VALVULOPATHY
![](09ce08f0.jpg) The mitral valve has a glistening white appearance and on pathologic review the abnormality is a "stuck on" plaque-like structure on the valve leaflet without destruction of the leaflet itself. We also know that patients who have carcinoid syndrome have a high level of circulating serotonin. Patients with carcinoid disease that have higher serotonin levels are more likely to develop cardiac disease.
We examined a tricuspid valve that was removed from a patient who had carcinoid heart disease and again noted the thickening of the cordal structures and leaflets, and the glistening white appearance
Valvulopathy: Pathology
Myxomatous degeneration of the mitral valve
Calcific stenosis of the aortic valve
Endocarditis and stenosis/ regurgitation
Carcinoid degeneration of the tricuspid valve
Compensatory cardiac chamber enlargement
Valvulopathy: Laboratory
Echocardiogram defines valve structure and function
Blood cultures for infection
Chest X-ray may demonstrate chamber enlargement
Valvulopathy: Clinical Presentation
Dyspnea, orthopnea
Palpitations
Chest pain
Findings are relative to the individual lesion
ascultory changes: murmurs
pulse changes: weak or bounding
Peripheral/ pulmonary edema
The most common heart valve abnormality is called mitral valve prolapse (MVP), which affects between 5 and 20 percent of the population. It is more commonly found in women than men and can go undetected for years, as symptoms usually do not occur until adolescence or even adulthood. MVP is most commonly diagnosed among patients between the ages of 20 and 40.
What is it?
Mitral valve prolapse is a condition of the mitral valve, a two-flapped heart valve between the left atrium and left ventricle. In MVP, one or both of the valve flaps are too large, and the mitral valve does not close evenly with each heartbeat.
Because of this imperfect closing, the valve itself slightly balloons back into the left atrium, sometimes causing what is known as a "click". With the flap there may sometimes be a slight backward leaking of blood (regurgitation) as well, resulting in a heart murmur.
Mitral valve prolapse seems to be an inherited disorder, although the precise genes are not known. If proper precautions are taken (see below) it will not affect life expectancy, and generally has no impact on normal activities.
What are the symptoms?
Approximately 60 percent of individuals with MVP never exhibit any symptoms. Generally, a stressful situation (childbirth, change in job situation, viral illness) brings on symptoms which ordinarily would not be present. Some of these symptoms include the following:
Irregular heartbeat or palpitations, particularly when lying on the left side
Tachycardia, increased heartbeats or pounding of the chest, often after exertion
Non-specific chest pain lasting from a few seconds to several hours, occurring at rest rather than during exertion
Panic attack, a sudden feeling of anxiety or doom
Fatigue and weakness, even after slight exertion (including minor housework); sometimes misdiagnosed as Chronic Fatigue Syndrome
Migraine headaches, resulting from abnormal nervous system control of blood flow
As noted above, many people with MVP never exhibit any of the above symptoms.
The condition can be detected during a routine check-up with a simple stethoscope.
After the ventricle begins to contract, a clicking sound can be heard - the sound of the abnormal valve fighting the pressure of the left ventricle.
The diagnosis can be confirmed with an echocardiogram or cardiac echo; the echocardiogram can also determine the level of severity of the prolapse and the degree of regurgitation.
Most patients can be monitored simply, with a follow-up checkup every few years.
Patients with pronounced regurgitation problems (blood leaking backward) may be monitored more closely.
What are the risks and problems associated with MVP?
Just as many MVP patients exhibit no symptoms, very few patients ever experience any complications arising from this syndrome.
Rare complications include chest pain (angina pectoris) and irregular heart beat (arrhythmia), both of which can be treated with medication, usually a beta-blocker.
Another rare complication involves formation of blood clots on the valve, making an MVP patient vulnerable to strokes; this problem requires treatment with an anticoagulant (blood thinner) medication.
The most common and serious MVP-related problem, endocarditis, involves bacterial infection of the mitral valve.
Although it can be fatal if left untreated, endocarditis can be easily prevented. MVP patients are most commonly vulnerable to introduction of bacteria into the bloodstream (and endocarditis) when they are undergoing certain medical procedures, particularly dental work or minor surgery.
Because of this, patients should inform their doctor or dentist that they have MVP, and be given antibiotic prophylaxis (preventative treatment) before the procedure.
When does a patient require antibiotics?
The American Heart Association has recently updated its recommendations for surgical and dental treatments in patients who have MVP and other heart disorders. The use of antibiotics is important for preventing bacterial endocarditis, a rare but potentially fatal infection that causes inflammation of the heart's valves, or its inner lining. The use of antibiotics for individuals who have MVP has been somewhat controversial.
