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BLOOD VESSELSDr. Dexter MD FRC Path
Undercover Professor
DEPARTMENT OF PATHOLOGYSGUSOM GRENADA (W.I.)
Anatomy & Histology
Three layers ofarteries
Intima endothelial cells
Media smoothmuscle cells
Adventitia connectivetissue
3
ENDOTHELIAL CELL FUNCTIONS
Maintenance of Permeability Barrier Elaboration of Antithrombotic & Prothrombotic
Molecules Modulation of Blood Flow and Vascular
Reactivity Endothelin/NO
Regulation of Inflammation and Immunity IL-1, IL-6
Regulation of Cell Growth PDGF/TGF-
Oxidation of Low-Density Lipoprotein
Endothelial Dysfunction Endothelial stimulation rapid, re-
versible; independent of new proteinsynthesis.
Endothelial activation - alteration in
gene expression and protein synth
5
Characteristics of an activated endothelial cell
A normal arterial wall has anticoagulant properties and low leukocyte adhesivity
Complement products
Hypoxia
MHC moleculesViruses
Coagulation proteAdvanced glycosylationend products
Vasoactive mediaLipid products
Growth factorsHemodynamic forces
Cytokines / chemoBacterial productsAdhesion moleculCytokines
Induced genActivators
Endothelial activation
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Vascular Smooth Muscle Cells
Functions
Vasoconstriction and dilation in
response to normal or pharmacological
stimuli
Elaboration of growth factors andcytokines
Migration to the intima and proliferation
Diseases of Blood Vessel
Two principal mechanisms
1. Narrowing or complete obstructiothe lumen either
progressively (atherosclerosis) or
suddenly (thrombosis)
2. Weakening of the wall of the vessleading to dilatation or rupture.
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Other Categories
Hypertension
Inflammatory disorders -vasculitis
Congenital Malformations
Neoplasms
ARTERIOSCLEROSIS
ATHEROSCLEROSISLARGE BV
INTIMA
MEDIAL CALCIFICSCLEROSIS
MEDIA
ARTERIOLOSMALL
FULL TH
HYPER
HYALINE
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Atherosclerosis
Derived from Greek word ATHEROS soft gruel or porridge like
Chronic inflammatory disorder of
intima of large arteries characterizedby formation of fibro fatty plaques
called atheroma.
EPIDEMIOLOGY
Five Leading Causes of Death for Males and Females U.S
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Atherosclerosis Major RiskFactors
NONMODIFIABLE
Age
Gender
Geneticpredisposition
MODIFIABLE
Hyperlipidemia
Hypertension
Cigarettesmoking
Diabetes mellitus
Atherosclerosis Uncertain, Unquantifiable or Possibly L
Risk Factors
Hyperhomocystinemia
Lipoprotein (a)
Inhibitors of fibrinolysis
Low PA-1 Inhibitor
C-reactive protein
Lack of exercis
Type A perso Obesity Trans-unsat
intake
Postmenopaus
estrogen defic High carbohyd Chlamydia pne
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NON-MODIFIABLE RISK FACTORS
AGE - begins in childhood and progresses withage
40 -60 yrs five fold increase in incidence of MI
SEX - Males > females Uncommon in premenopausal women
Postmenopausal Increased incidence
Favorable response to HRT
GENETICS
Familial clustering of other risk factors
Familial hypercholesterolemia
MODIFIABLE RISK FACTO
HYPERLIPIDEMIA
cholesterol most important
Major component associated with riskLDL bad cholesterol.
inverse relationship between HDL levelgood cholesterol.
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Evidences linking cholesterol with
Atherosclerosis
Genetic defects in lipoprotein metabolism Familial hypercholesteremia defect in LDL
receptor hepatic uptake of LDL levelsof LDL
LDL because of abnormal apo E it fails tobind to LDL receptor
Genetic or acquired diseases like DM &
hypothyroidism premature & severe
A.S.
Evidences linking cholesterol w
Atherosclerosis
Atheromas contain cholesterol & cholestero
levels of cholesterol & LDL severity o
High cholesterol diets produce experimenta
levels of cholesterol by diet/drugs progof A.S.
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RISK FACTORS
HYPERTENSION
More importance after age of 45
169/95- 5 fold risk of developing IHD ascompared to 140/90
Anti hypertensive therapy reduces
incidence of A.S. associated diseases
stroke, IHD
RISK FACTORS
CIGARETTE SMOKING
Smoking & A.S. Made For Each Other
1/> packs/day for several years death raincreases up to 200%
DIABETES MELLITUS
Cholesterol predisposition to AS
100x increased risk of AS induced gangrof the lower extremities.
risk of strokes and M.I. AS in young patientsSuspect DM
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RISK FACTORSPLASMA HOMOCYSTEINE
Hyper-Homocystinuria -Patients haveremature vascularisease
Can be caused byow folate & vitaminB intake
RISK FACTORS
LIPOPROTEIN (a)
Altered form of LDL
Potentially atherogenic effects
Lipid accumulation
Endothelial cell modulation
Smooth muscle cell proliferation
Control of neo-vascularization
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LESSER FACTORS
Stress
Type A personality
Obesity
MULTIPLE RISK FACTORS MAYHAVE A MULTIPLICATIVE & NOT
ADDITIVE EFFECT
LOWER THE BETTER
Blood sugar
Blood pressure
Body weight
LDL
Stress levels
No. of cigarettes
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PATHOGENESIS
RESPONSE TO INJURY HYPOTHESIS
Chronic inflammatory response of thearterial wall initiated by some form of the
injury to the endothelium.
RESPONSE TO INJURY HYPOTHE
Role of endothelial cells endothelia
Endothelial activation
Increased permeability
Increased adhesion of leukocytes
Increased expression of adhesion molecules
Growth factors
Determinants of endothelial alterations
Hemodynamic factors increased frequenc
lesions at branch points, ostia of vessels
Hypercholesterolemia
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RESPONSE TO INJURY HYPOTHE
Role of lipids
Cytotoxic to endothelium
Oxidized (modified) lipids have atherogproperties
readily ingested by macrophages foam ce
chemotactic for circulating monocytes
inhibits the motility of macrophages alreadylesion
stimulates release of growth factors and cy
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RESPONSE TO INJURY HYPOTHESIS
Role of macrophages Cytokines like IL-1, TN(procoagulant
endothelium)F
Growth factors
Role of smooth muscle proliferation
Conversion of fatty streak into fibrofattyatheroma
Role of T-cells
Recruitment and activation of monocytes
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Atherosclerotic plaque
Three principal components
Cells smooth muscle cells,
macrophages, and other leukocytes
Extracellular matrix including collelastic fibers, and proteoglycans
Intracellular and extracellular lipiddeposits
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Complicated lesion ofatherosclerosis
Calcification may be patchy or massive.
