CARDIOVASCULAR PATHOLOGY

(Blood Vessels & Heart)

By:
BRIG TARIQ SARFRAZ
MBBS, MCPS, FCPS, FRCPath (UK)
FELLOW ROYAL COLLEGE OF PATHOLOGISTS, LONDON (UK)
PROFESSOR OF PATHOLOGY
ARMY MEDICAL COLLEGE, RAWALPINDI
 SEQUENCE
• DISEASES OF BLOOD VESSELS
- Vasculitis
- Aneurysms
- Tumours of Blood vessels
 SEQUENCE
• DISEASES OF HEART
- Arteriosclerosis / Atherosclerosis
- Ischaemic Heart Disease
- Rheumatic Heart Disease
- Diseases of Endocardium,
Myocardium & Pericardium
- Tumours of Heart
 ARTERIOSCLEROSIS
(ATHEROSCLEROSIS)
AND
ISCHAEMIC HEART DISEASE
 Arteriosclerosis

Arteriosclerosis
Hardening

Atherosclerosis MONCKEBERG Medial Calcific Arteriolosclerosis

Large BV Sclerosis Small vessel
Intima Media Full thickness

Hyaline Hyperplastic
 ATHEROSCLEROSIS
• “It is a disease of elastic and muscular
arteries characterized by intimal lesions
called atheromas or fibrofatty plaques that
protrude into the lumen, weaken the
underlying media, and undergo a series of
complications”.
 HISTORICAL
BACKGROUND
* AS has afflicted man since ancient times.
* Identified in Egyptian and Grecian
mummies (1500) BC
* Recognized by physicians at autopsy in
16th century
* Defined and described in 19th century
EPIDEMIOLOGY
INCIDENCE
HIGHEST : North America, Europe,
Australia, New Zealand &
Russia
LOW : Japan, China, Africa
Central and South
America & Asia
 Common Sites:

• Large BV : Aorta,
Carotid & Iliac.
(large vessels)
• Medium BV :
Coronary, Cerebral,
Limbs.
• Small BV & Veins:
Never affected.
 Risk Factors for Atherosclerosis*

MAJOR
Non-modifiable Potentially
Controllable
Increasing age Hyperlipidemia
Male gender Hypertension
Family history Cigarette smoking
Genetic abnormalities Diabetes Mellitus
 Risk Factors for Atherosclerosis
MINOR / LESSER / UNCERTAIN

Obesity Alcohol
Physical inactivity Lipoprotein (a)
Stress (type A Hardened (trans)
personality) unsaturated fat
intake
Postmenopausal Chlamydia
estrogen deficiency, pneumoniae
High carbohydrate
intake
RISK FACTORS
AGE
* Progressively rises with age
* Peak between 40-60 years in males and 6-
7th decade females
* Out of all deaths due to AS/MI, 98%
between the ages of 35-64 years
RISK FACTORS
HYPERTENSION
* High BP accelerates AS
* After age 45 years, strong risk factor than
hypercholesterolemia
* BP exceeding 169/95, 5 times higher risk of
IHD
* Both systolic and diastolic pressure
important
 RISK FACTORS

DIABETES MELLITUS

* Induces hypercholesterolemia
* Two - fold increase of MI in DM
* 100 - fold increased risk of AS - induced
gangrene of lower extremities
 RISK FACTORS
CIGARETTE SMOKING
* Definite association with AS and MI
* Degree of AS in coronary vessels higher
in smokers
* One or more packs per day for several
years death rate increases by 200%
 RISK FACTORS
HYPERLIPIDEMIA
 Overwhelming evidence
 Higher level of cholesterol,
higher risk
 Direct association with high
LDL cholesterol level
 Inverse relationship with
HDL level
PATHOGENESIS: Old theories

INSUDATION / IMBIBITION THEORY

* Propounded by Virchow (1858)
* Low grade inflammation
* Increased infiltration of plasma
proteins and lipid into intima from
blood
PATHOGENESIS: Old theories
ENCRUSTATION / THROMBOGENIC
THEORY
* Proposed by Rokitansky
* Endothelial injury
* Formation of thrombi, composed of fibrin,
platelets, plasma proteins and leukocytes
* Incorporation of thrombi into intima
* Organization of thrombi
RESPONSE TO INJURY HYPOTHESIS (Current
Concept)
 IN PATHOGENESIS ROLE
OF:
• Endothelial cells
• Smooth muscle
• Macrophages
• Lipoproteins
• Platelets
ENDOTHELIAL CELLS
* Perform multifarious functions
* Some alterations in these cells and injury
leads to AS
* Endothelial alterations due to hemodynamic
disturbances and hypercholesterolemia
* Increased permeability, leukocyte adhesion,
expression of adhesion molecules
ARTERIAL SMOOTH MUSCLE
• Can synthesize CT matrix proteins,
collagen, elastic tissue and proteoglycans
• Can take up and degrade some lipids
• Undergo migration & proliferation due to
growth factors like PDGF, FGF, TGF- α
PLATELETS

* Adhere to site of endothelial injury

* Release of PDGF which is mitogenic to
smooth muscle
LIPOPROTEINS

* Play role in atherogenesis

* High level of LDL leads to increased rate
of penetration into arterial wall
* Promote formation of foam cells
* May affect endothelial cells by super oxide
and other oxygen free radicals.
* Oxidized lipoproteins
 MACROPHAGES
• ENGULFMENT OF LIPOPROTEINS
• OXIDATION OF LDL
• FOAM CELLS
• RELEASE OF CERTAIN PRODUCTS
IL-1,TNF, FGF etc.
MORPHOLOGY OF ATHEROSCLEROSIS
Major Components of Atheromatous Plaque
Neovascularization at periphery

FIBROUS CAP
(smooth muscle cells, macrophages,
foam cells, lymphocytes, collagen,
elastin, proteoglycans,
neovasularization)
NECROTIC CENTER
(cell debris, cholesterol crystals, foam
cells, calcium)
MEDIA
Natural History of Atherosclerosis
 Development of
Coronary Atherosclerosis:
 ATHEROSCLEROSIS