The new guidelines suggest that for many procedures, most MVP patients do not need antibiotics; antibiotics are only required for those individuals who have valve leakage (mitral regurgitation) either detected as a heart murmur or through an ultrasound, or people with greatly thickened valve tissues.
The rare exception
Although most MVP patients do very well with the treatments and preventive measures outlined above, there is sometimes need for heart surgery to either repair or replace the mitral valve. This occurs only among patients who experience severe mitral regurgitation, which can result in progressive heart enlargement, and ultimately, heart failure.
Surgeons are more likely to perform corrective surgery rather than replace the valve with an artificial one, mainly because the introduction of an artificial valve requires lifelong use of blood thinners to prevent clotting. If a patient is found to have regurgitation problems, surgery is recommended and performed at as young an age as possible, reducing the risk of further damage to the heart.
Calcific Aortic Stenosis
Etiology:
• 3 different etiologies:
• Rheumatic fever.
• Congenital aortic stenosis.
• Congenital bicuspid aortic valve.
• Unknown: Degenerative calcific aortic stenosis.
Pathogenesis:
• Rheumatic calcific aortic stenosis: Fusion of the valve commissures in the healing phase of valvulitis initiates stenosis. Deformity with obstruction to blood flow results in hemodynamic fibrosis and calcification, which progresses as obstructions increase.
• Congenital bicuspid valve: Not functionally stenotic at birth or in early adult life, but susceptible to acquired fibrosis and calcification causing stenosis in 6th and 7th decades. This susceptibility may be due to hemodynamic cummulative injuries due to the deformity.
• Degenerative calcific stenosis: This is acquired on a previously normal valve. Pathogenesis unknown, but often attributed to "wear and tear" effects implying hemodynamic injury. A weak explanation. Original deformity not obstructive
Epidemiology:
• Rheumatic: Less than 10% of cases in U.S.. Usually associated with healed rheumatic mitral valvulitis.
• Congenital bicuspid valve: Deformity alone does not cause stenosis, in contrast to congenital aortic stenosis present from birth. (see pathogenesis).
• Degenerative calcific stenosis: An acquired disease of old age manifested usually in the eighth decade
General Gross Description:
• The left ventricle is hypertrophied in all these cases, regardless of pathogenesis.
• The rheumatic cases show fusion of the cusp commissures with variable degrees of superimposed fibrosis and calcifications.
• The bicuspid cases show thickening of the cusps with variable degrees of calcifications. One of the cusps will show a thickened fold dividing it into two smaller cusps, as if it resulted from a congenital fusion of one commissure. The calcifications can be deforming and bulky.
• The so-called "degenerative cases" of unknown cause show 3 distinct separate cusps, thickened by calcifying fibrosis, and distorted by bulky irregular or nodular protruding calcific excresences.
General Microscopic Description:
• Fibrosis with hyalinization and calcification is the predominant microscopic change common to all forms.
• The deforming calcific masses which dominate the gross changes are amorphous calcified matter separated by a hyalin matrix. This is different from usual dystrophic calcification which is restricted to calcification of existing structures or scarring. These "degenerative" changes are not characteristic of arteriosclerosis.
Clinical Correlations:
• Asymptomatic until orifice area reduced to 1/3 normal.
• Blood pressure normal while asymptomatic. Reduced pulse pressure thereafter.
• Characteristic palpable thrill and murmur.
• Angina pectoris, exertional dyspnea, and syncope, are cardinal symptoms.
• Sudden death occurs in 10-20% of cases at average age of 60, presumably due to arrhythmia.
• Death occurs in 7th and 8th decades.
• Average survivals after onset of following manifestations are as follows: congestive heart failure - 1.5-2 yrs.; dyspnea - 2 yrs.; angina pectoris or syncope - 3yrs.
• Valve replacement indicated when any of the 3 cardinal symptoms appear.
• Asymptomatic diagnosed cases require careful followup.
Bicuspid Aortic Valve
Etiology:
• Congenital
Pathogenesis:
• Unknown for the congenital lesion.
• Acquired subsequent calcific stenosis attributed to "wear and tear" mechanisms with hemodynamic changes attributed to deformities.
Epidemiology:
• Affects 1-2% of the population.
General Gross Description:
• The two cusps are about equal in size. One of the two cusps is bisected into two deformed cusps by a thick fold extending from the annulus to the free margin, as if there were 3 cusps at first, one large and two smaller ones with fusion of the commissure between the two small ones.
• Fibrosis and extensive calcification may ensue causing a severe aortic stenosis. The resulting calcific deposits and excrescences can be very deforming.
