregate with a rare form of MVP called X-linked myxomatous valvular dystrophy

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Sex Females : males = 2/1. Age More frequent in 14 and 30 years , the defect is believed to be present at birth. Severe MVP occurs more frequently in older males >50ys

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Prevalence rates are: 1-2% in children 5-15% in adolescents and young adults . A report from California places the prevalence at only 0.6% In a series of 278 surgically removed mitral valves with pure regurgitation, MVP was present in 43%

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About 25% of people with MVP also suffer from lax joints , and a high arched palate (these patients may also have a degree of Marfan's syndrome), and other abnormalities of their skeleton such as scoliosis , a funnel chest and a straight back . It is more common in people who suffer from diseases such as Graves' disease , Marfan's syndrome and rheumatic heart disease , Duchenne muscular dystrophy , myotonic dystrophy , sickle cell disease . Isolated MVP has been independently associated with low body mass index ; however, the reason for this association remains unexplained

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A connective tissue abnormality , possibly related to collagen and elastic fibers metabolism , may underlie the idiopathic disorder. Urokinase-plasminogen activator , which is suggested in the pathogenesis of elastin and collagen degradation in arterial aneurysm , has also been implicated in MVP. Electron microscopy of the affected valve leaflets shows a haphazard arrangement, disruption, and fragmentation of collagen fibrils . Myxomatous proliferation of the mitral valve, in which the middle spongiosa layer is predominantly involved,

Pathology :

Pathology Myxomatous proliferation of mitral valve leaflets & quantity of acid mucopolysaccharide is increased . Regions of endothelial disruption are common & possible site for thrombus formation or endocarditis . Degeneration of collagen & myxomatous changes within the central core of chordae tendinae causes decrease of tensile strength & thus rupture

METABOLIC ABNORMALITIES IN MVPS:

METABOLIC ABNORMALITIES IN MVPS Autonomic nervous system dysfunction, Decreased intravascular blood volume, Renin-aldosterone regulation abnormality

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Classification of MVP Mitral Valve Prolapse syndrome Younger age (20-50) Predominantly females Click or click murmur present Benign long term course Myxomatous Mitral Valve Disease Older age- 40-70yr Predominantly males Thickened & redundant valve leaflets Progressive disease, requires surgery Secondary Mitral Valve Prolapse Marfan syndrome Hypertrophic cardiomyopathy Ehlers- Danlos syndrome Other connective tissue disorders

Symptomes:

Symptomes Majority are asymptomatic for entire life Palpitations (in 7.4% of patients) Dyspnoea ( unrelated to exertion) Fatigue Chest pain (atypical). Often substernal , prolonged, poorly related to exertion, and rarely resembles typical angina (in 10% of patients ) Syncope (in 0.9% of patients) “Symptoms of sympathetic excess ( eg , palpitations, dizziness, near syncope, migraines, anxiety)”

FACTORS THAT CAN INCREASE THE INTENSITY OR FREQUENCY OF MVPS SYMPTOMS: :

FACTORS THAT CAN INCREASE THE INTENSITY OR FREQUENCY OF MVPS SYMPTOMS: Emotional stress Lack of sleep Unaccustomed physical activity Lying on the left or right side Being in a hot, dry environment Menses Menopause Flu, cold Stimulants Alcohol Smoking Caffeine Sweets Skipping meals Dehydration

Signs :

Signs Asthenic, low body weight Low BP Orthostatic hypotension Straight back syndrome

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Mid-systolic click Late systolic murmur Click-murmur moves with LV volume changes Functional MVP can occur with completely normal valve leaflets: this is found in conditions of abnormal papillary muscle function due to myocardial ischaemia , and in DCM . Patients with HOCM are also at risk.