* Multifactorial
* Multifaceted
* Exact pathogenesis unknown
 PATHOGENESIS
NEW CONCEPTS
 Intimal Cell Mass and Neointima Formation
Hypothesis
 Hemodynamic Hypothesis
 Monoclonal (Mutagenic) Theory
ATHEROMA
ISCHAEMIC HEART DISEASE
ISCHAEMIC HEART DISEASE (IHD)

MANIFESTATIONS
MYOCARDIAL INFARCTION
ANGINA PECTORIS
CHRONIC ISCHAEMIC HEART DISEASE
SUDDEN CARDIAC DEATH
 PATHOGENESIS - IHD

• ROLE OF FIXED
CORONARY
OBSTRUCTION

• ROLE OF ACUTE PLAQUE

CHAHGE
 HAEMORRHAGE
 RURTURE
 ULCERATION

• ROLE OF CORONARY
THROMBOSIS

• ROLE OF
VASOCONSTRICTION
 ANGINA PECTORIS

• TYPES
STABLE / TYPICAL
PRINZMETAL / VARIANT
UNSTABLE / CRESCENDO
 MYOCARDIAL INFARCTION

TYPES
TRANSMURAL
SUBENDOCARDIAL
 Differences Between Subendocardial and
Transmural Infarcts
Subendocardial Infarcts Transmural Infarcts
Multifocal, Patchy Unifocal ,Solid
Inner 1/3 or 1/2 Full or nearly full thickness
Circumferential In distribution of a specific
coronary artery
Acute Plaque Change rare Acute Plaque Change
common
Coronary thrombosis rare Coronary thrombosis
common
Often results from Often causes shock
hypotension or shock
No epicarditis Epicarditis common
Do not form aneurysms May result in aneurysms
 RISK FACTORS - MI
• HYPERLIPIDEMIA
• CIGARETTE SMOKING
• DM
• FAMILY HISTORY
• OBESITY
• PHYSICAL INACTIVITY
• STRESS
• LIPOPROTEIN (a)
 Development of
Coronary Atherosclerosis:
 PATHOGENESIS-MI

• CORONARY ARTERY OCCLUSION

WITH CORONARY ATHEROTHROMBOSIS
PLAQUE DISRUPTION
PLATELET ADHESION, AGGREGATION
ACTIVATION OF EXTRINSIC SYSTEM
WITHOUT CORONARY ATHEROTHROMBOSIS
VASOSPASM – PLATELETS
EMBOLI
UNEXPLAINED
Atheroma with Thrombosis
 PATHOGENESIS – MI (Contd..)
• MYOCARDIAL RESPONSE
FUNCTIONAL
BIOCHEMICAL
MORPHOLOGICAL
 PATHOGENESIS – MI (Contd..)
• FUNCTIONAL CONSEQUENCES
MYOFIBRILLAR RELAXATION
LOSS OF CONTRACTILITY
ARRYHTHMIA
• BIOCHEMICAL CONSEQUENCES
ANAEROBIC GLYCOLYSIS
ATP DEPLETION
LACTIC ACID
LOSS OF GLYCOGEN
Progression of Myocardial Necrosis

ATION
 FACTORS FOR MYOCARDIAL DAMAGE - MI

• LOCATION, SEVERITY AND RATE OF

DEVELOPMENT OF OBSTRUCTION
• DURATION OF OCCLUSION
• METABOLIC/OXYGEN NEEDS OF
MYOCARDIUM
• COLLATERAL CHANNEL CIRCULATION
• CORONARY SPASM
• BLOOD PRESSURE
• HEART RATE AND RHYTHM
CORNARY ARTERIES AND RESPECTIVE AREAS OF INFARCT
Angiogram - Embolism Infarction
APPEARANCES OF MYOCARDIAL INFARCTION
APPEARANCES OF MYOCARDIAL INFARCTION
 CLINICAL FEATURES-MI
• PAIN CHEST, COLD SWEATS
• DYSPNOEA
• SYNCOPE/COLLAPSE
• SHOCK
• ECG – Q WAVE / NON - Q WAVE
• CARDIAC ENZYMES
CK (RISES WITHIN 2- 4 HRS, PEAK 24 HRS AND
BECOMES NORMAL 72 HRS)
 CLINICAL FEATURES-MI (Contd..)

• Cardiac Enzymes
• CK, AST, LDH
• Troponin-T (Trop-T)
• Troponin-I (Trop-I)
• Echocardiography
• Angiography
• CT Angiography
• SCANNING
• MRI
 COMPLICATIONS / CONSEQUENCES - MI

• CONTRACTILE
DYSFUNCTION
• ARRHYTHMIAS
• MYOCARDIAL RUPTURE
• PERICARDITIS
• RV. INFARCT
• INFARCT EXTENSION
• MURAL THROMBUS
• VENT. ANEURYSM
• HEART FAILURE
Coronary Angioplasty
SUDDEN CARDIAC DEATH
• CARDIAC DEATH WITHIN 1 HOUR
• CAUSES:
– CONGENTIAL STRUCTURAL OR
CORONARY ARTERIAL ABNORMALITIES
– AORTIC VALVE STENOSIS
– MITRAL VALVE PROLAPSE
– MYOCARDITIS
– DILATED OR HYPERTROPHIC
CARDIOMYOPATHY
– PULMONARY HYPERTENSION
– CONDUCTING SYSTEM DEFECTS
HYPERTENSIVE HEART DISEASE (HHD)

• SYSTEMIC (LEFT –SIDED)

HYPERTENSIVE HEART DISEASE
– LEFT VENT HYPERTROPHY
– HISTORY OR PATH EVIDENCE OF
HYPERTENSION
– INCREASED LEFT VENT THICKNESS (>2
CM)
– INCREASED WEIGHT 500 G OR MORE
 SYSTEMIC (LEFT –SIDED)
HYPERTENSIVE HEART DISEASE
PULMONARY HYPERTENSIVE HEART DISEASE
(COR PULMONALE)

