mitral valve prolapse (mvp) myxomatous degeneration of the mitral valve (floppy mitral valve,...

Mitral Valve Prolapse (MVP)• MYXOMATOUS degeneration of the mitral valve• (Floppy mitral valve, Billowing mitral leaflets).• Associated with connective tissue disorders like Marfan

syndrome.• Autosomal dominant

• Common in young women with familial incidence.• Usually posterior cusps prolapses.

• An 18-year-old Caucasian female is referred to the cardiologist because her primary care physician heard a cardiac murmur during a routine check-up.

• Careful auscultation reveals a mid systolic click which is followed by a short late systolic murmur. The murmur disappears on squatting. This patient’s condition is most likely related to an abnormality.

• What is the abnormality?

Causes of MVP

• Myxomatous degenaration• Large leaflets• Annulus dilation• Long chordae tendinae• Papillary muscle dysfunction.

• The conditions associated with MVP• Marfan syndrome• Thyrotoxicosis• RHD• IHD• Hypertrophic cardiomyopathy• ASD

• normal variant:- Mild mitral valve proplase is considered as normal.• common in Anxiety neurosis and in

females.

• Most commonly it involves the posterior cusps.• READ’S syndrome in which both anterior and

posterior cusps prolapse.• In 30 to 40% of the cases there is involvement of

tricuspid valve.• Aortic and pulmonary valve involvement is also seen

in some cases.

The mitral valve is floppy or prolapsed like balloon back to left atrium.

There is myxomatous degeneration in the tissue.The affected leaflets are redundant, rubbery and

thick.The tendinous cords are thin elongated and

sometime rupture.

• Histologically, the essential change is thinning of the fibrosa layer of the valve, on which the structural integrity of the leaflet depends accompanied by expansion of the middle spongiosa layer with increased deposition of myxomatous (mucoid) material.

• Clinical features• Present with • Atypical chest pain • palpitation , • dyspnea and fatigue• Midsystolic click and • systolic murmur due

to MR.

• Diagnosis• Chest x ray• ECG• Echocardiogram

• Complications• IE,• Arrhythmias• Stroke and sudden death.• MR.

• Mitral valve prolapse (MVP) is the most common cardiac abnormality predisposing to native

• valve bacterial endocarditis (NVBE) in the 15- to 60-year-old age group in the United States.

• MVP predisposes to infected vegetations on mitral leaflets. Rheumatic valvular disease is also a

• potential, although less common, precipitant of NVBE.

• A 75-year-old Caucasian male presents to your office complaining of worsening dyspnea and fatigue on exertion. He adds that he has had several episodes of severe lightheadedness during physical activity recently. Physical

examination reveals a harsh ejection-type systolic murmur at the base of the heart that radiates to the neck. You inform the patient that the most likely cause of his problem is:

• A. Repetitive inflammation of the aortic valve cusps• B. Infectious vegetation attached to his aortic valve• C. Extensive calcium deposition in his aortic valve leaflets• D. Congenital heart defect producing supravalvular stenosis• E. Mitral valve deformity due to myxomatous degeneration of the

leaflets

AORTIC STENOSIS

The most frequent calcific valvular disease

LVH (but no hypertension), ischemia

Cardiac decompensation, angina, CHF

50% die in 5 years if angina present

50% die in 2 years if CHF present

• Wear and tear of normal or congenital bicuspid valves• Wear and tear degeneration• And dystrophic calcification and

passive accumulation of calcium (hydroxyapatite)

• Chronic injury due to• Hypertension• Hyperlipidemia• Inflammation• Leads to calcification

• Obstruction to left ventricular outflow leads to narrowing of the valve orifice( 4 cm2)

• Severe narrowing is 0.5 needs surgical correction

• Moderate is 1.5 to 1 • Mild is 2.5 to 2

Clincial features

• Dyspnea,• Myocardial ischemia occur with out coronary

artery lesion.• angina pain Due to LVH there is ischemia• Syncope due to decreased blood flow.• Ejection systolic murmur in the aortic area

conducted to carotids.• Fatigue and pulmonary edema.• Sudden death.

ACUTE:-Inflammation

-Aschoff bodies

-Anitschkow cells

-Pancarditis

-Vegetations on chordae tendinae at leaflet junction

CHRONIC:THICKENED VALVES

COMMISURAL FUSION

THICK, SHORT, CHORDAE TENDINAE

RHEUMATIC fever and Rheumatic Heart Disease

Rheumatic fever (RF) is an acute, immunologically mediated, multisystem inflammatory disease that occurs a few weeks after an episode of group A β-hemolytic streptococcal pharyngitis.

Acute rheumatic heart disease (RHD) is the cardiac manifestation of RF and is associated with inflammation of the valves, myocardium, or pericardium.

• Acute RF is a hypersensitivity reaction induced by host antibodies elicited by group A streptococci.

• M proteins of certain streptococcal strains induce host antibodies that cross-react with glycoprotein antigens in the heart, joints, and other tissues.