Focal rupture or gross ulceration, or both,leading to
thrombus formation
cholesterol emboli or atheroemboli
Hemorrhage .
Superimposed thrombus
Aneurysmal dilatation atrophy of underlying
media
Sites of severeatherosclerosis in
order of frequency
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Atherosclerosis-ClinicalManifestations
Coronary heart disease(CHD)
Acute myocardialinfarction (AMI)
Angina Stable orUnstable
Chronic ischemic heartdisease leading tocongestive heartfailure(CHF)
Sudden cardiac death
Abdominal aorticaneurysm (AAA)
Cerebral vasculardisease
Stroke
Transient ischemicattack (TIA)
Chronic ischemicencephalopathy
Peripheral vasculardisease
Claudication
Ischemic bowel disease(mesenteric occlusion)
GangrenePRECPHAS
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Hypertension
Hypertension
Normal < 130 mm Hg systolic & < 85 mm diastolic)
Hypertension - Sustained increase in bloodpressure
Systolic > 140 mm Hg
Diastolic > 90 mm Hg
Mild + 20; Moderate +40; Severe +80 mm (systolic)
Malignant hypertension - > 210/120
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Hypertension
25% of persons in general population are
hypertensive
Leading risk factor MI, DM, Stroke
Silent Killer painless complications
Complications bring to diagnosis but late
Chronic, end organ & vascular damage
Regulation of BP:
BP = Cardiac Output x Peripheral Resista
Endocrine Factors
Renin, Angiotensin, ANP, ADH, Aldosterone.
Neural Factors
Sympathetic & Parasympathetic
Blood Volume
Sodium, Mineralocorticoids, ANP
Cardiac Factors
Heart rate & Contractility.
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Etiologic Classification:
Primary/Essential Hypertension (95%)Secondary Hypertension (5-10%)
Renal GN, RAS, Renin tumors
Endocrine Cushing, OCP, ThyrotoxicosisMyxdema, Pheochromocytoma, Acromegaly.
Vascular Coarctation of Aorta, PAN, Aorticinsufficiency.
Neurogenic Psychogenic, Intracranialpressure, polyneuritis etc.
Pathogenesis
Essential - multifactorial. Increased peripheral resistance (sympatheti stress, hormonal, neural.
Genetic, familial, life style.
Secondary - Known abnormal control. Increased blood volume - Sodium retention A
Aldosterone.
Increased sympathetic tone - Adrenal tumorsympathetic stimulation.
Increased vasoactive hormones - Cushing'sPheochromocytoma,
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GENETIC FACTORSINCREASED
SYMPATHETICACTIVITY
INCREASEDCOP
INCREASED PERIPHERALRESISTANCE
HYPERTENSION
INCREASED RENALSODIUM RETENTION
INCREASEDBLOOD VOLUME
INCREASED RENINALDOSTERONE ACTIVITY
AUTOREGULATION ARTERIOLAR NARROWING
CESS DIETARY SODIUMRONIC RENAL DISEASE
CUSHING SYNDROMEHYPERALDOSTERONISM
PHEOCHROMOCYTOMA
Malignant Hypertension
Greater than 210/120 BP
May complicate any type of HTN.
Necrotizing arteriolitis.
Rapidly progressive end organ dama
Renal failure
Left ventricular failure ypertensive encephalopathy
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Hypertension - complications: Large Blood Vessels
Atherosclerosis and its complications.
Small Blood Vessels
Arteriolosclerosis Hyaline & Hyperplastic
Heart
Left ventricular hypertrophy, Hypertensivecardiomyopathy IHD, MI.
Kidney
Nephrosclerosis Benign & Malignant.
Eyes:
Hypertensive retinopathy, hemor rhage
Brain: Haemorrhage, infarction (stroke),
Hyaline arteriosclerosis
Homogenous, pink, hyaline thickening of twalls of the arterioles with loss of underlyistructural details and with narrowing of thelumen.
Leakage of plasma components across vaendothelium and increasing extracellular mproduction by smooth muscle cells
Chronic hemodynamic stress in hypertensmetabolic stress in diabetes accentuates edothelial injury
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Hyperplastic arteriosclerosis
Related to more acute or severeelevations of blood pressure
Onion-skin, concentric, laminatedthickening of the walls of arterioles withprogressive narrowing of the lumens.
Necrotizing Arteriolitis - deposits offibrinoid and acute necrosis of the vessel
wall.
VASCULITIS
Inflammation and necrosis of the bloovessels, including arteries, veins, andcapillaries.
can be classified based on
Type of vessel involved
Etiology
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Type of vessel involvedEtiology
Direct infection- Bacterial, Rickettsial, Spirochetal, Fungal
Immunological
Immune complex mediated
(hepatitis B or C virus mediated)
SLE and rheumatoid arthritis
Drug induced
Antineutrophil cytoplasmic antibody (ANCA) mediated
Wegeners granulomatosis
Churg-Strauss syndrome
Direct antibody attack mediated
Goodpasture syndrome (anti-GBM antibodies)
Kawasaki disease (antiendothelial antibodies)
Cell mediated
Allograft organ rejection Unknown
Giant cell (temporal) arteritis
Takayasu arteritis
Polyarteritis nodosa
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Anti- Neutrophil Cytoplasmic Antibodies
(ANCA)
Heterogeneous group of auto-antibodiesagainst enzymes mainly found within the
primary granules in neutrophils and inlysosomes of monocytes and in endothelialcells
P- ANCA: microscopic polyangiitis &
Churg-Strauss C-ANCA: Wegners granulomatosis
c-ANCAc-ANCA Proteinase-3
p-ANCA - Myeloperox
IMMUNOFLUORESCENT STAINING
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Giant Cell (Temporal) Arteritis
Most common vasculitis
Affects mainly the arteries in the head
Temporal
Vertebral
Ophthalmic can lead to blindness
Pathogenesis unknown
T-cell mediated immunologic reactionagainst elastin?