• It is characterized by right ventricular

hypertrophy, dilation, and potentially
failure secondary to pulmonary
hypertension caused by disorders of
the lungs or pulmonary vasculature”
DISORDERS PREDISPOSING TO COR PULMONALE
 PULMONARY HYPERTENSIVE HEART
DISEASE (COR PULMONALE)

• ACUTE COR PULMONALE

• CHRONIC COR PULMONALE
 INCIDENCE OF MYOCARDIAL
INFARCTION (MI)
• 1-5 MILLIONS/YEAR USA
• 0-5 MILLIONS DIE ANNUALLY
• MORE IN MALES
• FALL IN DEATH RATE
PREVENTION
THERAPEUTIC ADVANCES
 APPROXIMATE TIME OF ONSET OF KEY
EVENTS IN ISCHEMIC CARDIAC MYOCYTES

FEATURE TIME
ONSET OF ATP DEPLITION SECONDS
LOSS OF CONTRACTILITY < 2 MIN
ATP REDUCED
TO 50% OF NORMAL 10 MIN
TO 10 % OF NORMAL 40 MIN
IRREVERSIBLE CELL INJURY 20-40 MIN
MICROVASCULAR INJURY > 1 HR
RHEUMATIC HEART DISEASE
 RHEUMATIC FEVER & RHEUMATIC
HEART DISEASE (RHD)

• DEFINITION
“ACUTE IMMUNOLOGICALLY MEDIATED
MULTISYSTEM INFLAMMATORY DISEASE
THAT OCCURS A FEW WEEKS AFTER AN
EPISODE OF GROUP- A, ß – HAEMOLYTIC
STREPTOCOCCAL PHARYNGITIS AND
OFTEN INVOLVES HEART”
 PATHOLOGY OF RHEUMATIC
HEART DISEASE

AETIOLOGY
1. INFECTIVE ELEMENT
2. PERSONAL SUSCEPTIBILITY
3. SOCIAL DISTRIBUTION
 MAJOR MANIFESTATIONS
• MIGRATORY POLYARTHRITIS-LARGE
JOINTS
• CARDITIS
• SUBCUTANEOUS NODDULES
• ERYTHEMA MARGINATUM – SKIN
• SYDENHAM’S CHOREA
 MINOR MANIFESTATIONS
• FEVER
• ARTHRALGIA
• INCREASED ESR
• LEUKOCYTOSIS
• C – REACTIVE PROTEIN
• ECG CHANGES: PROLONGED P- R
INTERVAL
 JONE’S CRITERIA FOR
DIAGNOSIS OF RF
DIAGNOSIS
• REQUIRES TWO MAJOR FEATURES
OR
• ONE MAJOR & TWO MINOR FEATURES +
RAISED ANTI-STREPTOCOCCAL
ANTIBODY LEVELS (ASO Titers) or DNAse
OR
POSITIVE THROAT CULTURE FOR GROUP
A, ß-HEMOLYTIC STREPTOCOCCUS
 PATHOGENESIS
• EXACT ETIOLOGY- UNKNOWN
• IMMUNE REACTION
• HYPERSENSITIVITY REACTION TO GROUP
A, ß-HEMOLYTIC STREPTOCOCCUS
• ABSENCE OF STREPTOCOCCI –LESIONS
• ANTIBODIES AGAINST M-PROTEINS
(Molecular Mimicry)
• CROSS REACTION WITH TISSUE
GLYCOPROTEINS
• AUTOIMMUNITY
• GENETIC SUSCEPTIBILITY
P A T H O G E N E S I S OF R H E U M A T I C H E A R T D I S E A S E
 PATHOLOGY OF RHEUMATTIC
HEART DIEASE
ACUTE R.H.D – HEART (Pancarditis)

1. PERICARDIUM
2. MYOCARDIUM
3. ENDOCARDIUM
 MORPHOLOGY
ACUTE RHEUMATIC FEVER

• MYOCARDITIS
– ASCHOFF BODIES – FIBRINOID
NECROSIS, T-LYMPHOCYTES, PLASMA
CELLS, Plump-activated-MACROPHAGES
AND GIANT CELLS.
– ANTISCHKOW CELLS (CATERPILLAR)
ASCHOFF GIANT CELLS-plump-activated-
macrophages
• PERICARDITIS – BREAD AND BUTTER
 MORPHOLOGY (Contd..)

• ENDOCARDITIS
SMALL VEGETATIONS
ALONG LINE OF CLOSURE
– VALVES VERRUCAE
• LEFT ATRIUM
• Mac CALLUM PLACQUES
 RHEUMATIC HEART DISEASE
(RHD)
• ACTIVE LESION
a) EXUDATIVE LESION
i. COLLAGEN DEGENERATION
ii. OEDEMA
iii. CELLULAR INFILTRATE
b) ASCHOFF BODY

• HEALED LESION
a) FIBROSIS
b) MYXOMATOUS CHANGES
c) CALCIFICATION
 MORPHOLOGY-CHRONIC RHD
• SOLITARY MITRAL VALVE (65-70%)
– LEAFLETS THICKENING,
COMMISSURAL FUSIONS AND
SHORTENING, THICKENING AND
FUSION OF TENDINOUS CORDS
(FISH-MOUTH APPEARANCE)
• AORTIC/MITRAL VALVE (20-25%)
• TRICUSPID & PULM. VALVE (RARE)
• DILATATION OF LEFT ATRIUM
• MURAL THROMBUS
 CLINICAL FEATURES
• 10-42 DAYS AFTER PHARYNGITIS
• MOSTLY CHILDREN (5-15 YEARS)
• MIDDLE AGED 20%
• MIGRATORY POLYARTHRITIS
• TACHYCARDIA, ARRHYTHMIA
• PERICARDIAL RUB
• RAISED ASOT
 EFFECTS / COMPLICATIONS
• VALVULAR STENOSIS / DEFORMITY
• LEFT ATRIAL DILATATION / HYPERTROPHY
• ATRIAL FIBRILLATION
• MURAL THROMBUS – EMBOLISM
• CHRONIC CONGESTION OF LUNG
• RIGHT VENTRICULAR HYPERTROPHY & CHF
• INCREASED RISK OF IE
• ADHESIVE PERICARDITIS
CAUSES OF DEATH IN RHEUMATIC
HEART DISEASE