General Microscopic Description:
• Fibrosis, hyalinization, and calcified amorphous matter are noted. The pathology is not that of arteriosclerosis or usual dystrophic calcification, which is not usually deforming.
Clinical Correlations:
• Asymptomatic until orifice area reduced to 1/3 normal.
• Blood pressure normal while asymptomatic. Reduced pulse pressure thereafter.
• Characteristic palpable thrill and murmur.
• Angina pectoris, exertional dyspnea, and syncope, are cardinal symptoms.
• Sudden death occurs in 10-20% of cases at average age of 60, presumably due to arrhythmia.
• Death occurs in 7th and 8th decades.
• Average survivals after onset of following manifestations are as follows: congestive heart failure - 1.5-2 yrs.; dyspnea - 2 yrs.; angina pectoris or syncope - 3yrs.
• Valve replacement indicated when any of the 3 cardinal symptoms appear.
• Asymptomatic diagnosed cases require careful followup.
MITRAL VALVE PROLAPSE
Mitral Valve
![](Symb075c00b700f000000000.gif) Prolapse is a common heart irregularity;
![](Symb075c00b700f000000000.gif) it has been estimated to occur in as many as 5% of the American population.
![](Symb075c00b700f000000000.gif) The condition is generally not life threatening and the majority of patients have no symptoms.
WHAT IS MITRAL VALVE PROLAPSE
![](Symb075c00b700f0ff000000.gif) The heart has two valves on each side.
![](Symb075c00b700f0ff000000.gif) The valves purpose is to make sure that blood flows through the heart the right way.
![](Symb075c00b700f0ff000000.gif) They act like a door. Mitral valve prolapse (MVP)is when the mitral valve flaps do not close properly.
![](Symb075c00b700f0ff000000.gif) As pressure builds inside the left ventricle (chamber) it pushes the mitral valve back("billow") into the left atrium (upper chamber) which may cause a small leak MVP is a common heart irregularity, and is also called the click murmur syndrome due to the click murmur heart sound that can be heard
![](0bda0fa0.gif) SOME OF THE MOST COMMON SYMPTOMS OF MVP SYNDROME
> Abnormally rapid throbbing or fluttering of the heart
>A feeling of apprehension, worry, uneasiness or dread that leads to problems in maintaining
attention and concentration.
>Chest pain >Rapid, irregular and shallow breathing.
>Feeling very tired for no apparent reason >
Depression >Sleep problems
>Problems with the digestive system >
Migraines >
Feeling dizzy and faint
>Cold hands and feet >
Being afraid that you are going crazy.
DETECTING
MITRAL VALVE PROLAPSE
MVP is being diagnosed today more frequently than in the past due to the heightened awareness of physicians, and better diagnostic tools.
It is most often diagnosed by stethoscope when listening very carefully to systolic click or murmur when they are present or by echocardiogram ( ultrasound imaging) which is a diagnostic test that utilizes sound waves to visualize the heart.
A 24 hour Holter monitor or an "event recorder" might be used to record abnormal rhythms.
Exercise testing(stress test) and the tilt table test are also helpful.
Approximately 5% of the population have been diagnosed with Mitral Valve Prolapse.
![](Symb075c00b700f000000000.gif) systolic click murmur syndrome, Barlow's Syndrome, floppy- valve syndrome, billowing mitral valve syndrome
![](Symb075c00b700f000000000.gif) one of the most common cardiac disease, up to 15% of population
![](Symb075c00b700f000000000.gif) highly variable presentation
![](Symb075c00b700f000000000.gif) majority of patient has unknown cause
![](Symb075c00b700f000000000.gif) more common in female, between age 14-30, in thinner and heavier people
![](Symb075c00b700f000000000.gif) increase familial incidence indicating AD inheritance with incomplete penetrance
![](Symb075c00b700f000000000.gif) broad spectrum of severity : systolic click, MR murmur, mild prolapse, severe MR, rupture of chordae, LV failure
Association:
![](Symb075c00b700f000000000.gif) Marfan syndrome, Ehlers-Danols syndrome, pseudoxanthoma elasticum
![](Symb075c00b700f000000000.gif) Turner's syndrome, relapsing polychrondritis
![](Symb075c00b700f000000000.gif) rheumatic endocarditis, MS
![](Symb075c00b700f000000000.gif) SLE, MCTD, scleroderma, CREST
![](Symb075c00b700f000000000.gif) ostium secundum ASD
![](Symb075c00b700f000000000.gif) CAD, HOCM
![](Symb075c00b700f000000000.gif) Hurler's, Hunter's
![](Symb075c00b700f000000000.gif) hyperthyrodism,VWD, anorexia nervosa
Pathology:
