Histopathology - Heart Pathology 3

Front 1

Valvular disease can come to clinical attention due to?

Back 1

1. Stenosis - which is the failure of a valve to open completely, which impedes forward flow.

2. Insufficiency -(regurgitation or incompetence) results from failure of a valve to close completely, thereby allowing reversed flow. These abnormalities can be present 1. alone or coexist, and

2. may involve only a single valve (isolated disease) or more than one valve (combined disease)

Front 2

Generally, valvular stenosis leads to .....a...... of the heart,

whereas valvular insufficiency leads to .....b.... of the heart

Back 2

valvular stenosis leads to pressure overload of the heart, whereas valvular insufficiency leads to volume overload of the heart

Front 3

U

Back 3

B

Front 4

Classify Valvular heart disease

Back 4

CLA SSIFICATION OF VALVULAR ABNORMALITIES can be

1. congenital or acquired

2. CAUSES OF CONGENITAL VALVULAR ABNORMALITIES : Congenital heart diseases ACQUIRED VALVULAR ABNORMALITIES WITH THEIR CAUSES mitral stenosis aortic :postinflammatory scarring (rheumatic heart disease) mitral regurgitation : postinflammatory scarring ,infective endocarditis , mitral valve prolapse. aortic stenosis :postinflammatory scarring (rheumatic heart disease) senile calcific aortic stenosis calcification of congenitally deformed valve aortic regurgitation : postinflammatory scarring (rheumatic heart disease) infective endocarditis infective endocarditis, marfan’s syndrome. Abnormalities of Tensor Apparatus : Rupture of papillary muscle , Papillary muscle dysfunction (fibrosis),Rupture of chordae tendineae. Other Aortic Disease : Degenerative aortic dilation ,Syphilitic aortitis , Rheumatoid arthritis. Abnormalities of Left Ventricular Cavity and/or Annulus : LV enlargement (myocarditis, dilated cardiomyopathy) Calcification of mitral ring

Front 5

Cause of congenital Valvular heart disease

Back 5

1. Congenital heart disease -

a. Bicuspid aortic valve ( has only 2 functional cusps instead of d normal 3

Prone to early and progressive degenerative calcification

Front 6

ACQUIRED VALVULAR ABNORMALITIES WITH THEIR CAUSES

Back 6

ACQUIRED VALVULAR ABNORMALITIES WITH THEIR CAUSES

1.mitral stenosis aortic :

a. post inflammatory scarring (rheumatic heart disease)

2.mitral regurgitation :

a. postinflammatory scarring ,

b. infective endocarditis ,

c. mitral valve prolapse.

3. aortic stenosis :

a. postinflammatory scarring (rheumatic heart disease)

b. senile calcific aortic stenosis calcification of congenitally deformed valve

4. aortic regurgitation :

a. postinflammatory scarring (rheumatic heart disease)

b. infective endocarditis ,

c. marfan’s syndrome.

5. Abnormalities of Tensor Apparatus :

a. Rupture of papillary muscle ,

b. Papillary muscle dysfunction (fibrosis),

c. Rupture of chordae tendineae.

6.Other Aortic Disease :

a. Degenerative aortic dilation ,

b. Syphilitic aortitis ,

c. Rheumatoid arthritis.

7. Abnormalities of Left Ventricular Cavity and/or Annulus :

a. LV enlargement (myocarditis, dilated cardiomyopathy)

b. Calcification of mitral ring

Front 7

What is degerative valve disease

Back 7

Is a term used to describe changes that affect the integrity of Valvular extracellular matrix..

Front 8

Degerative changes includes ;

Back 8

1. Calcifications

2. Decreased number of valve fibroblasts and myofibroblasts

3. Alterations in the ECM

4. Changes in the production of nitric metalloproteinases or their inhibitors

Front 9

....... is the most common cause of aortic stenosis

Back 9

Calcific aortic degeneration

Front 10

Define

1. Rheumatic fever

2. Acute rheumatic cardis

3. Chronic rheumatic heart disease

Back 10

1. Rheumatic fever (RF) is an acute immunologically mediated, multisystem inflammatory disease that occurs a few weeks after an episode of group A streptococcal pharyngitis. It rarely follows infection from the skin.

2. Acute rheumatic carditis is a frequent manifestation during the active phase of RF and may progress over time to Chronic rheumatic heart disease (RHD),

Front 11

Valves and their involvement in chronic rheumatic heart disease

Back 11

1. In chronic disease state (ie chronic RHD), the mitral valve is virtually always involved. The mitral valve is affected alone in 65% to 70% of cases, and along with the aortic valve in another 25% of cases.