1. CARDIAC FAILURE
2. BACTERIAL ENDOCARDITIS
3. EMBOLISM
DISEASES OF ENDOCARDIUM
AND MYOCARDIUM
VEGETATIVE ENDOCARDITIS
• INFECTIVE ENDOCARDITIS
• NON INFECTIVE ENDOCARDITIS
1. NON BACTERIAL THROMBOTIC
ENDOCARDITIS
2. ENDOCARDITIS ASSOCIATED WITH
SYSTEMIC LUPUS ERYTHEMATOSUS
 INFECTIVE ENDOCARDITIS (IE)
• DEFINITION
“ Characterized by colonization or invasion of
the heart valves, the mural endocardium or
other cardiovascular sites by a microbiologic
agent, leading to the formation of bulky,
friable vegetations composed of thrombotic
debris and organisms, often associated with
destruction of the underlying cardiac
tissues.”
 INFECTIVE ENDOCARDITIS (IE) (Contd)

• TYPES
ACUTE INFECTIVE ENDOCARDITIS
HIGHLY VIRULENT ORGANISMS(S.
aureus), NORMAL HEART
SUBACUTE INFECTIVE ENDOCARDITIS
LOW VIRULENT ORGANISMS (S. viridans),
ABNORMAL HEART
CAUSES AND PATHOGENESIS -IE
PREDISPOSING FACTORS
• RHEUMATIC HEART DISEASE
• CONGENITAL HEART DISEASES
• MYXOMATOUS MITRAL VALVE
• DEGENERATIVE CALCIFIC
VALVULAR
STENOSIS
• BICUSPID AORTIC VALVE
• PROSTHETIC VALVE
• VASCULAR GRAFTS
 OTHER RISK FACTORS-IE
• NEUTROPENIA
• IMMUNODEFICIENCY
• DIABETES MELLITUS
• ALCOHOL
• INTRARENOUS DRUG ABUSE
• INDWELLING VASCULAR CATHETERS
 ACUTE VERSUS SUBACUTE ENDOCARDITIS
ACUTE ENDOCARDITIS SUBACUTE ENDOCARDITIS

Stormy onset Insidious appearance

Normal Heart
Highly Virulent Organism
Rapid Course (days-weeks)
Necrotizing, ulcerative, invasive Less destructive-MORE-COMMON
lesions
No evidense of healing
Fever with chills
Staphylococcus Aureus
Abnormal Heart
Low Virulent Organism
Protracted Course (weeks-
months)
Often signs of healing
Non specific constitutional
symptoms
Streptococcus Viridans
 CAUSATIVE ORGANISMS-IE
• STREPTOCOCCUS VIRIDANS (alpha-
HAEMOLYTICUS) - 50 to 60 %
• STAPHYLOCOCCUS AUREUS -10 to 20 %
• STAPHYLOCOCCUS EPIDERMIDUS –
PROSTHETIC VALVE
• HAEMOPHILUS INFLUNENZAE,
ACTINOBACILLUS, CARDIOBACTERIUM,
EIKENELLA, KINGELLA - (HACEK)
• FUNGI
• CHLAMYDIA
• RICKETTSIA
 SOURCE OF INFECTION-IE

• DENTAL/SURGICAL PROCEDURES
• CONTAMINATED INJECTIONS
• OCCULT SOURCE: GUT, ORAL CAVITY,
TRIVIAL INJURIES & INFECTED SITES
CULTURE NEGATIVE ENDOCARDITIS
(10%)

• PRIOR ANTIBIOTICS THERAPY

• IMPROPER TIMINGS – BLOOD
SAMPLING
• HACEK
• DIFFICULTIES IN ISOLATION
• DEEPLY EMBEDDED ORGANISMS
 MOROPHOLOGY -IE
• ACUTE IE - BULKY,
FRIABLE,
IRREGULAR
VEGETATIONS ON
VALVE CUSPS AND
CORDS
• SUBACUTE IE -
SMALLER
VEGETATIONS
 MOROPHOLOGY –IE (Contd..)
• NONVALVULAR IE -
VEGETATIONS
DOWNSTREAM
• WITH PROSTHETIC
VALVES - RING
ABSCESSES
• WITH I/V DRUG
ABUSE – RIGHT
SIDED VALVE
 CLINICAL FEATURES-IE
• FEVER, CHILLS
• FATIGUE
• LOSS OF WEIGHT
• MURMURS(90%)
• PETECHIAE
• SUBUNGUAL HAEMORRHAGES
• ROTH SPOTS-EYES
 COMPLICATIONS-IE
• CARDIAC COMPLICATIONS

 VAVULAR INSUFFICIENCY /
STENOSIS

 MYOCARDIAL RING ABSCESS

 PERFORATION-AORTA &
INTERVENTRICULAR SEPTUM

 SUPPURATIVE PERICARDITIS

 DEHISCENCE WITH
PARAVALVULAR LEAKS
EMBOLIC COMPLICATIONS - IE

• LEFT SIDED LESIONS

CEREBRAL INFARCT, BRAIN
ABSCESS, MENINGITIS, MI,
SPLENIC ABSCESS & RENAL
ABSCESS
• RIGHT SIDED LESIONS
PULMONARY INFARCT, LUNG
ABSCESS AND PNEUMONIA
 RENAL COMPLICATIONS -IE

• RENAL INFARCT
• FOCAL AND DIFFUSE
GLOMERULONEPHRITIS
• MULTIPLE RENAL ABSCESSES
 NONINFECTED VEGETATIONS
• Nonbacterial Thrombotic Endocarditis

• Endocarditis of Systemic Lupus Erythematosus

(Libman – Sacks Endocarditis)