• Incubation period 2- to 3 weeks.• More common in boys.• Occurs between 5 to 15 years of age group.

• After an initial attack there is increased vulnerability to disease reactivation with subsequent pharyngeal infections.

• Rheumatic carditis usually does not cause clinical manifestations for years or even decades after the initial episode of RF.

Pathogenesis.

• Acute RF is a hypersensitivity reaction induced by host antibodies elicited by group A streptococci.

• Antibodies against the M proteins of the streptococcal organism will cross react with the glycoprotein antigen of the heart and joints and other tissues.

• The Aschoff bodies made up of Inflammtory cells esp T lymphocytes occassional plasma cells and activated macrophages called Anitschkow cells and these anitschkow cells have abundant cytoplasm and central nucleus and the chromatin arranged in wavy ribbon form called caterpillar cells.

• Fish mouth or button hole due to fibrous bridging calcification along the commissural.

• Aschoff bodies can be found in any of the three layers of the heart-pericardium, myocardium, or endocardium (including valves)-

• Pancarditis(Inflammation of the all three layers )

• Valve involvement results in fibrinoid necrosis along the lines of closure forming 1- to 2-mm vegetations (verrucae)

• Chronic RHD is characterized by organization of the acute inflammation and subsequent scarring. The changes of the mitral (or tricuspid) valve include leaflet thickening, commissural fusion and shortening, and thickening and fusion of the chordae tendineae Fibrous bridging across the valvular commissures and calcification create "fish mouth" or "buttonhole" stenoses

• RHD is overwhelmingly the most frequent cause of mitral stenosis accounting for 99% of cases. The mitral valve alone is involved in 70% of cases of RHD

• With tight mitral stenosis, the left atrium progressively dilates and may harbor mural thrombi

CLINICAL FEATURES• Migratory Polyarthritis• Myocarditis• Subcutaneous nodules• Erythema marginatum• Sydenham chorea

Jones criteria

• Major criteria• Migratory polyarthritis• Carditis• Sydenham’s chorea• Subcutaneous nodules• Erthyema marginatum

• Minor criteria• Fever• Elevated ESR• Raised ASO• Prolonged PR

interval• Arthralgia

• Lab Raised ASO titer• Raised ESR• Prolonged PR interval• Echo reveals mitral stenosis• Treatment-• Benzathine penicillin 1.2 to 2.4 megaunits

ASHOFF BODIES (RF)

ASCHOFF BODIES

ANITSCHOW CELLS

INFECTIOUS ENDOCARDITIS

• MicrobesUsually strep viridans– Often Staph aureus in IVD users– Enterococci

–HAČEK (normal oral flora, gram - , in children)• Hemophilus influenzae• Actinobacillus• Cardiobacterium• Eikenella• Kingella

– Fungi, rickettsiae, chlamydia

Infective endocarditis

• Infective endocarditis (IE) is a serious infection which is characterized by microbial invasion of heart valves or mural endocardium-often with destruction of the underlying cardiac tissues-and results in bulky, friable vegetations composed of necrotic debris, thrombus, and organisms.

Commonly involved valves,

• Normal valves in acute IE• Mitral valve and aortic valve and mainly

tricuspid valve in drug abusers,• Prosthetic valves and deformed valves, • calcified aortic valves.

INFECTIVE ENDOCARDITIS• Acute: 50% mortality• It involves the normal valves

and highly virulent organism• Sub-acute: Low mortality low

virulent organisms.

Morphology

• In both acute and subacute forms of the disease, friable, bulky, and potentially destructive vegetations containing fibrin, inflammatory cells, and microorganisms are present on the heart valves.

• The aortic and mitral valves are the most common sites of infection,

• Tricuspid valve is a frequent target in the setting of intravenous drug abuse.

• These vegetations form • systemic emboli• or septic emboli

Pathogenesis

• IE is seen in • RHD• MVP• Prosthetic valves(S,epidermidis)• Drug abusers• Previously damaged or otherwise abnormal

valves (50% to 60% of cases) viridans Streptococci

Clinical features(Acute and subacute)

• Acute endocarditis Fever is the most

consistent sign.• Rapidly developing

fever, chills, weakness, and lassitude.

• Presence of a new murmur.

• Subacute IE (particularly in the elderly)

• fever may be absent,• fatigue, • weight loss, and a flulike

syndrome. Splenomegaly is common

Complications

• Begin within the first weeks of the onset of IE. • Glomerulonephritis due to glomerular

trapping of antigen-antibody complexes, thus giving rise to hematuria, albuminuria, or renal failure (glomerulonephritis);

• Septicemia,• Arrhythmias (suggesting invasion into

underlying myocardium),

• Ring abscess • Infective endocarditis

VEGETATIONS• INFECTIVE >5mm• NON-Infective <5mm

DIAGNOSIS DUKES CRITERIA

• MAJOR• Positive blood culture(s) indicating characteristic

organism or persistence of unusual organism• Echocardiographic findings, including valve-related

or implant-related mass or abscess, or partial separation of artificial valve

• New valvular regurgitation

Minor Criteria

• Fever• Vascular lesions, including • arterial petechiae, • subungual/splinter hemorrhages,• emboli, septic infarcts, • mycotic aneurysm,• intracranial hemorrhage, • Janeway lesions

• Immunologic phenomena, including glomerulonephritis,

• Osler nodes, • Roth spots, • Microbiologic evidence, including single

culture showing uncharacteristic organism• Echocardiographic findings consistent with but

not diagnostic of endocarditis, including new valvular regurgitation, pericarditis.

