HEART

1. CVS PATHOLOGY
VALVULAR DISEASES AND DEFORMITIES
SAMOEI – EGERTON UNIVERSITY, MBChB

2. CVS PATHOLOGY - HEART
1. Heart failure
2. Congenital heart diseases
3. Ischemic heart disease
4. Hypertensive heart disease
5. Cor pulmonale
6. Rheumatic fever and rheumatic heart disease
7. Non-rheumatic endocarditis
8. Valvular diseases and deformities
9. Myocardial disease
10. Pericardial disease
11. Tumours of the heart
12. Pathology of cardiovascular interventions

3. CONTENTS
1. Mitral stenosis
2. Mitral insufficiency
3. Aortic stenosis
4. Aortic insufficiency
5. Carcinoid heart disease
6. Myxomatous degeneration of mitral valve (Mitral valve prolapse)

4. INTRODUCTION
 Valvular diseases are various forms of congenital and acquired diseases which cause valvular
deformities.
 Many of them result in cardiac failure.
 Rheumatic heart disease is the most common form of acquired valvular disease.
 Valves of the left side of the heart are involved much more frequently than those of the right
side of the heart.
 The mitral valve is affected most often, followed in descending frequency, by the aortic
valve, and combined mitral and aortic valves.
The valvular deformities may result in: stenosis, insufficiency or both
Stenosis
 Is the failure of a valve to open completely obstructing forward flow of the blood.
Insufficiency or incompetence or regurgitation
 Is the failure of a valve to close completely resulting in back flow of the blood.

5. Etiology of acquired valvular heart diseases
1. RHD, the commonest cause
2. Infective endocarditis
3. Non-bacterial thrombotic endocarditis
4. Libman-Sacks endocarditis
5. Syphilitic valvulitis
6. Calcific aortic valve stenosis
7. Calcification of mitral annulus
8. Myxomatous degeneration (floppy valve syndrome)
9. Carcinoid heart disease.

6. MITRAL VALVE STENOSIS
• Occurs in approximately 40% of all patients with RHD.
• About 70% of the patients are women.
• The latent period between the rheumatic carditis and development of symptomatic
mitral stenosis is about two decades.
ETIOLOGY
 Generally rheumatic in origin.
Less common causes include:
 Bacterial endocarditis
 Libman- Sacks endocarditis
 Endocardial fibroelastosis
 Congenital parachute mitral valve.

7. Cont…
MORPHOLOGIC FEATURES
 Valve leaflets are diffusely thickened
especially towards the closing margin.
 Fibrous adhesions of mitral commissures
 Fusion and shortening of chordae
tendineae.
 Bases of leaflets of mitral valve may be
mobile while free margins have puckered
and thickened tissue with narrowed orifice
-‘purse-string puckering’.
 Rigid, fixed and immobile diaphragm-like
valve leaflets with narrow, slit-like or oval
mitral opening -‘button-hole’or ‘fish-
mouth’mitral orifice
EFFECTS
1. Elevation of LAP from the normal of 12
mmHg to about 25 mmHg
2. Dilatation and hypertrophy of the left
atrium.
3. Normal-sized or atrophic left ventricle
due to reduced inflow of blood.
4. Pulmonary hypertension
5. Chronic passive congestion of the lungs
6. Exertional dyspnea (chief symptom of
mitral stenosis).
7. Hypertrophy and dilatation of the right
ventricle
8. Dilatation of the right atrium when right
heart failure supervenes.

8. MITRAL VALVE INSUFFICIENCY
 Caused by RHD in about 50% of patients
 In contrast to mitral stenosis, pure mitral insufficiency occurs more often in men (75%).
 Mitral insufficiency is associated with some degree of mitral stenosis.
ETIOLOGY
All the causes of mitral stenosis may produce mitral insufficiency
 RHD, most common cause.
 Non-inflammatory calcification of mitral valve annulus (in the elderly)
 Myxomatous transformation of mitral valve (floppy valve syndrome)
 Rupture of a leaflet
 Rapture of chordae tendineae
 Rapture of papillary muscle.
 Myocardial infarction
 Myocarditis
 Left ventricular failure in hypertension.

9. Cont…
MORPHOLOGIC FEATURES
RHD
 Rigidity, deformity and retraction of valve
leaflets
 Fusion of commissures
 Shortening and fusion of chordae tendineae.
Myxomatous degeneration of the mitral valve
leaflets (floppy valve syndrome)
 Prolapse of one or both leaflets into the left
atrium during systole.
In non-inflammatory calcification of mitral
annulus seen in the aged
 Irregular, stony-hard, bead-like thickening in
the region of mitral annulus without any
associated inflammatory changes.
EFFECTS
1. Increase in left ventricular EDV & pressure
2. Dilatation and hypertrophy of the left
ventricle.
3. Rise in LAP and dilatation of left atrium.
4. Pulmonary hypertension
5. Pulmonary edema/congestion
6. Exertional dyspnea and orthopnea
7. Hypertrophy and dilatation of the right
ventricle
8. Dilatation of the right atrium
9. Right heart failure
10. Decreased cardiac output
11. Fatigue and weakness

10. Mitral valve disease

11. AORTIC STENOSIS
• Aortic stenosis comprises about one-fourth of all patients with chronic valvular heart disease.
• About 80% patients of symptomatic aortic stenosis are males.
It is of 2 main types: non-calcific and calcific type (most common).
Non-calcific aortic stenosis
 The most common cause of non-calcific aortic stenosis is chronic RHD.
 Other causes are congenital valvular and subaortic stenosis and congenitally bicuspid aortic
valve.
Calcific aortic stenosis
 Calcific aortic stenosis is more common type.
Causes;
 Healing by scarring followed by calcification of aortic valve such as in RHD, bacterial
endocarditis, Brucella endocarditis, Mönckeberg’s calcific aortic stenosis, healed congenital
malformation and familial hypercholesterolaemic xanthomatosis.