Clinical features
Age: 50 and above
Gender: M:F: 1-1
Symptoms, Vague: fever, weight lossfatigue, facial pain, headache
Ocular symptoms: diplopia, progress
hazy vision, loss of vision
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Giant cell arteritis
Diagnosis: Temporal Artery Biopsy
2-3 cm segment
multiple sections
elastic trichrome stain
Treatment: Steroids, Analgesics
Prognosis: Good
Elevated ESR (sed-rate)
Association with polymyalgia rheumatica
Takayasu arteritis
Granulomatous vasculitis ofmedium and large arterieswith obliteration of lumen
Most commonly affects archof aorta with narrowing orvirtual obliteration of theorigins of great vesselsarising in the arch
Can also involve pulmonary,
coronary, and renal arteries. Etiopathogenesis
Unknown
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Clinical features
Most common in females < 40 years
Ocular changes: visual disturbances, retinalhemorrhages, blindness
Progressive diminution of upper limb pulses withcoldness or numbness of fingers - PulselessDisease
Low BP in upper limb
Neurologic defects dizziness, focal weakness
or complete hemiparesis
Polyarteritis Nodosa (PAN
Systemic vasculitis
Transmural necrotizing inflammation
small or medium sized muscular arte
Typically involves renal and visceral
arteries and spares the pulmonarycirculation.
Arterioles, capillaries, and venules ar
affected
Associated with Hepatitis B antigen (
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re
Remember
No glomerulonephritis
Pathguy..com
Disease of
oung adultsCourse may becute,bacute, or
ronic and isequentlymittent.0% of
atients haveBV antigen inerum
Clinical features Morphology
Gross - Distribution of lesions in descending ordfrequency Kidney, Heart, Liver, GIT
Lesions have a predilection for branching points abifurcations
Histologically Acute stage - Transmural inflammation, fibrinoid necros
inner half of the vessel wall.
Later stages - inflammation is replaced by fibrous thickeof the vessel wall. Risks of thrombus or infarction.
All stages of activity may coexist in different vor even within the same vessel characteristifeature
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Complications - PAN
Weakening of arterial wall owing to
inflammatory process may causeaneurysmal dilatation or localized rupture.
Impairment of perfusion causingulcerations, infarcts, ischemic atrophy, or
hemorrhages in the areas supplied by
these vessels.
Kawasaki syndrome
Arteritis involving the large, medium,small arteries (often coronary arteries
Associated with mucocutaneous lymnode syndrome
Usually in young children and infantscases < 4 years)
Epidemic in Japan Leading cause of acquired heart dise
in children in US(can lead to MI)
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Kawasaki disease
Pathogenesis Unknown
Autoantibodies against endothelium, smoothmuscle cells, leading to acute vasculitis
Clinical features Fever
Conjunctival and oral erythema
Edema of the hands and feet
Skin rash often with desquamation
Enlargement of cervical lymph nodes
Buerger disease(Thromboangitis obliterans
Characterized by segmental, thrombosing, and chronic inflammation of medium sized asmall arteries, characterized by microabces
Mainly the tibial and radial arteries and somsecondarily extending to veins and nerves oextremities(painful)
Predisposing factors
Cigarette smoking
Hypersensitivity reaction to tobacco direct end
injury Genetic influence
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Wegener Granulomatosis Classic triad
1. Acute necrotizing granulomas of upper and lower respiratorytract
2. Focal necrotizing or granulomatous vasculitis affecting small tomedium sized vessels, most prominent in lungs, and upperairways but affecting other sites as well
3. Renal disease in the form of focal or necrotizing, oftencrescentic glomerulitis
Limited WG No renal involvement
Males > females
Average age of onset = 40 years
95% of the patients have c-ANCA
Clinical features
Persistent pneumonitis with bilateralnodular and cavitary infiltrates
Chronic sinusitis
Mucosal ulceration of the nasophary
Evidence of renal disease
Untreated patients die within 1 year.
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Morphology
Gross ulcerativelesions of the nose,pharynx, palate
Lungs - dispersedfocal necrotizinggranulomascoalesce to
produce nodulesthat may undergocavitations.
Aneurysms
Localized abnormal dilatation of a blo
vessel caused by a congenital oracquired weakness in the media.
Aneurysms can be classified based o
Composition of wall
Gross morphology
Etiology
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Composition of the aneurysmal sac
True
Complete but attenuated vessel wall. The bloodremains within the confines of the circulatorysystem.
Atherosclerotic, syphilitic, and congenitalvascular aneurysm
False (pseudoaneurysm)
is an extravascular hematoma that
communicates with the intravascular space.
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Etiology
Atherosclerosis
Syphilis
Mycotic infective.
Vasculitis (PAN, Kawasakis disease)
Congenital defect in media Marfanssyndrome, Berry aneurysm
Iatrogenic Arteriovenous aneurysms for
chronic renal failure patients for dialysis
Atherosclerotic aneurysm
Most common site abdominal aorta
Abdominal aortic aneurysm - diamete
increased at least 50%.
Most frequent aneurysms, usually
developing after the age of 50 years.
6% after the age of 80 years
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Clinical course Many aneurysms are asymptomatic.
Abdominal mass
Occlusion of a branch vessel- renal, mesentericvertebral vessels.
Embolism from atheroma or mural thrombus.
Impingement on an adjacent structure
compression of ureter or erosion of vertebrae.
Rupture with massive or fatal hemorrhage.
The risk of rupture - about 2% for a smallabdominal aortic aneurysm (50%.
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Syphilitic aneurysms
Seen in the tertiary stage of the syphilis.
Due to obliterative endarteritis of the vasa
vasorum of the aorta - Destruction of the
tunica media
Leading to narrowing of the lumen of thesevessels which causes ischemic injury of
the aortic media followed by inflammationand scarring.