![](Symb075c00b700f000000000.gif) disproportion between the connective tissue elements of the MV apparatus and the muscular support structures
![](Symb075c00b700f000000000.gif) the leaflets, annulus and the chordae are too large for the LV comparatively
![](Symb075c00b700f000000000.gif) histology showed disorganized collagen fibres in the valve leaflets and increase content of acid mucopolysaccharide
![](Symb075c00b700f000000000.gif) posterior leaflet is usually more affected than the anterior, elongated chordae contributed to the MR
![](Symb075c00b700f000000000.gif) the MVP cause excessive stress to the papillary muscle --> dysfunction and ischemia of the muscle and subjacent myocardium --> rupture of the chordae --> MR --> more stress to the MV apparatus
Symptoms:
![](Symb075c00b700f000000000.gif) chest pain (most frequent cause for seeking medical advice), may be due to papillary muscle ischemia, coronary spasm or oesophageal motility disorder
![](Symb075c00b700f000000000.gif) palpitation, dysponea, decrease ET, malaise
![](Symb075c00b700f000000000.gif) syncope, pre-syncope
![](Symb075c00b700f000000000.gif) anxiety, panic disorder
Terminology:
mitral valve prolapse syndrome : auscultatory or echo evidence of MVP with non-specific symptoms
mitral valve prolapse : auscultatory or echo evidence of MVP who are either aysymptomatic or have clinical manifestations directly attribute to the valvular abnormality
Physical Examination:
![](Symb075c00b700f000000000.gif) mid or late systolic click (sudden tensing of the slack elongated chordae or by the prolapseing leaflet when it reaches the maximum excursion)
![](Symb075c00b700f000000000.gif) high pitch late systolic cresendo-decrescendo murmur
![](Symb075c00b700f000000000.gif) intervention which decrease the LV volume cause the click to occur earlier e.g. valsalva, standing
![](Symb075c00b700f000000000.gif) intervention which increase the LV volume delay the click-murmur complex e.g. squatting, isometric exercise
ECG:
![](Symb075c00b700f000000000.gif) usually normal
![](Symb075c00b700f000000000.gif) biphasic or inverted T waves in II, III, aVf
![](Symb075c00b700f000000000.gif) occasional SVT or VE
ECHO:
![](Symb075c00b700f000000000.gif) systolic displacement (billowing) of the MV leaflets into the LA beyond the plane of the mitral annulus in parasternal view
![](Symb075c00b700f000000000.gif) late systolic posterior motion of the mitral leaflet on M-mode
![](Symb075c00b700f000000000.gif) thickened and redundancy of the leaflets
![](Symb075c00b700f000000000.gif) doppler --> MR severity
![](Symb075c00b700f000000000.gif) LV or LA dilation (LA dilatation is shown to be the best predictor for future valvular replacement)
Complications:
![](Symb075c00b700f000000000.gif) SVT, VE, tachyarrthymias (may be due to stimulation of the atrial pacemaker by the MR jet or the prolapsed valve, origin of impulse from the electrically active cells recently shown to have adrenergic receptors in the MV leaflet
![](Symb075c00b700f000000000.gif) sudden death (rare)
![](Symb075c00b700f000000000.gif) severe MR, chordae rupture
![](Symb075c00b700f000000000.gif) infective endocarditis
![](Symb075c00b700f000000000.gif) systemic embolisation usually cerebral, TIA
![](Symb075c00b700f000000000.gif) serious complication occurs in less than 5% of all patients
![](Symb075c00b700f000000000.gif) patient with leaflet thicker than 5mm, severe MR (systolic murmur), old age (>45), male are predisposed to more complications
Treatment:
![](Symb075c00b700f000000000.gif) reassurance for mild or asymptomatic patients
![](Symb075c00b700f000000000.gif) prevention of infective endocarditis with antibiotic prophylasis (especially for MR+ and thicken MV leaflet)
![](Symb075c00b700f000000000.gif) -blocker for arrthymias and chest pain (atypical)
![](Symb075c00b700f000008000.gif) mitral valve repair for severe MR
![](Symb075c00b700f000000000.gif) aspirin or even anticoagulant for TIA
Suggested Protocol of Management:
![](Symb075c00b700f000000000.gif) No specific treatment and no antibiotic prophylasis : for woman age under 45 with no systolic murmur and no MR in doppler --> FU 5 yearly
![](Symb075c00b700f000000000.gif) antibiotic prophylasis : for MR+ and murmur+ patients
![](Symb075c00b700f000000000.gif) antihypertensive treatment : for older man with HT and severe MR --> FU 2-3 yearly
![](Symb075c00b700f000000000.gif) antihypertensive, antibiotic prophylasis +/- surgery : for severe MR and older patients
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