RHD IE NBTE SLE

 MAJOR CAUSES OF MYOCARDITIS
INFECTIONS
Viruses (e.g. coxsackievirus, ECHO, influenza, HIV
cytomegalovirus)
Chlamydia (e.g. C, psittaci)
Rickettsiae (e.g. R, typhi, typhus fever)
Bacteria e.g. Corynebacterium diphtheriae,
Neisseria meningococcus, Borrelia (Lyme disease)
Fungi (e.g. Candida)
Protozoa (e.g. Trypanosoma, Chagas disease,
disease, toxoplasmosis)
Helminths (e.g. trichinosis)
 MAJOR CAUSES OF MYOCARDITIS (CONTD)

Immune-Mediated Reactions
Postviral
Poststreptococcal (rheumatic fever)
Systemic lupus erythematosus
Drug hypersensitivity (e.g. methyldopa,
sulfonamides)
Transplant rejection
Unknown
Sarcoidosis
Giant cell mycoarditis
LYMPHOCYTIC MYOCARDITIS
HYPERSENSITIVITY MYOCARDITIS
GIANT CELL MYOCARDITIS
MYOCARDITIS OF CHAGAS DISEASE
CLINICAL FEATURES - MYOCARDITIS

• Broad clinical spectrum

• Asymptomatic to CHF or sudden
death
• Fever, dyspnoea, fatigue, palpitation,
chest pain
• May mimic MI
• May proceed to DCM
DISEASES OF PERICARDIUM
 PERICARDITIS & EFFUSION
• Normal fluid 30-50 ml
• Blood, Pus, Straw coloured fluid
• Slow accumulation (<500 ml) - Globular
Heart
• Rapid collection - tamponade
CAUSES OF PERICARDITIS
 MORPHOLOGICAL CLASSIFICATION

• Serous pericarditis
• Serofibrinous / fibrinous pericarditis
• Purulent pericarditis
• Haemorrhagic pericarditis
• Caseous pericarditis
• Adehesive mediastinopericarditis
• Constrictive pericarditis
HAEMORRHAGIC PERICARDITIS
• CAUSES
– T.B
– Malignancy
– Trauma
– Cardiac surgery
– Bleeding diathesis
– Anti - coagulant
therapy
 CASEOUS PERICARDITIS
• Tuberculosis
• Fungi
• Results in calcific, fibrocalcific
pericarditis
 FIBRINOUS & SEROFIBRINOUS
PERICARDITIS
• MI, post infraction
syndrome, uraemia,
radiations, rheumatic
fever, SLE, trauma
• Friction rub
• Yellow cloudy exudate
• Dry rough, granular
surface
• Bread and Butter
appearance
 PURULENT PERICARDITIS
• Source of infection
– Direct spread
– seeding from blood &
lymphatics
– introduction during surgery
• Thin to creamy pus (400-
500 ml)
• May lead to constrictive or
mediastino- pericarditis
 CONSTRICTIVE PERICARDITIS

• Fibrous / Fibrocalcific
case

• Concretio cordis

• Limits diastolic function

• Low cardiac output

• Pericardiotomy
 ADHESIVE MEDIASTINAL
PERICARDITIS
• Suppurative, caseous, irradiation,
cardiac surgery
• Obliterated pericardial sac
• Strain on cardiac function
• Retraction of ribs, diaphragm & pulsus
paradoxus
• Leads to cardiac hypertrophy or
dilatation
 COMPLICATIONS OF
PERICARDITIS
• Tamponade
• Adhesive pericarditis
• Impairment of cardiac function
• Adhesion with surrounding structures
• Constriction
TUMOURS OF HEART
 TUMOURS OF THE HEART

• PRIMARY ( RARE)
– Myxoma, Rhabdomyoma, Fibroma,
Lipoma, Papillary Fibroelastoma,
Angiosarcoma, Other Sarcomas
• METASTATIC (5% Cancerous patients)
 CARDIAC MYXOMA
• Most common primary tumour
• Atria (90%) - Fossa ovalis
• Mostly single (<1 cm-10 cm)
• Hard to soft gelatinous mass
• Myxoma cells, EC, Smooth
Muscle & undiff. Cells
• Hamartoma / thrombus?
 CARDIAC MYXOMA - (contd..)

• Mobile, wrecking - ball effect

• Ball - valve obstruction
• Embolization
• Constitutional symptoms due to IL-6
• Surgical removal - curative
• Familial cardia myxoma (10%)
 CARDIAC RHABDOMYOMA
• Most common - infants & children
• Obstruction - valve / chamber
• Grayish-white mass
• Spider cells, large rounded cells.
 CARDIAC LIPOMA
• Subendocardium, myocardium or
subpericardium.
• LV, right atrium or atrial septum
• Asymptomatic
• Ball - valve obstruction
• Arrhythmia
 PAPILLARY FIBROELASTOMA

• Incidental finding - autopsy

• Located on valves
• Hair-like projections
• Myxoid connective tissue, elastic fibers
& mucopolysaccharides
CARDIAC EFFECTS OF NON-CARDIAC
TUMOURS
• Melanoma, Leukemia, Lymphoma,
Carcinoma breast, lung, oesophagus &
kidney
• Effects due to infiltration, mediators or
tumour therapy
• Pericardial effusion, tamponade
CARDIOVASCULAR EFFECTS OF
NON-CARDIAC NEOPLASMS
CARDIOMYOPATHIES
 CARDIOMYOPATHY
• “Heart disease resulting from a primary
abnormality in the myocardium”
 HYPERTROPHIC
CARDIOMYOPATHY (HCM)

• “It is characterized by myocardial

hypertrophy, abnormal diastolic filling and
in about 1/3 cases intermittent left
ventricular outflow obstruction”.
MORPHOLOGY – HCM
PATHOGENESIS – HCM
 CLINICAL FEATURES
• Reduced chamber size and stroke
volume
• Impaired diastolic filling
• Exertional dyspnoea
• Anginal pain
• Atrial fibrillation
• Ventricular arrhythmia
• Sudden death
DILATED CARDIOMYOPATHY (DCM)