12. Cont…
MORPHOLOGIC FEATURES
 Fibrous thickening of aortic cusps
 Calcific nodularity of the closing edges.
 Calcified nodules are often found in the
sinuses of Valsalva.
 In rheumatic aortic stenosis, the
commissures are fused and calcified
 In non-rheumatic aortic stenosis there is
no commissural fusion
EFFECTS
 Concentric hypertrophy of the left ventricle.
 Cardiac failure
 Dilatation as well as hypertrophy of the left
ventricle (eccentric hypertrophy) following
cardiac failure.
 Exertional dyspnea d/t elevation of pulmonary
capillary pressure.
 Angina pectoris d/t elevation of pulmonary
capillary pressure and usually develops due to
increased demand of hypertrophied
myocardial mass.
 Syncope d/t coronary insufficiency.
 Sudden death
N/B - The 3 cardinal symptoms of aortic stenosis:
 Exertional dyspnea, angina pectoris &
syncope are

13. AORTIC INSUFFICIENCY
• About 75% of all patients with aortic insufficiency are males with some having
family history of Marfan’s syndrome.
ETIOLOGY
 Chronic RHD in about 75% of patients.
 Syphilitic valvulitis
 Infective endocarditis
 Congenital subaortic stenosis (congenitally bicuspid aortic valve)
 Myxomatous degeneration of aortic valve (floppy valve syndrome)
 Traumatic rupture of the valve cusps
 Dissecting aneurysm
 Marfan’s syndrome
 Ankylosing spondylitis.

14. Cont…
MORPHOLOGIC FEATURES
• Aortic valve cusps are thickened,
deformed and shortened and fail to
close.
• Distension and distortion of the ring
EFFECTS
1. Increase in left ventricular EDV.
2. Hypertrophy and dilatation of the left ventricle
3. Left ventricular failure
4. Increase in LAP
5. Pulmonary hypertension
6. Right heart failure
7. Low diastolic pressure
8. High pulse pressure
9. Rapidly rising and collapsing water hammer pulse
(Corrigan’s pulse)
10. Booming ‘pistol shot’ sound over the femoral artery
11. Systolic and diastolic murmur heard over the femoral
artery when it is lightly compressed (Durozier’s sign).
12. Angina pectoris occurs d/t increased myocardial demand
or due to coronary insufficiency.

15. Aortic valve disease.

16. CARCINOID HEART DISEASE
ETIOLOGY
• Carcinoid syndrome developing in patients with extensive hepatic metastases from a
carcinoid tumour is characterised by cardiac manifestations in about half the cases.
• The lesions are characteristically located in the valves and endocardium of the right side of
the heart.
Pathogenesis
 Pathogenesis of the cardiac lesions is not certain.
 But in carcinoid tumour with hepatic metastasis, there is increased blood level of serotonin
secreted by the tumour.
 The increased concentration of serotonin reaches the right side of the heart and causes the
lesions but serotonin is inactivated on passage of the blood through the lungs and hence the
left heart is relatively spared.
 In addition, high levels of bradykinin may play contributory role in carcinoid heart disease.
 However, chronic infusion of serotonin or bradykinin in experimental animals has not
succeeded in producing cardiac lesions; hence the exact pathogenesis of carcinoid heart
disease remains obscure.

17. Cont…
MORPHOLOGIC FEATURES
 Lesions are limited to the right side of the
heart mostly.
 Both pulmonary and tricuspid valves as
well as the endocardium of the right
chambers show characteristic cartilage-
like fibrous plaques.
 Similar plaques may occur on the intima
of the great veins, the coronary sinus and
the great arteries.
 Occasionally, the lesions may be found on
the left side of the heart.
EFFECTS
 Pulmonary stenosis
 Tricuspid regurgitation
To a lesser extent;
 Pulmonary regurgitation
 Tricuspid stenosis.

18. MITRAL VALVE PROLAPSE
• Synonyms; Myxomatous/mucoid degeneration of mitral valve or ‘floppy valve syndrome’
• Occurs in young patients between the age of 20 and 40 years
• More common in women.
• Seen in 5% of general adult population.
ETIOLOGY
• Unknown.
• But in some cases it may be genetically determined collagen disorder.
• Association with Marfan’s syndrome has been observed in 90% of patients.
• Others have noted myxomatous degeneration in cases of Ehlers-Danlos syndrome and in
myotonic dystrophy.
• However, the myxomatous valvular changes seen in the aged patients are not related to this
entity.

19. Cont…
MORPHOLOGIC FEATURES
 Any cardiac valve may be involved but mitral
valve is affected most frequently.
 Most severe and most common in the posterior
leaflet of the mitral valve.
 Affected leaflet is opaque white, soft and
floppy.
 Cut section of the valve reveals mucoid or
myxoid appearance.
 A significant feature is the aneurysmal
protrusion of the affected leaflet and hence the
name ‘mitral valve prolapse’ and ‘floppy valve
syndrome’.
 Microscopically, the enlarged cusp shows
loose connective tissue with abundant mucoid
or myxoid material due to abundance of
mucopolysaccharide.
EFFECTS
• Mid-systolic click followed by a systolic
murmur
Complications;
 Superimposed infective endocarditis
 Mitral insufficiency
 Arrhythmias.
 Rarely, sudden death from serious
ventricular arrhythmias.