1. 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.

2. 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?

4. Causes of MVP
Myxomatous degenaration
Large leaflets
Annulus dilation
Long chordae tendinae
Papillary muscle dysfunction.

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

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

7. 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.

8. 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.

9. 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.

10. systolic murmur due to MR.
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

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

12. 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.

13. 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

14. 50% die in 2 years if CHF present
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
LVH is almost a reflex knee jerk conclusion to AS, but in this case there may be NO systemic hypertension.

15. Wear and tear of normal or congenital bicuspid valves
Wear and tear degeneration
And dystrophic calcification and passive accumulation of calcium (hydroxyapatite)

16. Chronic injury due to
Hypertension
Hyperlipidemia
Inflammation
Leads to calcification

17. 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

18. 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.

19. ACUTE: CHRONIC: -Inflammation -Aschoff bodies -Anitschkow cells
-Pancarditis
-Vegetations on chordae tendinae at leaflet junction
CHRONIC:
THICKENED VALVES
COMMISURAL FUSION
THICK, SHORT, CHORDAE TENDINAE
Granuloma:Giant Cell::Aschoff Body:Anitschkow Cell

20. 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.

21. 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.

22. 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.

23. 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.

24. 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.

25. 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)

26. 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

27. 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

28. CLINICAL FEATURES Migratory Polyarthritis Myocarditis
Subcutaneous nodules
Erythema marginatum
Sydenham chorea
A GREAT classical Sydenham chorea (St. Vitus “Dance”) can be seen at

29. Jones criteria Fever Elevated ESR Raised ASO Prolonged PR interval
Major criteria
Migratory polyarthritis
Carditis
Sydenham’s chorea
Subcutaneous nodules
Erthyema marginatum
Minor criteria
Fever
Elevated ESR
Raised ASO
Prolonged PR interval
Arthralgia

30. The great BRIDGE between infectious and autoimmune diseases!
Also note this is a PAN-carditis, i.e., :
1) Endocardium
2) Myocardiom
3) Pericardium

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

32. ASHOFF BODIES (RF)

33. ASCHOFF BODIES

34. ANITSCHOW CELLS

35. 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
What is a haček?

36. 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.

37. 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.

38. INFECTIVE ENDOCARDITIS
Acute: 50% mortality
It involves the normal valves and highly virulent organism
Sub-acute: Low mortality low virulent organisms.
What is difference between “ACUTE” and “SUBACUTE” bacterial endocarditis? Ans: RATE and SEVERITY. The histology may be identical.

39. 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.

40. These vegetations form
systemic emboli
or septic emboli

41. 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

42. 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

43. 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),

44. Ring abscess
Infective endocarditis

45. VEGETATIONS INFECTIVE >5mm NON-Infective <5mm
Vegetations: 1) rheumatic = small, at chordae tendinae junction, 2) infectious = big (>5 mm), 3) lupus (Libman-Saks) = BOTH sides
4) NBTE = non-bacterial thrombotic endocarditis (<5 mm)

46. 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
Another diagram which shows “quantification” of a diagnosis

47. Minor Criteria Fever Vascular lesions, including arterial petechiae,
subungual/splinter hemorrhages,
emboli, septic infarcts,
mycotic aneurysm,
intracranial hemorrhage,
Janeway lesions

48. 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.

49. Pathologic criteria
Microorganisms demonstrate in vegetations,
Histologic confirmation of active endocarditis in vegetation.

50. The criteria holds for
2 major, OR
3 minor and 1 major OR
5 minor
Always helps in the diagnosis of IE

51. Splinter hemorrhages, Janeway lesions (palms, soles), Osler’s “nodes” (raised), Roth’s spots (eye).
Do you think that for every skin lesion you see there may be 10 visceral lesions which you do NOT see? Ans: Yes, I believe so!

52. 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?

53. 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.

54. 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.

55. It is present in
healthy individuals,
Diseases associated with general debility or wasting are associated with an increased risk of NBTE, called marantic endocarditis.

56. 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.

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

58. 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.

59. 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.

60. A 30-year-old woman presents with a heart murmur
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

61. Libman-Sacks Endocarditis
Libman-Sacks endocarditis refers
to sterile vegetations that can develop on the cardiac valves of patients with systemic lupus erythematosus.

62. Embolization of the lesion is very rare.
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.

63. Libman sack endocarditis
Commonly involves mitral and tricuspid valve.
Vegetations are present under the surface of the AV valves.

64. 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
Most carcinoids are from the GI tract, but only 10% of carcinoids produce “carcinoid syndrome”.
Why are carcinoids selectively nasty to the RIGHT heart intima? ANS: Lungs break down serotonin.

65. ARTIFICIAL VALVES Mechanical Xenografts (porcine)
60% have complications within 10 years

66. Prosthetic Cardiac Valves
There are Two types of prosthetic valves.
1.Mechanical valves
2.Bioprosthetic valves

67. Mechanical valves Bioprosthetic valves
Double tilting disk devices made of pyrolytic carbon
Excellent durability
Require chronic anticoagulation,
cause significant red cell hemolysis due to shear stresses
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.