“A form of cardiomyopathy
characterized by
progressive cardiac
hypertrophy, dilation and
contractile dysfunction.”
 CAUSES OF DCM
• Myocarditis – coxsakie virus B &
Enterovirus
• Alcohol abuse
• Chemotherapeutic agents – doxorubin
• Pregnancy associated
• Genetic influence
• Idiopathic
MORPHOLOGY OF DCM
 CLINICAL FEATURES – DCM
• Commonly affects 20 to 60 years old
• Slowly progressive CHF
• Less ejection fraction
• Death due to arrhythmia, cardiac failure
• Embolization
DISEASES OF BLOOD VESSELS
 VASCULITIS
DEFINITION
“Inflammation of the wall of any type
of vessel in any organ leading to
wide spectrum of clinical
manifestations”
TYPES
 Generalized
 Localized
 CLASSIFICATION

►Two systems used

1-Based on pathogenesis
2-Based on size of the involved
vessels
►Clinical implications of
mechanism of vasculitis
CLASSIFICATION OF VASCULITIS
BASED ON
PATHOGENESIS
 DIRECT INFECTION
►RICKETTSIAL-ROCKY MOUNTAIN SPOTTED
FEVER

►BACTERIAL-NEISERRIA

►SPIROCHAETAL-SYPHILIS

►FUNGAL-ASPERGILOSIS, MUCORMYCOSIS

►VIRAL-HERPES ZOSTER
 CLASSIFICATION OF VASCULITIS
BASED ON PATHOGENESIS (Contd…)

► IMMUNOLOGIC
 IMMUNE COMPLEX MEDIATED
►INFECTION-HEPATITIS
B&C
►HENOCH-SCHONLEIN PURPURA(HSP)
►SLE &RA
►DRUGS
►CRYOGLOBULINAEMIA
►SERIUM SICKNESS
 CLASSIFICATION OF VASCULITIS
BASED ON PATHOGENESIS (Contd..)

► ANCA MEDIATED
 WEGENER GRANULOMATOSIS (C-ANCA)
 MICROSCOPIC POLYANGITIS (P-ANCA)
 CHURG STRAUSS SYNDROME (P-ANCA)
► DIRECT ANTIBODY ATTACK MEDIATED
 GOOD PASTURE SYNDROME
 KAWASAKI DISEASE
 CLASSIFICATION OF VASCULITIS
BASED ON PATHOGENESIS (Contd..)

► CELL MEDIATED
 ALLOGRAFT ORGAN REJECTION
 INFLAMMATORY BOWEL DISEASE
 PARANEOPLASTIC
► UNKNOWN
 GIANT CELL ARTERITIS
 TAKAYASU ARTERITIS
 POLY ARTERITIS NODOSA (PAN)
Classification of Vasculitis Based on
Size of Vessel involved
 CLASSIFICATION on the basis of
SIZE
► Large Size ► Medium Size ► Small Size
1. Giant Cell 1. Polyarteritis 1. Wegner’s
Nodosa Granulomatosis
2. Takayasu’s
2. Microscopic
2. Kawasaki
Polyangitis
Disease 3. Churg Strauss
3. Buerger’s Syndrome
Disease 4. HENOCH-
SCHONLEIN
PURPURA(HSP)
LARGE VESSELS VASCULITIDIES
1- GIANT CELL (TEMPORAL)
ARTERITIS

►“It is acute and chronic often

granulamatous inflammation of
medium sized and small
arteries”
 SITES OF INVOLVEMENT

►Temporal arteries*
mainly
►Vertebral arteries
►Ophthalmic arteries
►Aortic arch
 MORPHOLOGY
► Short segment* involved
► Nodular thickening
due to intimal fibrosis
► Narrowing of lumen –
thrombosed
► Granulamatous inflammation
of inner half of T. media
and of the inner elastic
lamina causing Elastic
lamina fragmentation
with infiltrates of T cells and
Macrophages
► Nonspecific panarteritis in
chronic settings
 PATHOGENESIS

►Exact unknown
►Immunologic reaction against
elastin(Anti endothelial and anti
Smooth muscle Igs)
►T. cell mediated injury suspected
(main entity)*
 CLINICAL FEATURES
► Most common in older individuals (>50 yoa)
► Vague symptoms e.g. fever, fatigue &
weight loss
► Facial pain or headache – unilateral
► Nodular and painful superficial temporal
artery
► Diplopia to vision loss
► Raised ESR
 DIAGNOSIS & PROGNOSIS

►Urgency
►Diagnosis by biopsy
►Negative biopsy – Treatment
on clinical ground.
 2- TAKAYASU ARTERITIS

► Granulomatous arteritis of large and

medium arteries
► Called PULSELESS* disease
► Raised ESR
► Involves aorta*Aortic-Arch primarily
► In <50 yoa
► Histologically similar to GCA
MEDIUM VESSELS VASCULITIDIES
1- POLYARTERITIS NODOSA (PAN)

“ It is a systemic vasculitis
manifested by transmural
necrotizing inflammation of small
or medium sized muscular
arteries typically involving renal
and visceral vessels but sparing
the pulmonary circulation*”
 ETIOLOGY

►Mostlyidiopathic
►30% - positive for HBsAg*
 ORGANS INVOLVED

►Kidneys
►Heart
►Liver
►GIT
►Pancreas
►Testes
►Skeletal muscle
►Nervous system
►Skin
 NATURE OF LESION
► Focal, random and episodic
► Irregular aneurysmal dilatation,
nodularity, vascular obstruction
and infarction
► All stages of activity in different
vessels or in same vessel
► String of pearl appearance on
imaging showing varying
► Necrotic Aneurysm and adjacent
fibrosis
 CLINICAL FEATURES

► Disease of young adults*

► Course: acute, subacute, chronic and
remittent
► Wide spread
► Malaise, FUO, weight loss, hypertension
, aches & pains, malena, peripheral
neuritis
► Renal invdvement – No
glomerulonephitis
 DIAGNOSIS & PROGNOSIS

