1. VALVULAR HEART DISEASES
8/1/2014

2. Normal heart valves

3. Normal heart valves

4. Valvular heart disease
• A major group of cardiac pathology affecting
cardiac valves.

5. Clinical consequences
1. Stenosis – failure of a valve to open completely,
obstructing forward flow.
2. Insufficiency (regurgitation ) - failure of a valve to
close completely, allowing (backflow) of blood.
Stenosis or regurgitation can occur alone or together in
the same valve.

6. What is STENOSIS ?
What is REGURGITATION?

7. Clinical consequences
• Abnormal flow through diseased valves - produces
abnormal heart sounds (murmurs)
• severe lesions can even be palpated as thrills.
• severity - quality and timing of the murmur
(e.g., harsh systolic or soft diastolic murmurs)

8. Valvular heart disease
Types
1. Congenital valvular heart diseases
2. Acquired valvular heart diseases

9. Congenital valvular heart diseases
 most common – bicuspid aortic valve
• neither stenotic nor incompetent through early life
• more prone to early and progressive degenerative
calcification

10. Acquired valvular
heart diseases

11. Most Common Causes
1. AS: calcification of anatomically normal and
congenitally bicuspid aortic valves
2. AR: dilation of ascending aorta, usually related to
hypertension and aging
3. MS: rheumatic heart disease
4. MR: myxomatous degeneration (mitral valve prolapse)

12. Aortic stenosis
Most common cause –
calcification of
1.
anatomically normal (senile calcific aortic stenosis) and
2.
congenitally bicuspid aortic valves

13. Pathogenesis
• Degenerative changes due to aging process (wear and tear)
• Repetitive mechanical stresses to valves —40 million
beats/yr
• chronic injury due to hyperlipidemia, hypertension,
inflammation, atherosclerosis leads to .......
• dystrophic calcification (deposits of calcium phosphate salts)
• Normal valves - Senile calcific aortic stenosis >70 yrs
• Bicuspid valves – more stress – earlier calcification <50 yrs

14. MORPHOLOGY
• heaped-up calcified masses on outflow side of cusps

15. Clinical Features
• gradual narrowing of the valve orifice ( 0.5 to 1 cm2 in severe
AS ; normal, ∼4 cm2 )
• Left ventricular pressures - > 200 mm Hg
• Pressure overload  concentric LVH
• hypertrophied myocardium -prone to ischemia and angina
• Systolic and diastolic dysfunction – CHF

16. calcific aortic stenosis
Prognosis
• Asymptomative at earlier stage – excellent
• Late stage - development of angina, CHF, or syncope
 poor prognosis
• without surgical intervention, 50% to 80% die within 2
to 3 years

17. Most Common Causes
1. AS: calcification of anatomically normal and
congenitally bicuspid aortic valves
2. AR: dilation of ascending aorta, usually related to
hypertension and aging
3. MS: rheumatic heart disease
4. MR: myxomatous degeneration (mitral valve prolapse)

18. Myxomatous Mitral Valve
• Mitral prolapse - parachute-like protrusion of value into the
left atrium
• “floppy” and prolapse— balloon back into LA during systole.
• Men = women
Two types
1. P
2. Secondary where MR due to others(e.g., IHD).

19. Myxomatous Mitral Valve
Causes
1. primary myxomatous degeneration
• intrinsic defect of connective tissue synthesis or remodeling
(e.g., Marfan syndrome)
2. Secondary
• results from injury to the valve myofibroblasts, by
chronically aberrant hemodynamic forces

20. Pathogenesis
 Myxoid degeneration due to accumulation
of glycosaminoglycan, within the connective tissue
matrix of the valve.
 many cases, degeneration limited to mitral valve
 Marfan syndrome - degeneration is more extensive
and involves other heart valves.

21. Morphology
• Characterized by ballooning
(hooding) of the mitral
leaflets
• affected leaflets are
enlarged, thick, and rubbery
• L A - dilated due to longstanding volume overload.

