VALVULAR HEART DISEASES

8/1/2014
Normal heart valves
Normal heart valves
Valvular heart disease

• A major group of cardiac pathology affecting
cardiac valves.
Clinical consequences
1. Stenosis – failure of a valve to open completely,
obstructing forward flow.
2. Insufficiency (reg...
What is STENOSIS ?
What is REGURGITATION?
Clinical consequences
• Abnormal flow through diseased valves - produces
abnormal heart sounds (murmurs)
• severe lesions ...
Valvular heart disease

Types
1. Congenital valvular heart diseases
2. Acquired valvular heart diseases
Congenital valvular heart diseases
 most common – bicuspid aortic valve
• neither stenotic nor incompetent through early ...
Acquired valvular
heart diseases
Most Common Causes
1. AS: calcification of anatomically normal and
congenitally bicuspid aortic valves
2. AR: dilation of ...
Aortic stenosis

Most common cause –

calcification of
1.

anatomically normal (senile calcific aortic stenosis) and

2.

...
Pathogenesis
• Degenerative changes due to aging process (wear and tear)
• Repetitive mechanical stresses to valves —40 mi...
MORPHOLOGY

• heaped-up calcified masses on outflow side of cusps
Clinical Features
• gradual narrowing of the valve orifice ( 0.5 to 1 cm2 in severe
AS ; normal, ∼4 cm2 )
• Left ventricul...
calcific aortic stenosis
Prognosis

• Asymptomative at earlier stage – excellent
• Late stage - development of angina, CHF...
Most Common Causes
1. AS: calcification of anatomically normal and
congenitally bicuspid aortic valves
2. AR: dilation of ...
Myxomatous Mitral Valve
• Mitral prolapse - parachute-like protrusion of value into the
left atrium

• “floppy” and prolap...
Myxomatous Mitral Valve
Causes

1. primary myxomatous degeneration
• intrinsic defect of connective tissue synthesis or re...
Pathogenesis
 Myxoid degeneration due to accumulation
of glycosaminoglycan, within the connective tissue
matrix of the va...
Morphology

• Characterized by ballooning
(hooding) of the mitral
leaflets
• affected leaflets are

enlarged, thick, and r...
Clinical features
• Most – asymptomatic
• minority - palpitations, dyspnea, or atypical chest
pain
• Auscultation - midsys...
complications
• 3% - develop complications
(1) IE
(2) MR , sometimes with chordal rupture
(3) stroke or systemic infarct, ...
Complications

• pronounced hooding
of mitral leaflet with

thrombotic plaques
Most Common Causes
1. AS: calcification of anatomically normal and
congenitally bicuspid aortic valves
2. AR: dilation of ...
Rheumatic heart disease

1. cardiac manifestation of rheumatic fever.
2. Chronic rheumatic heart disease
Acute Rheumatic Fever
• acute, immunologically mediated, multisystem
inflammatory disease 2- to 3-weeks after group A βhem...
PATHOGENESIS
• Heart valves - common antigenic sequences with GAS
bacteria (Mprotein= Glycoprotein antigen)
• GAS pharygit...
Rheumatic Valvular Disease
Rheumatic heart disease

• Pathological Changes Of Heart In Acute Rhumatic
Fever
Morphology
Aschoff bodies or Rheumatic granuloma
•

fibrinoid necrosis surrounded by lymphocytes,

plasma cells and plump ...
Anitschkow cells
• modified
macrophages
• nuclei that have

central caterpillarshaped wavy
chromatin
Aschoff bodies or Rheumatic granuloma
Morphology

Pancarditis:

• Diffuse inflammation and Aschoff Bodies in any of the
3 layers of heart – pericardium, myocard...
Morphology
Pancrditis
• Pericardium: “Bread and Butter” Pericarditis
• Myocardium: Myocarditis (Scattered Aschoff bodies)
...
“Bread and Butter”
Pericarditis
Macculum plaques

• irregular thickenings of
endocardium in left
atrium caused by

regurgitant blood flow
Macculum plaques

Subendocardial fibrosis
Vegetations

• vegetations (verrucae)
along the lines of
closure of valves
Clinical Features of ARF