Pathologic criteria

• Microorganisms demonstrate in vegetations,• Histologic confirmation of active

endocarditis in vegetation.

• The criteria holds for • 2 major, OR• 3 minor and 1 major OR 5 minorAlways helps in the diagnosis of IE

• A 50-year-old man with adenocarcinoma of the pancreas is brought to the emergency room in a comatose state. A CT scan of the brain is consistent with a recent infarct in the left

• temporal lobe. Blood cultures are negative. The patient never regains consciousness and expires 2 days later. The heart at autopsy is shown in the image. Which of the following is the most likely underlying cause of stroke in this patient?

NBTE

• Non bacterial thrombotic endocarditis• (Marantic endocardits)• Vegetations seen along the line of closure of

the valve leaflets• Seen in elderly and malignant patients.• Concomitantly seen with venous and PE

suggesting Hyper coaguable state.• Also in mucin producing tumors.

Nonbacterial Thrombotic Endocarditis

• These are deposition of variably sized masses of fibrin, platelets, and other blood components on cardiac valves.• the valvular lesions of NBTE are • Sterile and • Absence of Microorganisms.

• It is present in • healthy individuals,• Diseases associated with general

debility or wasting are associated with an increased risk of NBTE, called marantic endocarditis.

Pathogenesis

• Occur in hypercoagulable states,• Sepsis with disseminated intravascular

coagulation • Hyperestrogenic states, • Malignancy, particularly mucinous

adenocarcinomas., • Endocardial trauma (e.g., from an indwelling

catheter) is also a well-recognized predisposing condition.

Morphology

• NBTE vegetations are sterile, nondestructive, and small (1mm); they occur singly or multiply along the line of closure of the leaflets or cusps.

• Clinically it is significant by embolizing to the brain, heart, or other organs. NBTE can also serve as a potential nidus for bacterial colonization and thus the development of IE.

NBTE AND DISSEMINATED CANCER

• The pathogenesis of nonbacterial thrombotic endocarditis (NBTE) often involves a

• Hypercoagulable state. When hypercoagulability is the result of the procoagulant effects of

• circulating products of cancers, the resulting cardiac valve vegetations may also be called

• MARANTIC ENDOCARDITIS.

• Or • NON BACTERIAL • ENDOCARDITIS.

• A 30-year-old woman presents with a heart murmur. There is a history of recurrent episodes of arthritis, skin rash, and glomerulonephritis.

• Blood cultures are negative. Laboratory tests• for antinuclear antibodies (ANA) and anti–double-stranded DNA

are positive. Which of the following is the most likely cause of heart murmur in this patient?

• (A) Libman-Sacks endocarditis• (B) Mitral valve prolapse• (C) Myocardial infarct• (D) Mitral valve prolapse• (E) Rheumatic fever

Libman-Sacks Endocarditis

• Libman-Sacks endocarditis refers • to sterile vegetations that can develop on the

cardiac valves of patients with systemic lupus erythematosus.

• These lesions are due to accumulation of immune complex fibrin and mononuclear cells.

• With increasing use of steroids for treatment of lupus, Libman-Sacks endocarditis has become fairly uncommon.

• Embolization of the lesion is very rare.

Libman sack endocarditis

• Commonly involves mitral and tricuspid valve.• Vegetations are

present under the surface of the AV valves.

Carcinoid Syndrome• Episodic skin flushing• Cramps• Nausea & Vomiting• Diarrhea

•↑serotonin, ↑ 5HIAA in urine(Hydroxyindeloacetic acid)

• FIBROUS INTIMAL THICKENING– RV, Tricuspid valve, Pulmonic valve (all RIGHT side)– Similar to what Fen-Phen does on the LEFT side

ARTIFICIAL VALVES• Mechanical• Xenografts (porcine)

• 60% have complications within 10 years

Prosthetic Cardiac Valves

• There are Two types of prosthetic valves.•1.Mechanical valves•2.Bioprosthetic valves

Mechanical valves• Double tilting disk devices

made of pyrolytic carbon• Excellent durability• Require chronic

anticoagulation,• cause significant red cell

hemolysis due to shear stresses

Bioprosthetic valves• Glutaraldehyde-fixed porcine or

bovine tissue.• Do not require anticoagulation

but are less durable and can fail because of matrix deterioration.

• Biologic valve leaflets undergo some degree of stiffening after implantation

• Calcification is common. Tear and valvular insufficiency is also common.