22. Clinical features
• Most – asymptomatic
• minority - palpitations, dyspnea, or atypical chest
pain
• Auscultation - midsystolic click, caused by abrupt
tension on valve leaflets as valve attempts to close
• diagnosis can be confirmed by echocardiography

23. complications
• 3% - develop complications
(1) IE
(2) MR , sometimes with chordal rupture
(3) stroke or systemic infarct, resulting from embolism
(4) arrhythmias, both ventricular and atrial

24. Complications
• pronounced hooding
of mitral leaflet with
thrombotic plaques

25. Most Common Causes
1. AS: calcification of anatomically normal and
congenitally bicuspid aortic valves
2. AR: dilation of ascending aorta, usually related to
hypertension and aging
3. MS: rheumatic heart disease
4. MR: myxomatous degeneration (mitral valve prolapse)

26. Rheumatic heart disease
1. cardiac manifestation of rheumatic fever.
2. Chronic rheumatic heart disease

27. Acute Rheumatic Fever
• acute, immunologically mediated, multisystem
inflammatory disease 2- to 3-weeks after group A βhemolytic streptococcal infections (pharyngitis)
• Occurs commonly in children (4 to 9 years)

28. PATHOGENESIS
• Heart valves - common antigenic sequences with GAS
bacteria (Mprotein= Glycoprotein antigen)
• GAS pharygitis - Formation of antistreptococcal Abs
• cross reacts with Cardiac myosin and Sarcolemma
• joints (Antibody against Streptococcal hyaluronic acid
cross reacts with connective tissue proteoglycans)
• Only 3% of infected patients develop rheumatic fever
depends on individual immune response

29. Rheumatic Valvular Disease

30. Rheumatic heart disease
• Pathological Changes Of Heart In Acute Rhumatic
Fever

31. Morphology
Aschoff bodies or Rheumatic granuloma
•
fibrinoid necrosis surrounded by lymphocytes,
plasma cells and plump activated macrophages
(Anitschkow cells)
• pathognomonic of rheumatic carditis

32. Anitschkow cells
• modified
macrophages
• nuclei that have
central caterpillarshaped wavy
chromatin

33. Aschoff bodies or Rheumatic granuloma

34. Morphology
Pancarditis:
• Diffuse inflammation and Aschoff Bodies in any of the
3 layers of heart – pericardium, myocardium,
endocardium (including valves)

35. Morphology
Pancrditis
• Pericardium: “Bread and Butter” Pericarditis
• Myocardium: Myocarditis (Scattered Aschoff bodies)
• Endocardium: Fibrinoid necrosis along the lines of
closure of valves forming 1 to 2 mm vegetations
(verrucae)

36. “Bread and Butter”
Pericarditis

37. Macculum plaques
• irregular thickenings of
endocardium in left
atrium caused by
regurgitant blood flow

38. Macculum plaques
Subendocardial fibrosis

39. Vegetations
• vegetations (verrucae)
along the lines of
closure of valves

40. Clinical Features of ARF
• Following upper airway infection with GAS
• Silent period of 2 - 3 weeks
• Sudden onset of fever, pallor, malaise, fatigue
Arthritis - occurs in 75%

41. • two of five major criteria, OR
• one major criterion and two minor criteria

42. Sydenham's chorea
• movement disorder
• described as 'rapid, irregular, aimless and involuntary'.
• affect the muscles in the limbs, face and trunk.
• girls > boys
• 25% - develop chronic rheumatic valve disease.

43. Erythema marginatum
• occurs in < 5% of patients.
• start as red macules that
fade in the centre
• remain red at the edges
• mainly on trunk and
proximal extremities
• but not the face.

44. Investigations

45. Rheumatic heart disease
1. cardiac manifestation of rheumatic fever.
2. Chronic rheumatic heart disease

46. Chronic rheumatic heart disease
• Develope in 50% of rheumatic carditis.
• 2/3 - women.
• history of rheumatic fever or chorea in 50%
• > 90% - mitral valve is affected
• 25% - Isolated mitral stenosis
• 40% - mixed mitral stenosis and regurgitation
• others - aortic valve , tricuspid and pulmonary valve

47. Pathogenesis
• main pathological process - progressive fibrosis.
• characterized by organization of the acute
inflammation and subsequent scarring.
• Aschoff bodies are replaced by fibrous scar
• Fusion of the mitral valve commissures and shortening
of the chordae tendineae  mitral stenosis

48. Morphology
• Fibrous bridging across the valves and calcification create
“fishmouth” or “buttonhole” stenoses

49. Major causes of death in RHD
 Cardiac failure
• Bacterial Endocarditis
• Embolism

50. So…………
 The first step in preventing Rheumatic fever &
Rheumatic heart disease is to detect & treat
STREPTOCOCCAL PHARYNGITIS.

51. Diagnosing a streptococcal pharyngitis

52. you must know why ………………