• Following upper airway infection with GAS
• Silent period of 2 - 3 weeks

• Sudden onset of fe...
• two of five major criteria, OR
• one major criterion and two minor criteria
Sydenham's chorea

• movement disorder
• described as 'rapid, irregular, aimless and involuntary'.
• affect the muscles in...
Erythema marginatum
• occurs in < 5% of patients.
• start as red macules that
fade in the centre

• remain red at the edge...
Investigations
Rheumatic heart disease

1. cardiac manifestation of rheumatic fever.
2. Chronic rheumatic heart disease
Chronic rheumatic heart disease
• Develope in 50% of rheumatic carditis.

• 2/3 - women.
• history of rheumatic fever or c...
Pathogenesis
• main pathological process - progressive fibrosis.
• characterized by organization of the acute
inflammation...
Morphology

• Fibrous bridging across the valves and calcification create
“fishmouth” or “buttonhole” stenoses
Major causes of death in RHD
 Cardiac failure
• Bacterial Endocarditis

• Embolism
So…………

 The first step in preventing Rheumatic fever &
Rheumatic heart disease is to detect & treat
STREPTOCOCCAL PHARYN...
Diagnosing a streptococcal pharyngitis
you must know why ………………
Rheumatic heart disease
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  • fenfluramine/phentermine
  • Rheumatic heart disease

    1. 1. VALVULAR HEART DISEASES 8/1/2014
    2. 2. Normal heart valves
    3. 3. Normal heart valves
    4. 4. Valvular heart disease • A major group of cardiac pathology affecting cardiac valves.
    5. 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. 6. What is STENOSIS ? What is REGURGITATION?
    7. 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. 8. Valvular heart disease Types 1. Congenital valvular heart diseases 2. Acquired valvular heart diseases
    9. 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. 10. Acquired valvular heart diseases
    11. 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. 12. Aortic stenosis Most common cause – calcification of 1. anatomically normal (senile calcific aortic stenosis) and 2. congenitally bicuspid aortic valves
    13. 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. 14. MORPHOLOGY • heaped-up calcified masses on outflow side of cusps
    15. 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. 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. 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. 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. 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. 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. 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. 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. 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. 24. Complications • pronounced hooding of mitral leaflet with thrombotic plaques
    25. 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. 26. Rheumatic heart disease 1. cardiac manifestation of rheumatic fever. 2. Chronic rheumatic heart disease
    27. 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. 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. 29. Rheumatic Valvular Disease
    30. 30. Rheumatic heart disease • Pathological Changes Of Heart In Acute Rhumatic Fever
    31. 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. 32. Anitschkow cells • modified macrophages • nuclei that have central caterpillarshaped wavy chromatin
    33. 33. Aschoff bodies or Rheumatic granuloma
    34. 34. Morphology Pancarditis: • Diffuse inflammation and Aschoff Bodies in any of the 3 layers of heart – pericardium, myocardium, endocardium (including valves)
    35. 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. 36. “Bread and Butter” Pericarditis
    37. 37. Macculum plaques • irregular thickenings of endocardium in left atrium caused by regurgitant blood flow
    38. 38. Macculum plaques Subendocardial fibrosis
    39. 39. Vegetations • vegetations (verrucae) along the lines of closure of valves
    40. 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. 41. • two of five major criteria, OR • one major criterion and two minor criteria
    42. 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. 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. 44. Investigations
    45. 45. Rheumatic heart disease 1. cardiac manifestation of rheumatic fever. 2. Chronic rheumatic heart disease
    46. 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. 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. 48. Morphology • Fibrous bridging across the valves and calcification create “fishmouth” or “buttonhole” stenoses
    49. 49. Major causes of death in RHD  Cardiac failure • Bacterial Endocarditis • Embolism
    50. 50. So…………  The first step in preventing Rheumatic fever & Rheumatic heart disease is to detect & treat STREPTOCOCCAL PHARYNGITIS.
    51. 51. Diagnosing a streptococcal pharyngitis
    52. 52. you must know why ………………
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