►Angiography (string of pearl

appearance*)
►Tissue biopsy (kidneys or skin)
necrotizing arteritis
►Untreated – fatal
►Corticosteroids and
cyclophosphamide* – 90% cure
 2-KAWASAKI Disease
► Triad- *
 Fever, Conjunctivitis & Erythematous Rash on Palms
and Soles
 Enlarged Cervical LN hence also called
MUCUCUTANEOUS LN SYNDROME*
► Inchildren <4yoa*
► Histology is similar to PAN less the fibrinoid
necrosis*
► Coronary artery involved first *(Anuerysm and
Rupture)
► Aspirin* is indicated
 3-BUERGER’S DISEASE
► Thromboangiitis Obliterans
► HEAVY SMOKERS
► ARTERIES & VEINS OF
EXTREMITIES
► COLD SENSITIVITY & LEG
CLAUDICTION
► NODULAR PHLEBITIS
► THROMBOSING ACUTE &
CHRONIC INFLAMMATION
► Thrombus may contain small
micro abscesses
► May extend into Veins and
Nerves
SMALL VESSELS VASCULITIDIES
 1- WEGENER’S GRAULOMATOSIS
(Granulomatous Polyangitis)
►Anecrotizing vasculitis characterized by triad:
1. Acute necrotizing granulomas of
respiratory tract (the lungs)*
2. Focal necrotizing or granulamatous
vasculitis of small to medium sized vessels
of lungs and upper airways
(Nasophyranx)*
3. Renal disease - necrotizing crescentic
glomerulitis(Kidneys)*
 PATHOGENESIS

►Exact unknown
►Hypersensitivity to inhaled
infectious or environmental agents
►Immune mechanisms-cell mediated
►c-ANCA present in serum of 90%
patients
 MORPHOLOGY
► Upper respiratory tract
 Ulcers nose, throat, palate,
pharynx
 Geographic patterned
Necrotizing granulomas
► Lungs – nodules and cavities
– necrotizing
granulomas
► Renal lesions – focal
necrotizing glomerulonephitis
& crescentic glomerulonephitis
 CLINICAL FEATURES

► Peak in forties
► Persistent pneumonitis
► Chronic sinusitis
► Mucosal ulceration of nasopharynx
► Renal disease
► Skin rashes, fever, muscle pains and
neuritis
 PROGNOSIS
►Untreated – 80% die within one year
►Good response with cyclophosphamide
and steroids
 Microscopic Polyangiitis
► Nacrotizingbut no GRANULOMA
► No Nasophyrangeal involvement
► P-ANCA positive
► Leukocytoclastic Vasculitis
► Pauci Immune injury
 Churg Strauss Syndrome
► Nacrotizing
► Peripheral
eosinophilia
► Granuloma and Asthma is there
► P-ANCA positive
 Behcet Disease
► Triad of
1. Aphthous Ulcers
2. Genital Ulcers
3. Uveitis
 VARICOSE VEINS

► SITES
 SUPERFICIAL LEG VEINS
 GASTRO-OESPOHAGEAL VEINS
 ANO-RECTAL
► CAUSES
 FAMILIAL
 POSTURE
 PRESSURE – PREGNANCY
 VARICOSE VEINS (Contd..)
► MORPHOLOGY
 DILATATION, TORTUOSITY, ELONGATION AND
SCARRING
 VALVULAR DEFORMITIES
► CLINICAL COURSE
 VENOUS CONGESTION & DISTENSION
 THROMBOSIS
 STASIS DERMATITIS
 VARITOSE ULCERS
 GASTROINTESTINAL HAEMORRHAGE
 THROMBOPHLEBITIS

►SITES
 DEEP LEG VEINS –(90%)
 PERIPROSTATIC VENOUS
PLEXUS
 PELVIC VEINS
 DURAL SINUSES
 PORTAL VEINS & TRIBUTARIES
 CAUSES OF VENOUS THROMBOSIS
► IMMOBILITY: CCF, POST OPERATIVE, BED REST,
FRACTURES

► MALIGNENT NEOPLASIA

► PREGNANCY & CHILD BIRTH

► OESTROGENTHERAPY (ORAL CONTRACEPTIVE,

TREATMENT OF CARCINOMA PROSTATE)

► POLYCYTHEMIA,ANTITHROMBIN – III
DEFICIENCY AND DEHYDRATION
 THROMBOPHLEBITIS (Contd..)
► CLINICAL FEATURES
 OEDEMA & SWELLING
 REDNESS
 HOMAN’S SIGN
 MIGRATORY THROMBOPHLEBITIS
(TROUSSEAU SIGN)
 PULMONARY EMBOLISM
 PAINFUL WHITE LEG
 ANEURYSM
• DEFINITION
“It is localized, permanent, abnormal
dilatation of blood vessels that occurs
mostly in the aorta or the heart”
• TYPES:
– MACROSCOPIC
SHAPE
– TRUE OR FALSE
 ANEURYSM (Contd..)
• CAUSES
– CONGENITAL DEFECT
– INFECTIONS (MYCOTIC)
– SYPHILIS
– TRAUMA
– ATHEROSCLEROSIS
– CYSTIC MEDIAL DEGEBERATION
– IMMUNOLOGIC
PATHOGENESIS of ANEURYSM
• Due to compromise of the structure or
function of the CT in the vessel wall
– Defective CT as in Marfan’s (fibrillin) or Ehler
Danlos(Collagen defect)
– Imbalance of Collagen degradation and
synthesis(raised MMPs)
– Vessel wall atrophy due to (most common for
Aortic aneurysms)
• HTN(Ascending/THORACIC aneurysm and
dissection)
• ATHEROSECLEROSIS (AAA)
TYPES & SITES OF ANEURYSMS
 ABDOMINAL AORTIC ANEURYSM

• > 50 YEARS, MALE

• BELOW RENAL ARTERIES AND ABOVE
BIFURCATION OF AORTA
• ATHEROSCLEROSIS
• HYPERTENSION
• FAMILIAL
• CONNECTIVE TISSUE COMPONENT DEFECT
• METALLOPROTEINASES (MMPs)
 COURSE OF DISEASE
• RUPTURE &
HAEMORRHAGE
• OCCLUSION OF BRANCH
VESSELS
• EMBOLISATION
• COMPRESSION OF
NEARBY STRUCTURES
• PULSATING MASS
AORTIC DISSECTION / DISSECTING
HAEMATOMA
• ETIOLOGY
– HYPERTENSION (40-60 YRS)
– MARFAN SYNDROME (YOUNGER)
– ARTERIAL CANNULATION
– PREGNANCY
 AORTIC DISSECTION/DISSECTING
HAEMATOMA (Contd..)