Mitral Valve Prolapse: variable murmur :

Mitral Valve Prolapse : variable murmur Second heart sound may be diminished by late systolic murmur with crescendos into S2

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Common findings are as follows : (as Marfan s.) Hypomastia Thin children Height-to-weight ratio greater than normal Arm span greater than height (dolichostenomelia( Arachnodactyly Scoliosis Narrow anteroposterior chest diameter (straight back( Pectus excavatum or pectus carinatum Crowding of teeth Joint hypermobility

Auscultation:

Auscultation Most important finding: mid  late systolic click . Acute tensing of the mitral valve chordae Variable murmurs: High pitched late systolic crescendo-decrescendo murmur , Occasionally “whooping” or “honking” at the apex S1 C S2 MR MVP

Echocardiography :

Echocardiography Confirmatory Prolapse of mitral leaflet into left atrium Thickening of mitral valve (>5mm )

M-mode at Mitral Valve:

Distance Systole M-mode at Mitral Valve Diastole Time MV prolapse posterior leaflet

M-mode at Mitral Valve:

Distance Systole M-mode at Mitral Valve Diastole Time MV Prolapse – both leaflet

ECG :

ECG Negative or biphasic T waves & nonspecific ST changes in leads II, III, aVF & occasionally antero-lateral leads Arrhythmias Atrial or Ventricular PC PSVT (most common) & ventricular tachyarrhythmia Bradyarrhytmias due to SAN dysfunction Varying degrees of AV blocks Incidence with WPW & MVP has increased Increased association with Long QT syndrome Mechanism of arrhythmia not clear.

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Stress scintigraphy (Differentiates MVP with IHD) Angiography

Course:

Course General outcome is excellent , large group remain asymptomatic Serious complications occur in 1% patient 4% died during 8yrs Most of the risk factors were based on Severity of MR , Ejection fraction (<50%), Left atrial dimensions (>40mm), Age (>50yr) Risk of development of IE is greater in men >50yrs , So, males above 50, and the presence of regurgitation place patient at higher risk for complications

Complications:

Complications Arrhythmias (Usually PVC, PSVT>>VT) TIA (embolic – rare) IE (if associated with MR) Sudden death (rare) especially in patients with: = Severe MR = Severe valvular deformity , = Complex Vent. Arrhy ., = Prolonged QT = Young women with a history of : Recurrent syncope, Sustained SVT, Complex VT

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CNS ( Thromboembolic ) Acute hemiplegia Cerebellar infarcts Unexplained stroke of young Syncope Moderate to severe MR can lead to Sudden death, Atrial fibrillation, Rupture of the chordae , Heart failure or Infective endocarditis , (in 0.1-0.3 %) An extreme form of prolapse could include chordal rupture , in which the prolapsed mitral valve is flail .

Treatment :

Treatment Usually not needed in ED Beta blockers may be used for patients with palpitations, chest pain, or anxiety Warfarin (PT-INR = 2-3) in patients with AF or risk for embolization : Aspirin (160 mg/d in patients with MVP and (AF without MR), HTN, HF, and above 50. Avoidence of alcohol, tobacco, and caffeine to relieve symptoms

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Asymptomatic patients without arrhythmias/IE should be reassured & follow up examination every 3 to 5yrs to be done Patients with palpitations, arrhythmias should undergo EP study to characterize arrhythmias & RF if necessary for AV bypass tracts in prolonged SVT episodes

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Additional dental care recommended for patients at risk for IE includes the following: Regular toothbrushing after eating No cookies, sweets, or sweet drinks between meals Regular dental checks every 6 months Fluoride supplements in locations where the fluoride in drinking water is <0.3 ppm for children <2 years or <0.7 ppm for children <2 years Dental treatments (> 2) scheduled at an interval of 14 days or longer

Medication Summary :

Medication Summary Anti-CHF therapy Antibiotic prophylaxis during surgery, dental, and genitourinary procedures - Only necessary if associated MR is present, Antiarrhythmic therapy - May be indicated in patients with documented and/or symptomatic arrhythmia, Beta-blockers - May be beneficial for symptom prevention, reduction in ectopy , treatment of vasodepressor syncope, panic attacks, or antiarrhythmic therapy Antiplatelet therapy - Used in patients with thromboembolic episodes ACE inhibitors - Used in patients with significant MR Low-dose aspirin and/or anticoagulant therapy - Considered in patients with thromboembolic episodes

Surgical Care :

Surgical Care Patients with CHF and/or severe MR with MVP may require MV surgical repair or replacement .