Clinical course
Encroachment on the mediastinal structures byenlarged heart (Cor bovinum)
Respiratory difficulties.
Difficulty in swallowing.
Persistent cough due to pressure on recurrent laryngea
Pain caused by erosion of the ribs or vertebrae.
Valvular incompetence (aortic regurgitation) leadleft ventricular hypertrophy, cardiac ischemia dueobstruction to coronary ostia.
Rupture of the aneurysm.
Most common cause of death - heart failure d
aortic regurgitation.
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Aortic dissection (dissectinghematoma)
Entry of blood in between and along thelaminar planes of media and its extensionalong the length of the vessel.
Often ruptures causing massivehemorrhage
Not usually associated with marked
dilatation of aorta
Etiopathogenesis
Most commonly men 40-60 years of age, whom hypertension is almost always pres(>90% of cases of dissection).
Younger patients with abnormality of conntissue (Marfans syndrome).
As a complication of arterial cannulation (e.g. during diagnostic
catheterization or cardiopulmonary bypass)
During pregnancy - unknown reason. Can result from cystic medial necrosis (los
elastic tissue)
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Morphology
Intimal tear - starting point
Occurs most commonly in the ascending aorta,1 or 2 cm above the aortic ring.
The dissection separates the inner two thirds ofthe aorta from the outer third.
The dissection can extend proximally toward theheart as well as distally along the aorta to
variable distances. Re-rupture into the lumen of the aorta - double-
barreled aorta.
Double-barreled aorta.
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2 Types
Type A - the morecommon (and dangerous)proximal lesions,involving either theascending portion only orboth ascending anddescending aorta.
Type B distal lesionsnot involving theascending part andusually beginning distal tosubclavian artery
Type A Type B
Clinical features
Sudden onset of excruciating (tearing
pain, usually beginning in the anteriochest, radiating to the back, and mov
downwards as the dissection progres
Loss of one or more arterial pulses is
common
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Complications
Most common cause of death - rupture of thedissection into any of the three major bodycavities (i.e., pericardial. Pleural, and peritoneal)
Retrograde dissection into the aortic root leadingto disruption of aortic valvular apparatus.
Compression of spinal arteries - Transversemyelitis.
Cardiac tamponade, aortic insufficiency, andmyocardial infarction can also occur.
Mycotic (infectious) aneurys
result from the weakening of the vesswall by a microbial infection.
Common sites of involvement include
aorta, cerebral vessels, and mesenterenal, and splenic arteries.
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Blood supply
Functionally
- Right & left coronary arteries are end arteries
- Numerous interconnections collateral
circulation contains little blood in normal hearts
- Collaterals open up when one artery is severely
narrowed
Most blood flow to the myocardium is duringdiastole
Heart failure(congestive heart failure)
Is the inability of the heart, working a
normal or elevated filling pressure, to
pump enough blood to meet the meta
demands of the body.
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Etiology Systolic dysfunction inability to contract properly
MI
Hypertension
Volume overload valvular regurgitation
Cardiomyopathy
Diastolic dysfunction - Inability of the heart chamberto relax/expand and fill sufficiently during diastole
Massive ventricular hypertrophy
Amyloidosis
Constrictive pericarditis
CHF can also be categorize
Left-sided failure left ventricle is the failin
chamber
Systemic hypertension
Mitral or aortic valve disease
Ischemic heart disease
Cardiomyopathy
Right-sided failure
Left heart failure
Intrinsic disease of lung parenchyma/vasculat
COPD, Pulmonary hypertension
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Compensatory Mechanisms
- Adrenergic stimulation by endogenouscatecholamines
- Myocardial hypertrophy
- Concentric hypertrophy - hypertension
- Eccentric hypertrophy valvular regurgitation
- Myocardial dilation - Frank Starlings law increased force of contraction with dilation
Compensated heart failure dilatedventricle is able to maintain cardiac oto meet the needs of the body
Decompensated heart failure failingmyocardium is no longer able to prop
sufficient blood to meet the needs of body, even at rest.
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HEARTFAILURE
DECREASEDDECREASEDDECREASEDDECREASED
CARDIAC OUTPUTCARDIAC OUTPUTCARDIAC OUTPUTCARDIAC OUTPUTRENALRENALRENALRENAL
OPERFUSIONOPERFUSIONOPERFUSIONOPERFUSION
SODIUM &SODIUM &SODIUM &SODIUM &
ER RETENTIONER RETENTIONER RETENTIONER RETENTION
RENINRENINRENINRENIN----ANGIOTENSIN SYSTEMANGIOTENSIN SYSTEMANGIOTENSIN SYSTEMANGIOTENSIN SYSTEM
EDEMA
PASSIVE CONGESTIONPASSIVE CONGESTIONPASSIVE CONGESTIONPASSIVE CONGESTION
OF PULMONARY CIRCULATIONOF PULMONARY CIRCULATIONOF PULMONARY CIRCULATIONOF PULMONARY CIRCULATION
PULMONARY ARTERIALPULMONARY ARTERIALPULMONARY ARTERIALPULMONARY ARTERIAL
HYPERTENSIONHYPERTENSIONHYPERTENSIONHYPERTENSION
RIGHT HEART FAILURE
SEVERE &SEVERE &SEVERE &SEVERE &SUSTAINEDSUSTAINEDSUSTAINEDSUSTAINED
SYSTEMIC VENOUSCONGESTION
PULMONARY EDEMAPULMONARY EDEMAPULMONARY EDEMAPULMONARY EDEMA
FORWARD FAILUREFORWARD FAILUREFORWARD FAILUREFORWARD FAILURE BACKWARD FAILUREBACKWARD FAILUREBACKWARD FAILUREBACKWARD FAILURE
Pathological consequences
of congestive heart failure
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Clinical features
Left ventricular failure
Dyspnea
Orthopnea
Paroxysmal nocturnal dyspnea
Right ventricular failure
Systemic venous congestion
Distended neck veins
Enlarged tender liver
Soft tissue edema
Types of heart disease
Five categories of disease account
nearly all cardiac mortality
1. Ischemic heart disease (IHD)
2. Hypertensive heart disease (systemicpulmonary)
3. Valvular heart disease
4. Non-ischemic (primary) myocardial dis
5. Congenital heart disease
101
ISCHEMIC HEART DISEASE (IHD)
Definition
Generic term for imbalance
between myocardial need for and
supply of oxygenated blood
Epidemiology
Leading cause of death for both menwomen in the US and other industria
nations
Each year nearly half a million Ameridie of IHD.