• INTIMAL TEARS
• DISSECTION
ALONG LAMINAR
PLANES
• DOUBLE
BARRELED -
chronic
• CYSTIC MEDIAL
DEGENERATION
 AORTIC DISSECTION / DISSECTING
HAEMATOMA (Contd..)

• TYPE A & B
• AORTIC RUPTURE
• SUDDEN
EXCRUCIATING PAIN
• EXTENSION INTO
BRANCHES

Type-A Type-B
 SYPHILITIC ANEURYSM
• TERTIARY STAGE
• THORACIC AORTA
• OBLITERATIVE END ARTERITIS
OF VASA VASORUM
• MEDIAL DESTRUCTION DUE TO
ISCHAEMIA
• TREE BARKING
• CAR BOVINUM
• COMPRESSION EFFECTS
 VASCULAR TUMOURS

BENIGN TUMOURS
• CAPILLARY HAEMANGIOMA
• CAVERNOUS HAEMANGIOMA
• PYOGENIC GRANULOMA
• CYSTIC HYGROMA
• GLOMUS TUMOUR
BORDERLINE / LOW GRADE MALIGNANT
TUMOURS
– KAPOSI SARCOMA
– HAEMANGIOENDOTHELIOMA

MLAIGNANT VASCULAR TUMOURS

– ANGIOSARCOMA
 HAEMANGIOMA
• TYPES
– CAPILLARY
– CAVERNOUS
• ANGIOMATOSIS
• AGE
• COSMETIC DISTURBANCE
• BRAIN-MOST
THREATENING
 CAPILLARY HAEMANGIOMA

• SITES
– SKIN, MUCUS MEMBRANE
(MOUTH) LIVER, SPLEEN &
KIDNEY ETC,
• JUVENILE
(STRAWBERRY TYPE)
• FEW mm TO SEVERAL
cms
• LOBULATED BUT
UNCAPSULATED
 CAVERNOUS HAEMANGIOMA
• AGE & SITE
• LARGE VASCULAR
CHANNELS
• INTRAVASCULAR
THROMBOSIS
• LESS CIRCUMSCRIBED
 PYOGENIC GRANULOMA
(Lobular Capillary Haemangioma)

• SITES - SKIN, GINGIVA

• CAUSES
– TRAUMA, CHRONIC IRRITAION,
PREGNANCY
• GRANULOMA GRAVIDARUM
 LYMPHANGIOMA

• CYSTIC HYGROMA
– CHILDREN
– NECK, AXILLA OR
RETROPERITONIUM
– CYSTIC SPACES WITH LYMPHOID
AGGREGATES
 GLOMUS TUMOUR

• BENIGN, PAINFUL TUMOUR

• MODIFIED SMOOTH MUSCLE
CELLS OF GLOMUS BODY
• FINGER NAILS, SKIN, SOFT
TISSUE, G.I.T
• MINUTE HAEMORRHAGES
• VASCULAR CHANNELS & MASSES
OF GLOMUS CELLS
 ANGIOSARCOMA
• MLAIGNANT ENDOTHELIAL
TUMOUR
• SKIN, SOFT TISSUE, BREAST &
LIVER
• ARSENIC, THOROTRAST, PVC
• POST MASTECTOMY
• HIGHLY DIFFERNTIATED TO
ANAPLASTIC
• LOCAL INVASION TO DISTANT
METASTASIS
 HAEMANGIOPERICYTOMA
• DERIVED FROM PERICYTES
• SLOWLY GROWING & PAINLESS
• THIGH & RETROPERITONEUM
• SILVER STAINS FOR CONFIRMATION
• 50% METASTASIZE LIVER, LUNGS &
BONES.
 KAPOSI SARCOMA
• BORDERLINE, LOW GRADE
MALIGNANT
• UNCERTAIN PATHOGENESIS
• VIRAL-ASSOCIATED; HIV, HERPES
VIRUS-8
• COURSE & PRESENTATION VARY
WIDELY
 KAPOSI SARCOMA
• CHRONIC, CLASSIC OR EUROPEAN
OLDER MEN OF EASTERN EUROPE (JEWS)
NOT ASSOCIATED WITH HIV
RED TO PURPLE PLAQUES, LOWER
EXTREMITIES
LOCALLY PERSISTENT, LAPSES &
REMISSIONS
 KAPOSI SARCOMA (Contd..)
• LYMPHADENOPATHIC, AFRICAN
OR ENDEMIC
• YOUNG BANTU CHILDREN – S. AFRICA
• LOCALIZED/GENERALIZED
LYMPHADENOPATHY
• AGGRESSIVE DISEASE
• SPARSE SKIN LESIONS
 KAPOSI SARCOMA (Contd..)
• AIDS-ASSOCIATED (EPIDEMIC)
• ¼ OR MORE AIDS PATIENTS
• HOMOSEXUAL MEN
• SKIN, LYMPH NODE, GIT,
METASTASIS
• SECOND MALIGNANCY –
LYMPHOMA
 KAPOSI SARCOMA (Contd..)

• TRANSPLANT ASSOCIATED
• HIGH DOSE
IMMUNOSUPPRESSIVE THERAPY
• SKIN OR WIDE METASTASIS
MORPHOLOGY OF KAPOSI SARCOMA

THREE STAGES
PATCH, PLAQUE & NODULE
THANK YOU