Tricuspid valve involvement is infrequent, and the pulmonary valve is only rarely affected.

Front 12

Pathogenesis of rheumatic fever and rheumatic heart disease

Back 12

PATHOGENESIS:

Acute Rheumatoid fever typically appears 10 days to 6 weeks after an episode of pharyngitis..Acute rheumatic fever results from immune responses to group A streptococci, which happen to cross-react with host tissues. Antibodies directed against the M proteins of streptococci have been shown to cross-react with self antigens in the heart. In addition, CD4+ T cells specific for streptococcal peptides also react with self proteins in the heart, and produce cytokines that activate macrophages (such as those found in Aschoff bodies). Damage to heart tissue may thus be caused by a combination of antibody- and T cell–mediated reactions. RHD is characterized principally by deforming fibrotic valvular disease, particularly mitral stenosis, of which it is virtually the only cause..

Front 13

RHD is characterized principally by ?

Back 13

RHD is characterized principally by deforming fibrotic valvular disease, particularly mitral stenosis, of which it is virtually the only cause..

Front 14

Rf and rheumatic heart disease

1. Acute?

2.

Back 14

ACUTE RF-

1. Aschoff bodies consist of plasma cells, and plump activated macrophages called Anitschkow or caterpillar cells (pathognomonic for RF)

2. pericarditis, myocarditis, or endocarditis (pancarditis)

3. Overlying these necrotic foci are small (1- to 2-mm) vegetations, called verrucae

CHRONIC RHD Thickening of chordae tendineae

Valvular effects :leaflet thickening, commissural fusion and shortening, and thickening and fusion of the tendinous cords Fibrous bridging across the valvular commissures and calcification create “fish mouth” or “buttonhole” stenoses

Front 15

What are anitachkow or caterpillar celebrate

Back 15

Front 16

Clinical features of Rheumatic fever and rheumatic heart disease

Back 16

CLINICAL FEATURES:

Acute RF Fever, pericardial friction rubs, weak heart sounds, tachycardia, features of myocarditis. Painful and swollen joints . Migratory polyarthritis of the large joints, pancarditis, subcutaneous nodules, erythema marginatum of the skin, and Sydenham chorea.

Chronic RHD is characterized by Various cardiac murmurs, features of heart failure, arrhythmias (particularly atrial fibrillation in the setting of mitral stenosis)

Front 17

Complications of

Back 17

Acute RF: Chronic RHD Arrhythmias Myocarditis may lead to heart failure

Chronic RHD- Cardiac hypertrophy and dilation Arrhythmias Heart failure Thromboembolic complications Infective endocarditis

Front 18

Define infective endocarditis

Back 18

INFECTIVE ENDOCARDITIS : Is an infection characterized by colonization or invasion of the heart valves or the mural endocardium by a microbe.

This leads to the formation of vegetations composed of thrombotic debris and organisms, often associated with destruction of the underlying cardiac tissues.

Front 19

Clinical features of infective endocarditis

Back 19

1. Fever ,weakness, lassitude, fatigue, loss of weight, and murmurs.

2. Splinter or subungual hemorrhages, Janeway lesions (erythematous or hemorrhagic nontender lesions on the palms or soles)

3. Osler nodes (subcutaneous nodules in the pulp of the digits) and

4. Roth spots(retinal hemorrhages in the eyes)

Front 20

Diagnosis of infective endocarditis

Back 20

1. Requires either Pathologic or Clinical Criteria

PATHOLOGIC CRITERIA: i.Microorganisms demonstration by culture or histologic examination in a vegetation, embolus from a vegetation, or intracardiac abscess

ii.Histologic confirmation of active endocarditis in vegetation or intracardiac abscess

Front 21

Diagnostic Criteria for Infective Endocarditis

Back 21

If Clinical Criteria are used, diagnosis can be made using:

i. 2 major

ii. 1 major + 3 minor OR

iii. 5 minor criteria

Front 22

Four major form of vegetative endocarditis

Back 22

i. Rheumatic heart disease (RHD)

ii. Infective endocarditis (IE)

iii. Noninfected endocarditis: a.Nonbacterial thrombotic endocarditis (NBTE)

b.Libman-Sacks endocarditis (LSE)

Front 23

About NBTE

1. Define

2. Xterised by

3. Seen in?

4. Morphology

Back 23

2. NBTE is characterized by the deposition of small sterile thrombi on the leaflets of the cardiac valves. 3. NBTE is often seen in those with cancer or sepsis , underlying systemic hypercoagulable state(DVT or Pulmonary emboli) , indwelling catheter etc.

