Mitral Valve Prolapse Overview

Mitral valve prolapse (MVP) is also called click-murmur syndrome, floppy mitral valve syndrome, and Barlow syndrome after the doctor who first described MVP. The mitral valve is one of 4 valves in the heart. It opens and closes to control blood flow between the heart's left atrium and the left ventricle. The mitral valve has 2 flaps, or "leaflets." In mitral valve prolapse, one or both leaflets of the valve are too large, or the chordae tendinea (the strings attached to the underside of the leaflets, connected to the ventricular wall) are too long (redundant), resulting in uneven closure of the valve during each heartbeat. Because of uneven closure of the leaflets, the valve bulges back, or "prolapses," into the left atrium like a parachute. When this happens, a very small amount of blood may leak through, moving backward from the ventricle to the atrium. The valve still works well, and the heart pumps normally. Prolapse does not cause damage to the heart over time. Only 2% of people have other structural heart problems along with mitral valve prolapse. Previously called the most common heart valve abnormality, mitral valve prolapse was thought to have affected 5-20% of the general population, mainly women. Now with newer, wiser echocardiographic criteria, it is thought to affect only 2-3% of the general population, and it is most often diagnosed in people aged 20-40 years.

Mitral Valve Prolapse Causes

For most people, the cause for mitral valve prolapse is unknown.
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Some people may inherit the condition, especially those associated with connective tissue disorders like Marfan's syndrome. Marfan's syndrome is an inherited disorder of connective tissue that causes abnormally long limbs, loose joints, and bulges (aneurysms) in the aorta, the main artery from the heart. Up to 40% of people have dysautonomia, an imbalance of the autonomic nervous system. This is the part of the nervous system that controls involuntary body functions such as breathing and the beating of the heart. This may lead to a large number of symptoms that seem serious to the person with the symptoms but are usually not serious (that is, they are not heart conditions).

Mitral Valve Prolapse Symptoms

About 60% of people with mitral valve prolapse have no symptoms. A stressful situation, such as childbirth, job change, or viral illness, can bring on symptoms that may include the following:
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Irregular heartbeat or palpitations, especially while lying on the left side

Chest pain - Sharp, dull, or pressing, lasting from a few seconds to several hours, usually not related to myocardial ischemia (that is, not a threatened heart attack) Fatigue and weakness, even after little exertion Dizziness Light-headedness when rising from a chair or a bed Shortness of breath Low energy level, often misdiagnosed as chronic fatigue syndrome

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The numerous symptoms of dysautonomia include the following:

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Panic attacks Anxiety Fatigue Migraine headaches Irritable bowel Depression (in 70% of cases)

People will also have symptoms related to associated illnesses, such as Marfan's syndrome or hyperthyroidism (increased thyroid hormone).

mitral regurgitation
Background
Mitral regurgitation, in the acute and chronic decompensated states, is commonly encountered in the emergency department. An understanding of the underlying etiologies and pathophysiology of the condition is critical to direct appropriate treatment.

Pathophysiology
Mitral regurgitation can be divided into the following 3 stages: acute, chronic compensated, and chronic decompensated. In the acute stage, which usually occurs with a spontaneous chordae tendineae or papillary muscle rupture secondary to myocardial infarction, a sudden volume overload occurs on an unprepared left ventricle and left atrium. The volume overload on the left ventricle increases left ventricular stroke work. Increased left ventricular filling pressures, combined with the transfer of blood from the left ventricle to the left atrium during systole, results in elevated left atrial pressures. This increased pressure is transmitted to the lungs resulting in acute pulmonary edema and dyspnea. If the patient tolerates the acute phase, the chronic compensated phase begins. The chronic compensated phase results in eccentric left ventricular hypertrophy. The combination of increased preload and hypertrophy produces increased end-diastolic volumes, which, over time, result in left ventricular muscle dysfunction. This muscle dysfunction impairs the emptying of the ventricle during systole. Therefore, regurgitant volume and left atrial pressures increase, leading to pulmonary congestion.

