Mitral Valve Prolapse and Regurgitation Jason Infeld, MD, FACC Stern Cardiovascular Foundation

Stern Cardiovascular Foundation DISCLOSURE Jason Infeld MD, FACC Stern Cardiovascular Foundation I have the following personal financial relationships with commercial interests to disclose: NONE

Mitral Valve Prolapse (MVP) MVP is the most common cause of mitral regurgitation and of congenital valvular heart disease in adults Definition and diagnostic criteria have changed leading to significant controversy Many common perceptions about this disease have been recently been shown to be false Disease was widely overdiagnosed in the 70’s and 80’s as echocardiography became more widely available 4

What is MVP? Systolic bowing of one or both mitral valve leaflets across the plane of the mitral valve annulus into the LA Disease is often benign, but may be associated severe complications including mitral regurgitation, endocarditis, and arrythmias. 5

Classic Mitral-Valve Prolapse during Systole Figure 1. Classic Mitral-Valve Prolapse during Systole. The parasternal long-axis view shows the mitral leaflets prolapsing (>2 mm), as indicated by the dotted line, into the left atrium during systole. LA denotes left atrium, and LV left ventricle. Freed L et al. N Engl J Med 1999;341:1-7

Classic Mitral-Valve Prolapse with Leaflet Thickening (Arrows) during Diastole Figure 2. Classic Mitral-Valve Prolapse with Leaflet Thickening (Arrows) during Diastole. The parasternal long-axis view demonstrates mitral-leaflet thickening (>=5 mm) during diastole. LA denotes left atrium, and LV left ventricle. Freed L et al. N Engl J Med 1999;341:1-7

How common is MVP? Early prevalence estimates between 5 and 20% and up to 35% in some studies Disease was thought to be more common in young women Studies were faulty due to severe selection bias and a lack of clear echocardiographic criteria 8

History of MVP Described accurately in the 60’s by Barlow in a group of patients with midsystolic clicks and mitral regurgitation seen during cardiac catheterization. Diagnosis was rare. 1970 – first description of M-mode echocardiographic findings. Echo led to sudden dramatic increase in the diagnosis of this entity Early studies shows prevalence as high as 35% “Mitral valve fiasco”

History of MVP 1980’s widespread use of 2- dimensional echo Use of apical 4-chamber view continued to lead to significant overdiagnosis 1987 – study published demonstrating the normal shape of the mitral valve as a “saddle” and that the 4-chamber view should not be used to make the diagnosis

Prevalence Framingham study - prevalence approximately 1.1% Reviewed echos of 3591 men and women 5 to 1 ratio of self-reported diagnosis of MVP and echocardiographic MVP Prevalence equal between men and women MVP patients were thinner and had more MR Average amount of MR was trace to mild 11

Echocardiography Apical 4-chamber view Parasternal Long-axis View 12

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Leaflet displacement Leaflet thickening Greater than 2mm above the plane of the mitral annulus in the parasternal long-axis view Leaflet thickening Greater than 5mm in the midportion of the anterior mitral leaflet 15

Echocardiography Classical vs. nonclassic MVP >2mm displacement and >5mm thickness are considered to have classic MVP Patients with leaflet thickeness <5mm have nonclassic MVP Symmetric vs Asymmetric prolapse 16

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Diagnostic Pitfalls Non-specific echo findings M-mode Apical 4-chamber view Physical exam. Midsystolic clicks are common in normal individuals. Symptoms: non-specific with significant overlap with other disease processes 18

Natural History of MVP MVP is generally benign, but serious complications do occur Complications of MVP are infective endocarditis, cerebrovascular accidents, atrial fibrillation, the need for mitral valve surgery, and death Complication rates are between 1 and 4% annually Complication rates vary amongst studies due to referral bias of the most serious cases to tertiary centers and maybe lower than reported Complications can be predicted by understanding simple risk factors 19

Complications Primary risk factors(RFs) for complications Moderate to severe MR EF less than 50% 20

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Complications Secondary risk factors Slight MR Left atrial dimension > 40 mm Flail leaflet Atrial fibrillation (AF) Age >50 years. 22

2006 ACC/AHA Guidelines Repeat echocardiography at yearly intervals in patients with high-risk findings on the initial echocardiogram (eg, diffuse thickening of the mitral leaflets and redundancy), or moderate MR. Clinical evaluation and repeat echocardiography every 6 to 12 months in patients with severe MR Clinical evaluation and echocardiography at any time there is a change in signs of symptoms. 24

Treatment of MVP

Endocarditis Prophylaxis The 2007 American Heart Association (AHA) guideline for the prevention of infective endocarditis made major revisions to the 1997 AHA guideline. MVP with mitral regurgitation is no longer considered a high risk valve lesion and prophylaxis is no longer recommended. Although MVP is associated with an increased risk of endocarditis, there are no convincing data that antibiotic prophylaxis is effective in preventing episodes of endocarditis

Treatment and F/u of MR 27

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Chronic Mitral Regurgitation Most patients asymptomatic even with severe MR Progressive dilatation of the LA and LV. LA enlargement may result in atrial fibrillation Moderate to severe MR may eventually result in LV dysfunction and development of CHF Pulmonary hypertension may occur with associated right ventricular dysfunction. Typically prolonged asymptomatic interval Maybe an accelerated phase as a result of ruptured mitral valve chordae leading to progressive left atrial and LV dysfunction and atrial fibrillation

Goals of Treatment Prevent irreversible LV dysfunction, pulmonary HTN, or atrial fibrillation in an asymptomatic patient Relieve symptoms of dyspnea and fatigue in symptomatic patients Prevent sudden cardiac death

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Mitral Valve Repair vs Replacement

Mitral Valve Repair Ideal treatment for mitral regurgitation. Avoids need for anticoagulation and long-term risks of valve prosthesis Preserves mitral valve anatomy leading to better post-operative LV function and survival Repair is surgeon specific and success is highly correlated with volume

Mitral Valve Repair Clinician needs to be able to determine the likelihood of repair Isolated posterior leaflet prolapse more amenable to repair Presence of severe anterior leaflet prolapse, severe valve thickening and calcification make repair less likely TEE is recommended pre-operatively to define pathology and mechanism of MR

How is it done?

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The Robot 41

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Flail Mitral Leaflet Subset of patients who do clinically worse even in the absence of progressive LV dilatation or dysfunction. Higher-risk of sudden cardiac death Referral for early surgical treatment if valve amenable to repair.

Flail Mitral Leaflet

TEE Plays an important role in the evaluation of MR due to the proximity of the TEE probe to the LA TTE can underestimate MR due to shadowing from calcification and prosthetic valves Defines mechanism and severity of MR Ideal test to assess if repair is feasible

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Questions?