Mitral Valve Prolapse

Dr Mohammed Haroon Rashid Resident, Department Cardiology

Mitral Valve
Consists of 6 major anatomical components
Annulus Leaflets Chordae tendinae Papillary muscles Posterior left atrial wall Left ventricular free wall

Structure of mitral valve

Features Annulus Leaflets

Description Saddle Shaped, 4-6cm2 Sail shaped AML & C shaped PML Anterolateral PM at 4 O Clock, Posteromedial PM at 7 O Clock position 120 in number

Papillary

Chordea Tendinae

Mitral Valve Prolapse: Introduction

A variable clinical syndrome that results from a diverse pathogenic mechanisms of one or more portions of mitral valve apparatus, valve leaflets, chordae tendinae, papillary muscle & valve annulus. Many names:
Systolic click murmur syndrome Barlow syndrome Billowing mitral cusp syndrome Myxomatous mitral valve syndrome Floppy valve syndrome Redundant cusp syndrome

Epidemiology
Prevalence of 2.4% of population Twice frequent in females than in males Severe MVP occurs more frequently in older males (>50yrs)

Etiology
Primary condition
Familial Autosomal trait Non familial

Secondary conditions
Heritable disorders of connective tissue

Classification
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

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

Clinical Diagnosis
Symptoms

Atypical chest pain Palpitations Dyspnea Fatigue Syncope

Asthenic, low body weight Normal Blood pressure Orthostatic hypotension Straight back syndrome

Signs

Auscultation
Mid or late systolic click, heard over apex Pansystolic murmur present if associated with severe mitral regurgitation Dynamic auscultation
Change in loudness as well as the time of occurrence of both click & murmur are diagnostic

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

Other diagnostic tests

ECG
Negative or biphasic t waves & nonspecific ST changes in leads II, III, aVF & occasionally anterolateral leads Arrhythmias Atrial or Ventricular PC PSVT (most common) & ventricular tachyarrhythmia Bradyarrhytmias due to sinus node dysfunction Varying degrees of AV blocks Incidence with WPW & MVP has increased Increased association with Long QT syndrome Mechanism of arrhythmia not clear, but diastolic depolarization of muscle fibers in anterior mitral leaflet in response to stretch has been demonstrated experimentally

Other diagnostic tests

Stress scintigraphy
Differentiate MVP with IHD

Angiography

Disease course
General outcome is excellent, large group remain asymptomatic Serious complications occur in 1/100 patient years 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

Predictors of clinical outcomes in MVP

Sudden Cardiac Death

Relation of SCD & MVP is not clear Evidence suggests that MVP increases the risk of SCD slightly, especially in patients with severe MR or severe valvular deformity, & those with complex ventricular arrhythmias, QT prolongation is higher.

Other complications
CNS
Acute hemiplegia TIAs Amaurosis fugax Cerebellar infarcts Unexplained stroke of young!

Management
Transesophageal echo in first degree should be done (Circulation 2005) Echo should confirm the diagnosis Asymptomatic patients without arrhythmias/IE should be reassured & follow up examination every 3 to 5yrs to be done (follow up with color doppler) Patients with palpitations, arrhythmias should undergo EP study to characterize arrhythmias & RF if necessary for AV bypass tracts in prolonged SVT episodes

Management
Beta blockers for palpitations secondary to PVCs & relieve chest discomfort Aspirin in documented cases of neurological event or if atrial thrombus exists (Hayek; Mitral valve prolapse. Lancet. 2005 Feb 511;365(9458):507-18)

Patients with severe MR & MVP may require MV surgical repair. Antibiotic prophylaxis for GI & GU procedures (NICE Clinical Guideline (March 2008)

Thank You !