Severe mitral regurgitation as depicted with color Doppler echocardiography. [ CLOSE WINDOW ] Severe mitral regurgitation as depicted with color Doppler echocardiography.

Frequency

United States

Previously, chronic rheumatic heart disease was the most common cause of acquired mitral valve disease in the Western world. More recently, however, mitral valve prolapse (MVP) has become the most common cause, being responsible for 45% of cases of mitral regurgitation. MVP has been estimated to be present in 4% of the population; however, significant regurgitation in this population only occurs in those with abnormalities of the valve.
International

In areas other than the Western world, rheumatic heart disease remains the leading cause of mitral regurgitation.

Mortality/Morbidity
The prognosis of patients with mitral regurgitation depends on the underlying etiologies and the state of the left ventricular function.
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Acute pulmonary edema and cardiogenic shock often complicate the course of acute regurgitation. The operative mortality in these cases approaches 80%. A patient with ruptured chordae tendineae and minimal symptoms has a much better prognosis. With chronic regurgitation, volume overload is tolerated very well for years before symptoms of failure develop. Left atrial enlargement predisposes patients to the onset of atrial fibrillation with the subsequent complication of embolization. In addition, these patients are susceptible to endocarditis. A study of the survival of patients with chronic regurgitation was performed using randomly selected patients. The study revealed that 80% of the patients were alive 5 years later, and 60% were alive after 10 years. Most patients with mitral valve prolapse are asymptomatic. Prolapse in those older than 60 years is frequently associated with chest pain, arrhythmias, and heart failure. The prognosis of these patients is good; however, sudden death, endocarditis, and progressive regurgitation occur rarely. When ischemic heart disease is the mechanism for regurgitation, the extent of anatomic disease and left ventricular performance are prognostic determinants. Complicating events include sudden death and myocardial infarction.

Sex
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In those younger than 20 years, males are affected more often than females. In those older than 20 years, no sexual predilection exists. Males older than 50 years are affected more severely.

Age
Of those cases caused by prior rheumatic disease, the mean age is 36, plus or minus 6 years.

Clinical

History
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Mitral regurgitation can be tolerated for many years. The initial symptoms of dyspnea and fatigue can rapidly progress to orthopnea and paroxysmal nocturnal dyspnea. Patients with anginal-type pain may have underlying ischemia. Atypical chest pain can be associated with MVP syndrome. In patients with mitral valve prolapse (MVP), palpitations and atypical chest pain are the most frequent complaints. Two thirds of these patients are female, often with an underlying panic disorder. With underlying coronary artery disease (CAD), regurgitation usually is associated with symptoms of angina pectoris. Regurgitation also can develop acutely with myocardial infarction, secondary to papillary muscle rupture. Coronary artery disease often is accompanied by dyspnea, fatigue, orthopnea, and fluid retention. Chest pain is usually minimal in these patients. When mitral regurgitation is due to left ventricular dilatation and altered valve function, patients often have chronic left-sided heart failure. In acute mitral regurgitation from sudden disruption of the mitral valve, the symptoms are due to acute pulmonary edema.

Physical
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The classic murmur of mitral regurgitation is a high-pitched holosystolic murmur beginning with the first heart sound and extending to the second heart sound. o The intensity usually is constant throughout systolic ejection, often radiating to the axilla. o The harshness of the murmur does not correlate with the magnitude of the valvular defect. o Patients with severe disease often have a third heart sound, a consequence of the increased ventricular filling volume that is ejected into the left ventricle under higher than normal pressure. o Patients with mitral valve prolapse often have a mid-to-late systolic click and a late systolic murmur. These patients are usually female and often have orthostatic hypotension. Patients with coronary artery disease can have the above mentioned murmur any time during systole, accompanied by an atrial gallop. In acute mitral regurgitation, the examination usually is consistent with acute pulmonary edema and left ventricular failure. o The heart size usually is normal, but an audible systolic thrill is often present. o The murmur often is harsh. It may be heard over the back of the neck, vertebra, and/or sacrum and may radiate to the axilla, back, and left sternal border.