4. Histologically they are composed of bland thrombi that are loosely attached to the underlying valve.

Front 24

About libman- sacks disease

1. Define

2. Encountered in

3. Assoc with

4. Morphology

Back 24

1. Endocarditis of SLE

2. It is encountered in SLE occasionally.

3. Morphology: Single or multiple, sterile, pink vegetations, Fibrinous eosinophilic material , some remnants of nuclei damaged by anti-nuclear antigen bodies and fibrinoid necrosis can be seen.

4. There is associated mitral and tricuspid valvulitis.

Front 25

Discuss cardiomyopathy

1. Define

2. It could be?

3. Aetiology

4. Forms

Back 25

1. Heart disease resulting from an abnormality in the myocardium. It usually produces abnormalities in cardiac wall thickness ,chamber size, and mechanical and/or electrical Dysfunction.

2. It could be Primary or Secondary. Primary cardiomyopathies are diseases predominantly confined to the heart muscle, whereas Secondary cardiomyopathies have myocardial involvement as a component of a systemic or multiorgan disorder.

3. Genetic and non genetic

i. GENETIC: eg Mutations in the gene encoding β-myosin heavy chain (β-MHC), etc.

ii. NON- GENETIC:

a. Myocarditis

b. Amyloidosis

c. Toxins: eg alcohol

d. Infiltrative disorders: eg Leukamia

e. Metabolic: eg hyperthyroidis , hyperkalemia

f. Idiopathic, etc.

4. a.Dilated cardiomyopathy (DCM)

b. Hypertrophic cardiomyopathy (HCM)

c. Restrictive cardiomyopathy.

d. Another rare form of cardiomyopathy, left ventricular non-compaction, is characterized by a distinctive “spongy” appearance of the left ventricular myocardium.

Front 26

About dilated, hypertropic, and restrictive cardiomyopathy

1. Definition / xterised by

2. Pathology

3. Morphology

4. Complications

Back 26

A. Dilated cardiomyopathy

1. It is characterized by progressive cardiac dilation and contractile (systolic) dysfunction, usually with concomitant hypertrophy.2. Pathology

2. Pathology - Impairment of contractility(Systolic dysfunction) There is defect in force generation and transmission

3. Morphology -

Grossly: Increase in size, heavy and flabby, due to dilation of all chambers .

Microscopy: Hypertrophy, enlarged nuclei but some are attenuated, stretched, and irregular. Interstitial and endocardial fibrosis.

B. Hypertropic cardiomyopathy

1. Is characterized by myocardial hypertrophy (massive), abnormal diastolic filling, and in some cases, it can also be associated with intermittent ventricular outflow obstruction.

2. Mechanism of action / pathology : Impairment of compliance(diastolic dysfunction) Systolic function is usually preserved and the heart is hypercontracting.

3. GROSS:

i. Massive myocardial hypertrophy, usually without ventricular dilation.

ii. Asymmetric septal hypertrophy : Is disproportionate thickening of the ventricular septum as compared with the free wall of the left ventricle (with a ratio greater than 1 : 3)

iii.Symmetrical hypertrophy MICROSCOPY:

i.Extensive myocyte hypertrophy ii.Haphazard disarray of bundles of myocytes

iii.Interstitial fibrosis

C. Restrictive cardiomyopathy

1. Is a disorder characterized by a primary decrease in ventricular compliance, resulting in impaired ventricular filling during diastole.

2. Pathology / Mechanism of action: Impairment of compliance(diastolic dysfunction) Ventricular filling is impaired because the ventricles are stiff.

3. Gross: The ventricles -normal or slightly enlarged size ; the cavities are not dilated, and Firm and noncompliant myocardium. Atrial dilation and hypertrophy is commonly observed.

Microscopy: Interstitial fibrosis; Amyloidosis can be seen if it is the cause of the cardiomyopathy.

Complications of cardiomyopathy

i. Cardiac fairlure

ii. Sudden death

iii. Atrial filbrillation

iv. Stroke

Front 27

Complications of cardiomyopathy

Back 27

i. Cardiac fairlure

ii. Sudden death

iii. Atrial filbrillation

iv. Stroke

Front 28

Hypertropic cardiomyopathy Vs dilated cardiomyopathy heart

Back 28

.The hypertropic cardiomyopathy heart is thick-walled, heavy,and hypercontracting in striking contrast to the flabby, hypocontracting heart of DCM.

Front 29

...... is one of the most common causes of sudden, otherwise unexplained death in young athletes

Back 29

HCM hypertropic cardiomyopathy is one of the most common causes of sudden, otherwise unexplained death in young athletes