Mitral Valve Prolapse, Flailed Mitral Valve Mitral Annular Calcification

Mitral Valve Prolapse, Flailed Mitral Valve Mitral Annular Calcification

Mitral Valve Prolapse  

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What is a syndrome? Mitral valve prolapse is called by many names. What makes it a syndrome? What population is affected predominately with this condition? What does the words myxomatous degeneration mean and how does it relate to mitral valve prolapse and mitral regurgitation? What portions of the mitral valve apparatus can be affected?

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3-5% of the population Young thin females within reproductive years Hereditary incidence and autosomal dominant “Since a diagnosis of MVP carries with it a need for antibacterial prophylaxis, as well as the emotional burden of ‘heart disease,’ it is important not to overdiagnosis it.” (M. Allen)

Classical Vs. Non-classical MVP 

Classical MVP  

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Associated with many conditions. Characterized by increased redundancy or thickening (myxomatous changes) of varying portions of the mitral valve leaflets Occurs quite commonly in inheritable disorders of connective tissue 

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Marfan syndrome

Osteogenesis imperfecta Ebstein’s anomaly Ischemic papillary muscle Ballet dancers and patients with anorexia nervosa

Classic MVP cont… 

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High incidence of patients with asthenic habitus Thoracic deformities (pectus excavatum) Scoleosis

Non-classic MVP 

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Primary condition unassociated with other diseases Mitral valve leaflet coaptation point on the ventricular side of the mitral annulus and no or minimal mitral regurgitation

Secondary MVP 

Associated with CAD due to displacement of a ischemic papillary muscle

Functional MVP 

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Disproportion of the mitral valve leaflets and chordae in relation to the internal left ventricular dimension. A reduction or alteration in left ventricular cavity size or shape may cause normal mitral valve leaflets to move past the mitral valve annulus during ventricular systole

Myxomatous Appearance 

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Loss or dissolution of normal dense collagen fibers, with replacement and invasion of a less sturdy type of connective tissue (spongiosa) Leaflets, chordae, annulus all may be affected by myxomatous proliferation Leaflets are thickened and redundant, chordae can be elongated. Both leaflets can be affected, but the posterior leaflet is more commonly involved.

What constitutes prolapse? 

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Abnormal superior systolic displacement of the mitral valve leaflets; one or both of the leaflets extend beyond the normal systolic coaptation point allowing MR to occur. The MR jet is not usually central but eccentric depending on the leaflet that is affected. E.g., if the anterior leaflet prolapses, the MR jet usually extends posteriorly.

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During systole, individual scallops or an entire leaflet may billow excessively into the left atrium. For severe MR to be present, both leaflets must be affected or one leaflet may be flail.

Signs and Symptoms  

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Large majority are asymptomatic Anxiety, perhaps precipitated by having been diagnosed with the presence of heart disease. Palpitations, chest discomfort, dyspnea, fatigability, syncope, pre-syncope. Rare cases of sudden death due to malignant ventricular dysrhythmias

Physical Examination 

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MVP is one of the few cardiac conditions in which the outward appearance may suggest the diagnosis Familial variety   

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Red haired, fair skinned Asthenic with long extremities Some degree of pectus excavatum and less commonly pectus carinatium Scoliosis Kyphosis Straight back (narrow A/P chest diameter) Small breasted in women

Heart Sounds 

Systolic click 

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Most important auscultatory finding at least 0.14 sec after S1. The mitral valve begins to prolapse when the reduction of left ventricular volume during systole reaches a critical point at which the valve leaflets no longer coapt; at that instant, the click occurs and the murmur commences.

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The click normally is found in mid to late systole after S1 Any maneuver that decreases left ventricular volume, such as a reduction of impedance to LV outflow, a reduction in venous return, or an augmentation of contractility, results in an earlier occurrence of the prolapse in systole. What maneuvers would enhance? What maneuvers would diminish? Why?

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The click and onset of the murmur move closer to S1. The S1 sound will appear to increase in intensity

Systolic Murmur 

MVP can be associated with our without a mid to late systolic, crescendo or crescendodecrescendo medium to highpitched murmur. This is due to what condition?

Chest X-ray 

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May show the thoracic abnormalities Cardiomegaly due to left atrial enlargement and left ventricular enlargement suggesting significant MR.

Echocardiography

Treatment  

Usually no treatment MVR/replacement may be indicated for the significant MR

Flailed Mitral Valve 

Define the term Flail.

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Flailed mitral valve is a ruptured chordae/papillary muscle that will result in the abnormal coaptation of the mitral valve

Etiology   

MVP Severe MR Papillary muscle dysfunction from MI or endocarditis

Signs and Symptoms 

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Acute sudden onset of CHF and acute pulmonary edema due to sudden increase in volume in the left atrium during systole. The LA does not have time to take defense against the sudden onset of blood volume an rise in pressure. The edges of the valve leaflet slips completely and points towards the left atria during systole

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Acute MR is poorly tolerated and frequently results in profound clinical deterioration. A large volume of regurgitation into a normal, non-compliant atrium results in high left atrial pressures. The left ventricle does not tolerate an acute volume load when compensatory mechanisms of dilation and hypertrophy do not have time to develop; left ventricular diastolic and left atrial pressures increase markedly

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Patients experience       

Dyspnea Orthopena Paroxysmal nochturnal dyspnea (PND) Chest discomfort Pulmonary edema Shock Surgery is need immediately 

Carpentier or Duran ring

Carpentier or Duran Ring

Mitral Annular Calcification  

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MAC Found in the valve rings or fibroskeleton Degenerative process >40 yrs old, esp. women Most common cardiac abnormality found at autopsy

MAC 

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Usually limited to the posterior medial portion of the annulus at the base of the posterior leaflet and there usually is no complication When the calcification is more extensive, it may involve the valve leaflets and cause limitation of motion leading to regurgitation, when it is severe it may be an important cause for MR or cause of a gradient during diastole

Etiology 

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Accelerated by systemic hypertension, AS, chronic renal failure with secondary hyperparathyroidism and diabeties Marfan and Hurler syndromes

Pathophysiology 

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Annulus normally measure 10 cm in circumference, it is soft and flexible during diastole and contraction of the ventricle cause the annulus to constrict. When there is MAC it may immobilize the basal portion of the mitral leaflets, preventing their normal excursion in diastole and coaptation in systole and aggravating the MR that results from loss of normal sphincteric action of the mitral valve

Signs and Symptoms 

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Arrhythmias (atrial fibrillation, PACs) Twice as likely to have thromboembolic events