Causes

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Acute rheumatic heart disease remains a significant consideration in those with mitral regurgitation who are younger than 40 years. Mitral valve prolapse (MVP) (ie, myxomatous degeneration) accounts for approximately 45% of the cases of mitral regurgitation in the Western world. o The causative agent is unknown in this condition. o Myxomatous degeneration is usually a slow process, with a major complication being the rupture of the chordae tendineae. (Acute regurgitation, as mentioned earlier, can be caused by chordae tendineae rupture or papillary muscle dysfunction.) o The literature now seems to suggest that MVP has become the most common cause of mitral regurgitation in the adult population. In addition, MVP and coronary artery disease (CAD) have become major mechanisms for incompetence of the mitral valve. o Ischemia is responsible for 3-25% of mitral regurgitation. o The severity of regurgitation is directly proportional to the degree of left ventricular hypokinesis. Mitral annular calcification can contribute to regurgitation. Impaired constriction of the annulus results in poor valve closure. Left ventricular dilatation and heart failure can produce annular dilatation and poor valve closure resulting in mitral regurgitation. Tendineae rupture can be due to endocarditis, myocardial infarction, or trauma. Papillary muscle dysfunction usually is caused by myocardial ischemia or infarction. Other causes include the following: o Ehlers-Danlos syndrome o Marfan syndrome o Osteogenesis imperfecta o Systemic lupus erythematosus (SLE)

Differential Diagnoses
Aortic Stenosis Congestive Heart Failure and Pulmonary Edema Mitral Valve Prolapse Myocardial Infarction Myocarditis

Other Problems to Be Considered

Idiopathic hypertrophic subaortic stenosis (IHSS)

Workup

Imaging Studies
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Chest radiography o The cardiac silhouette often is normal in patients with mitral valve prolapse (MVP). o With chronic mitral regurgitation, left ventricular and left atrial enlargement are present.

The left atrium can be large enough that it produces elevation of the left mainstem bronchus. o Occasionally, the double density sign can be seen along the right heart border, which is produced by the shadow of the wall of the dilated left atrium. o The heart size of patients with coronary artery disease (CAD) can range from normal to significant dilatation of the left ventricle and left atrium. o Mitral regurgitation presents with acute pulmonary edema and a normal cardiac silhouette with acute mitral regurgitation that is secondary to a rupture of a valve apparatus. Two-dimensional echocardiography o Evidence of posterior motion of valve leaflets during mid-systole is present in patients with mitral valve prolapse. o Annular calcifications may be seen in patients with coronary artery disease. In addition, evidence of posterior or inferior wall motion abnormalities may be observed. o With acute mitral regurgitation, the ruptured chordae tendineae or papillary muscle, as well as perforated interventricular septum, can be visualized. The left atrium and ventricle are generally of normal size. o Transesophageal echocardiography provides a better estimate of the severity of damage.
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Other Tests
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Electrocardiography o Chronic mitral regurgitation  Atrial fibrillation often is present secondary to a dilated left atrium.  The ECG shows evidence of left ventricular hypertrophy and left atrial enlargement. o CAD: Evidence of inferior and posterior Q waves may be present, indicating prior infarction. o MVP  Patients most commonly have ST- and T-wave changes, with T-wave inversions in the inferior leads.  ECG may reveal an underlying arrhythmia (eg, sinus arrhythmia, sinus arrest, atrial fibrillation, premature ventricular contractions [PVCs]). o Acute mitral regurgitation: ECG may reveal evidence of an acute myocardial infarction, more commonly inferior or posterior.

Procedures
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Cardiac catheterization o Angiography is considered to be the criterion standard in the assessment of the severity of the disease. o Mitral regurgitation is graded on a scale from 0 (none), 1 (mild), 2 (moderate), 3 (moderately severe), to 4 (severe). o The severity is based on the opacity of the left atrium. o The regurgitant volume can be calculated based on information from the catheterization.

In addition, this test will identify those with underlying CAD.