May affect any age, but most commo
older individuals
Males > females until the ninth decad
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IHD - syndromes
1. Various forms of angina pectoris (chest pain)
2. Acute myocardial infarction (MI)
3. Sudden cardiac death
4. Chronic ischemic heart disease with congestiveheart failure
Acute coronary syndromes
MI Unstable angina
Sudden cardiac death
ETIOLOGY
Atherosclerosis 90% of cases
Other causes of ischemia
Anemia - hypoxemia
Lowered systemic blood pressure - Sho
Increased cardiac demand hypertrophexercise
Vasculitis (Kawasaki, PAN)
Aortic dissection
105
ETIOPATHOGENESIS
Complex dynamic interaction between
the following factors
1. Fixed coronary obstruction
2. Acute plaque changes
3. Coronary intraluminal thrombosis
4. vasoconstriction
Role of Acute Plaque Chan
Disruption of plaque an inability of
plaque to with stand imposed mechastresses
Most often the initiating event
Changes in plaque morphology inclu
Hemorrhage
Rupture or fissuring
Erosion or ulceration
107
Vulnerable or Unstable Plaque
Characterized by
Moderately stenoticplaque (50-75%)
Thinner fibrous cap
A core rich in lipid,macrophages and T-cells
less evidence ofsmooth muscleproliferation
Markedly eccentric(not uniform aroundthe vesselcircumference)
METALLOPROTEINASES
T-cells activate
Role of Coronary Artery Thromb
Plaque rupture, erosion or ulceration exof thrombogenic lipid and subendothelialcollagen
Platelet aggregation, thrombin generation thrombus formation.
If the vessel is completely occluded MI
Incomplete obstruction Unstable angina or arrhythmias sudden card
death
Embolization to distal branches - micro infarct
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Role of vasoconstriction
Increases mechanical forces that can contrib-ute to plaque rupture
Stimulated by
Locally released platelet contents Thromboxane A2
Impaired secretion of EDRF Nitric oxide (NO)relative to contracting factors (Endothelin)
Increased adrenergic activity
Smoking
Four syndromes
Angina pectoris (AP)
Myocardial infarction (MI)
Chronic ischemic heart disease
Sudden cardiac death (SCD)
Consequences
111
ANGINA PECTORIS
Intermittent chest pain or discomdue to transient, reversible myocdial ischemia, not quite infarction
No elevation in cardiac enzymes
Three types Typical (stable) angina pectoris
Prinzmetal (variant) angina
Unstable angina pectoris
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Responds to vasodilatorsResponds to vasodilatorsieved by rest, vasodilators
Harbinger of subsequent MI pre-infarction angina
n - crushing or squeezingbsternal, may radiate down
left arm
Induced by disruption of
plaque with superimposed
thrombosis and possibly
vasospasm
Cause and mechanism not
clear
May be due to coronary artery
spasm
e to critical stenosis -
uction of coronary
fusion due to fixed
nosis
Associated with ST segment
elevation, indicative of
transmural ischemia
sociated with increased
mand -physical activity,
otional excitement
Pain that occurs with
progressively increasing
frequency and is precipitated
with progressively less effort,often occurs at rest, and
tends to be of more prolonged
duration.
Occurs at rest, awakens the
patient from sleep
st common form
UNSTABLE (CRESCENDO)PRINZMETAL VARIANT
ANGINA
(~50-75% occlusion)ANGINA
ABLE (TYPICAL) ANGINA
(~75% occlusion) MYOCARDIAL INFARCTIO(HEART ATTACK)
Indicates the development of an area ofmyocardial necrosis caused by local ische
About 1.5 million individuals in the US sufacute MI annually, and approximately oneof them die.
At least half of them die before they reachhospital.
Decreasing incidence since the early 1970
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Age Occurs at any age
Incidence increases with increasingage
Gender Males 4 5 : 1 ages 45 54
Males 2 : 1 up to age 80
No difference > age 80
Epidemiology MAJOR CONTRIBUTING FACTO
Hypercholesterolemia
Smoking
Hypertension
Diabetes mellitus
CRP: Good marker for risk of MI
117
Pathogenesis
Coronary artery occlusion 90% of cases Disruption of plaque 2/3rd are < 50% stenotic & 85%
have < 70% stenosis.