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Further Inpatient Care Admission of patients with MVP is seldom required, except in the case of complications or for consideration of surgery. Further Outpatient Care Repeat evaluations every 3-5 ys to identify any progression. IE prophylaxis is indicated in patients with MVP+MR while undergoing at-risk procedures. Patients with accessory pathways should have detailed EPS and radiofrequency ablation. Coronary artery anomalies should be excluded in patients with chest pain before they participate in sports. Mild MVP on echocardiography, in the absence of clinical findings (15-20% of patients), does not indicate true MVP syndrome. Parents and patients need to be reassured.

Patient Education :

Patient Education Careful explanation of the clinical findings and the nature of MVP help to reassure the anxious patient. Normal activity can be allowed if MR is not severe Antibiotic prophylaxis during surgery and dental procedures is only necessary if associated with MR. Patients with orthostatic syncope secondary to dehydration should take extra salt and water during and following sport activities. Pregnancy requires IE prophylaxis during delivery .

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sayedsyleem@yahoo.com والله من وراء القصد

References :

References Taub CC, Stoler JM, Perez-Sanz T, et al. Mitral valve prolapse in Marfan syndrome: an old topic revisited. Echocardiography . Apr 2009;26(4):357-64. [Medline] . Lou XF, Yang DY, Liu ZY, Wang HL, Li TS. [Clinical analysis of 120 cases of infective endocarditis]. Zhonghua Nei Ke Za Zhi . Jan 2009;48(1):35-8. [Medline] . Grau JB, Pirelli L, Yu PJ, Galloway AC, Ostrer H. The genetics of mitral valve prolapse. Clin Genet . Oct 2007;72(4):288-95. [Medline] . Movahed MR, Hepner AD. Mitral valvar prolapse is significantly associated with low body mass index in addition to mitral and tricuspid regurgitation. Cardiol Young . Apr 2007;17(2):172-4. [Medline] . Attias D, Stheneur C, Roy C, et al. Comparison of clinical presentations and outcomes between patients with TGFBR2 and FBN1 mutations in Marfan syndrome and related disorders. Circulation . Dec 22 2009;120(25):2541-9. [Medline] . Hepner AD, Ahmadi-Kashani M, Movahed MR. The prevalence of mitral valve prolapse in patients undergoing echocardiography for clinical reason. Int J Cardiol . Dec 15 2007;123(1):55-7. [Medline] . Cheunsuchon P, Chuangsuwanich T, Samanthai N, Warnnissorn M, Leksrisakul P, Thongcharoen P. Surgical pathology and etiology of 278 surgically removed mitral valves with pure regurgitation in Thailand. Cardiovasc Pathol . Mar-Apr 2007;16(2):104-10. [Medline] . Deng YB, Takenaka K, Sakamoto T, et al. Follow-up in mitral valve prolapse by phonocardiography, M-mode and two-dimensional echocardiography and Doppler echocardiography. Am J Cardiol . Feb 1 1990;65(5):349-54. [Medline] . Atalay S, Ucar T, Ozcelik N, Ekici F, Tutar E. Echocardiographic evaluation of mitral valve in patients with pure rheumatic mitral regurgitation. Turk J Pediatr . Apr-Jun 2007;49(2):148-53. [Medline] . Gutierrez-Chico JL, Zamorano Gomez JL, Rodrigo-Lopez JL, et al. Accuracy of real-time 3-dimensional echocardiography in the assessment of mitral prolapse. Is transesophageal echocardiography still mandatory?. Am Heart J . Apr 2008;155(4):694-8. [Medline] . Poothirikovil Venugopalan, MBBS, MD, FRCP(Glasg), FRCPCH; Chief Editor: Stuart Berger, MD

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