Remaining 10% of cases Vasospasm isolated, intense and relatively
prolonged with or without coronary atherosclerosis
Emboli from the left sided mural thrombus
Unexplained ? Disease of small intramural coronary vessels
? Hematological abnormalities like hemoglobinopathies
Myocardial response
Coronary arteryobstruction loss ofcritical blood supply tothe myocardium
Induces profoundfunctional,biochemical, andmorphologicalconsequences > 1 hrMicrovascular
injury
20-40 Irreversible cellinjury
ATP reduced
- To 50% of normal
- To 10% of normal
< 2 miLoss ofcontractility
SeconOnset of ATPdepletion
TimeFeature
119
CCCOOORRROOONNNAAARRRYYY CCCIIIRRRCCCUUULLLAAATTTIIIOOONNN AAANNNDDD TTTHHHEEE LLLOOOCCCAAATTTIIIOOONNN OOOFFF IIINNNFFFSSS AAARRRCCCTTT
Progression ofmyocardial
necrosis after coronaryartery occlusion
Begins insubendocardial region
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Morphology
The essential sequence of events in MI is
Coagulation necrosis followed by
Inflammation, resorption of necroticmyocardium followed by
Formation of granulation tissue and finally
Organization of granulation tissue to form a
collagen rich scar tissue
collagen cFirm, grey; scarring complete2-8 weeks
Active granulatiRed-gray depressed border10-14 days
Early coagulatioDark Mottling (Occasional)4-12 hr
LM FEATUGROSS FEATURESTIME
Dense collagenScarring complete2 months +
Well developed
Extensive macr
Maximum softening
(no myocytes/no collagen)
7-10 days
Macrophages a
Breakdown of m
Central softening (Yellow/tan)
Hyperemic border
3-7 days
Heavy neutrophMottling (Yellow/tan center)1-3 days
Early neutrophi
Coagulation ne
Contraction ban
Dark Mottling12-24 hr
Wavy fibersNo change1/2-4 hr
No changeNo change0-30 min
123
Contractile dysfunction cardiogenic shockArrhythmiasPapillary muscle dysfunctionExternal rupture of the infarct(3-7 days) Ventricular free wall Ventricular septum
Mural thrombiVentricular aneurysms(after fibrosis, 2+ months)
Pericarditis Acute fibrinous or fibro hemorrhagic
Autoimmune Dresslers syndrome
COMPLICATIONS Clinical features
Severe, crushing, substernal chest pwhich may radiate to neck, jaw, epigashoulder, or left arm
Levines sign (clenched fist over ches
Pain lasts several hours to days and significantly relieved by vasodilators
Diaphoresis, dyspnea, rapid weake p
Silent MI
elderly patients underlying Diabetes mellitus/ Hypertens
125
Clinical course
25% sudden death (SCD) vast majority of
deaths occurring before hospitalizationPatients reaching hospital alive
10 20% uncomplicated course
80 90% complicated but not necessarilyfatal course
Complicated course Cardiac arrhythmias 75 95%
LVF with pulmonary edema 60%
Cardiogenic shock 10%
Rupture of myocardium 4 8 %
Diagnosis of MI
Myocardial infarction is diagnosed wblood levels of sensitive and specificbiomarkers, such as cardiac troponin
the MB fraction of creatine kinase (CMB), are increased in the clinical sett
acute ischemia
JACC, vol 36, No. 3, 200
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Electrocardiographic abnormalities
segmentevation
wave
ersion ofwave
Cardiac Injury Markers
Creatine Kinase isoenzymes (CK-M
Troponins (I and T)
LD (lactate dehydrogenase, LDH)
Myoglobin
129
Troponin I and TTroponins are most sensitive and specific
Regulate calcium-mediated muscle contraction
Normally not detectable in circulation
Troponin I (TnI) commonly measured clinically
Rise in 2-4 hours
Peak at 48 hours
Persists for 7-10 days
Creatine Kinase
CK is a dimer composed of M and B
subunits: MM, MB, BB
CK-MB: most specific for heart, trace
amount in skeletal muscle
Rises within 2-4 hours of MI
Peaks at 24 hours
Disappears by 72 hours
131
Cardiac Injury Markers
Early MI, TnI and CK-MB with similar sensitivity
Troponin is most specific for cardiac injury
Troponin STAYS elevated long after CK is gone
Both elevate sooner in reperfused patients
2 days of unchanged TnI and CK-MB r/o AMI
Intervention
Primary prevention
Thrombolysis
Angioplasty
Coronary bypass graft (CABG)
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Chronic ischemic heart disease Insidious onset of congestive heart failure
in patients who have past episodes of MIor anginal attacks
Cardiac decompensation owing toexhaustion of the compensatoryhypertrophy of non- infarcted viablemyocardium or severe coronary
obstructive disease leading to diffusemyocardial dysfunction Dilation of all 4 heart chambers (may
be difficult to distinguish from dilatedcardiomyopathy
Sudden Cardiac Death
Unexpected death from cardiac causearly (usually within 1 hour) after orwithout the onset of symptoms
Causes IHD most common cause
Mitral valve prolapse
Aortic valve stenosis Dilated or hypertrophic cardiomyopathy
Myocarditis
135
Ultimate mechanism of SCD is a lethal
arrhythmia
Morphology
80-90% of cases critical stenosis of one ormore coronary arteries, high grade stenosisassociated with acute plaque disruption
10-20% - non atherosclerotic in origin
Hypertensive heart diseas
Presence of left ventricular hypertrop
an individual with a history of hyperteand in whom other causes of hypertr
have been excluded
137
Pathogenesis
Stimulus sustained pressure overload
Growth factorsLocal mechanical effects
Changes in genes controlling expression of actin, myosin
Hypertrophy
Morphology
Concentric hypertrophy of left ventricsymmetric, circumferential pattern
Long standing cases right ventricul
hypertrophy and dilation
Histologically enlarged myocytescontaining large, hyperchromatic,
rectangular box-car shaped nuclei
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Clinical features
Early stages asymptomatic
Angina pectoris
Signs and symptoms of heart failure withprogression
Cerebrovascular accidents (stroke)
Sudden cardiac death
Pulmonary heart Disease(Cor Pulmonale)
Disease of right sided cardiac chamb
secondary to pulmonary parenchymapulmonary vascular diseases
Excluded from this definition are
Pulmonary hypertension due to left heafailure
Pulmonary hypertension due to congenheart disease
141
Acute cor pulmonale
Pulmonary embolism causing sudden
increase in burden on the right heart
Right ventricle is dilated but nohypertrophy
Possible cause - Saddle embolus
Valvular Heart Disease
Congenital
Acquired
Valvular involvement by disease causes
Stenosis failure of a valve to open completethereby impeding forward flow
Incompetence, regurgitation, or insufficiency of a valve to close completely, thereby allowinreversed flow
Abnormalities of flow often produce abnor
heart sounds known as murmurs
143ocarditis
Marfan syndromeated cardiomyopathy
Syphilitic aortitisective endocarditis
Degenerative aortic dilation
hypertension
eumatic heart disease
Rheumatic heart diseasexomatous degeneration (mitral
ve prolapse
AORTIC REGURGITATIONTRAL REGURGITATION
Calcification of congenitally deformed
valve
Senile calcific aortic stenosis
Rheumatic heart diseaseeumatic heart disease
AORTIC STENOSISTRAL STENOSIS
Aortic valve diseasetral valve diseaseRheumatic fever
Acute, immunologically mediated, multisysinflammatory disease that follows an episorheumatogenic group A streptococcalpharyngitis after an interval of few weeks.
Occurs in only about 3% of patients with gstreptococcal pharyngitis
Mainly a disease of third world countries acrowded, economically depressed areas owestern world
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Pathogenesis
Exact pathogenesis is not known
Hypersensitivity reaction induced by group Astreptococci
Antibodies directed against the M proteins ofgroup A streptococci cross react with normalproteins present in heart, joints, and othertissues
Evidence in support of this hypothesis Streptococci are absent from the lesions
Symptoms do not develop until 2-3 weeks afterinfection
T-cells activated
by streptococcal
antigensB-cells produce anti-streptococcal
antibodies
Antibodies and T-cells cross rea
Cardiac sarcolemma and valvular gly
147
Morphology- Acute rheumatic fever
Acute rheumatic carditis inflammatory changesin all three layers of the heart Pancarditis
Myocarditis - Hallmark is the presence of Aschoffbodies within connective tissue of the heart.
Endocarditis edematous and thickened valveswith foci of fibrinoid necrosis
Pericarditis fibrinous pericarditis
Arthritis chronic inflammatory infiltrand edema in the joints and periartic
soft tissues.
arthritis is self limited and does not causchronic deformities
Erythema marginatum maculopapurash
Skin nodules contain focal lesions t
are essentially large Aschoff bodies
149
Chronic rheumatic heart disease
Chronic Mitral valvulitis Conspicuous irregular thickening and calcification of
the leaflets, often with fusion of the commissuresand the chordae tendineae
Chronic aortic valvulitis cusps are thickened, firm,and adherent to each other valve orifice is reduced torigid, triangular channel
Clinical features
Acute rheumatic fever Occurs anywhere from 10 days to 6 we
after an episode of group A streptococcpharyngitis
Peak incidence 5-15 years
Arthritis migratory, large joints
Carditis pericardial friction rub, weak sounds, congestive heart failure
Stenosis of mitral valve, most common characteristic complication
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Clinical features
Chronic rheumatic carditis
Valvulitis - murmurs
Cardiac hypertrophy and dilation
Congestive heart failure
Arrhythmias
Infective endocarditis
Diagnosis Jones criteria
Major manifestations (ACCNE) Migratory polyarthritisof large joints Pancarditis Sydenham chorea involuntary, purposeless, rapid move
Subcutaneous nodules Erythemamarginatum of skin
Minor manifestations Fever, Arthralgia, and elevated
Presence of either of the 2 major manifestations major and 2 minor manifestations
+Evidence of preceding streptococcal infection in tof elevated serum ASO titers or positive streptocthroat culture
153
Calcific aortic stenosis
Narrowing of the aortic valve lumen as aresult of deposition of calcium in the cuspsand the valve ring.
It can occur in Elderly patients as a degenerative process.
90% of the patients are > 65 years of age
Congenital bicuspid aortic valve(40-65 years)
Aortic valve scarred as a result of rheumaticfever(5-15 years)
Mitral valve prolapse
One of the most common cardiac dis
occurring in 3-5% of the general adupopulation.
Most cases are discovered between ages of 20 and 40 years
More common in females (7x)
May arise as a complication of Marfa
syndrome
155
Morphology
Soft and enlarged mitral valve cusps ballooning of the valve leaflets into the left
atrium during systole
The chordae tendineae which are often
elongated and fragile, may rupture in
severe cases
The mitral annulus may be dilated
Clinical features
Most patients are asymptomatic Palpitations
Fatigue or atypical chest pain
Mid-systolic click (when valve prolap
Severe complications in about 3 % o Mitral regurgitation and congestive hea
failure
Infective endocarditis
Ventricular arrhythmias
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Infective endocarditis
Infection of the cardiac valves or mural
surface of the endocardium resulting in theformation of adherent, bulky mass of
thrombotic debris and organisms termedvegetations
Classification
Clinical
Acute endocarditis destructive fulminant infrequently of a previously normal heart valve whighly virulent organism, that leads to death wdays to weeks of more than 50% of patients dantibiotics and surgery.
Sub acute endocarditis organisms of low vcause infection in a previously abnormal heart
particularly on deformed valves. Most patientsrecover with appropriate therapy.
Etiological Bacterial, Fungal, Rickettsial
159
Predisposing factorsPreexisting cardiac abnormality
Mitral valve prolapse most common factor
Chronic rheumatic valvulitis
Degenerative calcific aortic stenosis
Prosthetic heart valves 10-20% of cases
ntravenous drug abuse (Tricuspid valve!)
Transient bacteremia dental procedures,
urinary catheterization, endoscopy.
Pathogenesis
Hemodynamic factors Abnormal blood flow across a damaged
Endothelial injury
Focal deposition of platelets and fibrin
Adherence properties of microorgani Fibronectin
Adhesion factors - polysaccharides
161
Causative organisms Native valve
Most common (50-60%) Streptococcus viridans ; deformed
valves Staph. aureus (10-20%) previously healthy or deformed valves
HACEK GROUP Haemophilus, Actinobacillus,Cardiobacterium, Eikenella, Kingella
Prosthetic valve Staph. Epidermidis
Gram negative bacilli
fungi
Intravenous drug abuse Staph. Aureus most common
Streptococci
Gram negative bacilli
Fungi
Morphology
Mitral and aortic most commonly involved
Tricuspid valve I.V. drug abuse Acute bacterial endocarditis Gross
Bulky, friable vegetations that may obstruct thorifice
Rapid destruction of the valves rupture of thleaflets, chordae tendineae, papillary muscles
Ring abscess abscesses in perivalvularmyocardium
Microscopy Large number of organisms mixed with fibrin a
blood cells Minimal inflammatory response
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Subacute endocarditis
Gross
Vegetations are less friable
Associated with less valve destruction
Ring abscesses uncommon
Microscopy
Presence of granulation tissue
Fibrosis, calcification
Chronic inflammatory infiltrate
Clinical features
Acute bacterial endocarditis
High grade fever with chills
Features of septicemia
Subacute bacterial endocarditis
Low grade fever; malaise
Weight loss
Changing cardiac murmurs
Splenomegaly
Clubbing of fingers
165
Glomerulonephritis
Immune complex formationase of bacterial
en
CNS emboli and stroke,myocardial infarction, splenic or
kidney infarcts, mycotic aneurysmSplinter hemorrhagesnailsJaneway lesions (palms/soles)Roth spotsretinal hemorrhageOsler nodessubQ nodules inpulp of digits
Peripheral emboli
mentation
Congestive heart failure
Heart blocks (1, 2or 3)
Valvular insufficiency
AV conductionabnormalities
ue destruction
Joint, bone, organ diseaseSeeding of distant sitesConstitutional symptomsCytokine release
stent bacteremia
Host ConsequenceComplicationonsequence ofVegetation Diagnosis
Repeated blood cultures for both aer
and anaerobic organisms
Treatment
Difficult infection to eradicate avasculnature of the valves
Antibacterial therapy
167
Nonbacterial thromboticEndocarditis
Characterized by the presence of sterilevegetations
Pathogenesis incompletely understood Endothelial abnormalities
Hypercoagulable states
Adenocarcinomas(pancreatic & other abdominal)
Usually asymptomatic
Embolization and infective endocarditis possible complications
Libman- Sacks endocardi
Characterized by presence of sterilevegetations on the cardiac valves inpatients of SLE
No special predilection for the lines oclosure (vegetations on both sides)
Vegetations comprised of fibrin & WB
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Myocarditis
Generalized inflammation of the myocar-dium associated with necrosis anddegeneration of myocytes.
The inflammatory process plays a primary
role in the development of myocardial
injury.
Major causes of Myocardi
Transplant rejectionBacteria c. diphtheriae
Helminthes trichinosis
Protozoa chagasdisease, toxoplasmosis
Drugs sulfonamidesFungi aspergillus,candida
SLERickettsia typhus fever
Giant cell myoPost streptococcal rheumatic fever
Chlamydia- c. psittaci
SarcoidosisPost viralViral coxsackievirus,CMV, ECHO, HIV
UnknoImmune-mediated
Infections
171
Viral Myocarditis
Most common cause of myocarditis in US
Coxsackie A and B and other enter-
oviruses account for most cases
Pathogenesis
Direct viral cytotoxicity
Cell mediated immune reactions against
infected myocytes Diffuse lymphocyte infiltrate with patchynecrosisinfected myocyte
Clinical features
Most cases self limiting disease
Flu like symptoms viral myocarditis
Complications
Acute heart failure giant cell myocarditis
Chronic congestive heart failure viral myoca
Arrhythmias ventricular arrhythmias mostdangerous
173
Cardiomyopathy
Primary disease of the myocardium ex-cluding myocardial diseases caused byischemia, hypertension, valvular lesions,congenital anomalies, or inflammatorydisorders.
Three categories Dilated cardiomyopathy
Hypertrophic cardiomyopathy
Restrictive cardiomyopathy
Dilated Cardiomyopathy
Most common type of cardiomyopath(90%)
Characterized by
Progressive cardiac hypertrophy
Dilation(of all 4 chambers)
Contractile (systolic) dysfunction
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EtiopathogenesisIdiopathic unknown, majority of cases
Genetic mutations 20-30% of cases Dystrophin gene on X- chromosome
Mitochondrial genes abnormal oxidative phosphorylation
Alcohol direct cytotoxicity
Viral myocarditis
Nutritional disturbances thiamine deficiency,chronic anemia,
Pregnancy associated - peripartum cardiomyopathy pregnancy associated hypertension (reversible)
Nutritional disturbances
Clinical features
Fundamental defect is ineffectivecontraction
Ejection fraction is < 25% (normal 5065%)
20-60 year most common
Progressive congestive heart failure
refractory to therapy Death usually occurs due to progress
cardiac failure or arrhythmias
177
Hypertrophic cardiomyopathy
Characterized by
Myocardial hypertrophy
Abnormal diastolic filling
Intermittent left ventricular outflow obstructionin one third of cases
Primarily a diastolic rather than systolicdysfunction
Pathogenesis
Familial autosomal dominant conditio
50% of cases.
Occurs due to genetic defect in any o
following genes
-myosin heavy chain most common
Cardiac troponin T
-tropomyosin
Myosin-binding protein C
179
Clinical features
Exertional dyspnea
Angina or MI
Sudden death due to arrhythmias(especially in atheletes)
Restrictive Cardiomyopath(Decreased compliance)
Least common type of cardiomyopat Etiology
Endomyocardial fibrosis most commocause
Lofflers syndrome
Radiation fibrosis
Amyloidosis
Hemochromatosis
Metastatic tumors Sarcoidosis
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Congenital Heart Disease
Abnormalities of the heart or great vessels
that are present from birth
Most common form of heart disease
among children
1% of live births, higher in prematureinfants and stillborns
Incidence increased due to increased
diagnostic sensitivity
Etiology and pathogenes
Only 10% well-defined environmental or ginfluence
Trisomy 13,15,18,21, and Turner syndrom Trisomy 21 the most common known genetic c
Defect in sibling or parent the greatest risk
Environmental factors (congenital rubella)
183
Frequency of cardiacmalformations
1%Tricuspid atresia
1%Truncus Arteriosus
4%Transposition of the Great Vessels
5%Tetralogy of Fallot
4%Aortic stenosis
5%Coarctation of the Aorta
8%Pulmonary Stenosis
10%Atrial Septal Defect
7%Patent Ductus Arteriosus
42%Ventricular Septal Defect
% of Congenital
Heart Disease
Malformation
Malformation categories Based on flow of blood
Left-to-right shunt
Right-to-left shunt
Obstruction
Based on cyanosis
Cyanotic from birth
Cyanotic later in life
Not cyanotic
185
NormalFetal circulation
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Right-to-left Shunt:Cyanotic heart defects
At birth: The Ts (Tetralogy of Fallot,
Truncus Arteriosus, Tricuspid Atresia,Total Anomalous Pulmonary Venous
Connection, Transposition of the GreatVessels)
After birth, when right-sided pressures
increase: Atrial Septal Defect, VentricularSeptal Defect, Patent Ductus Arteriosus
Tetralogy of Fallot
Most common form of cyanotic cong
heart disease
The four features of tetralogy are
Ventricular septal defect
Pulmonary stenosis
overriding aorta
right ventricular hypertrophy
189
Clinical features
Usually present by 6 months
Dyspnea on exertion, cyanosis
Polycythemia cerebral thrombosis
Infective endocarditis
Pulmonary vasculature decreased
Associated with Downs Syndrome
191
Transposition of the Great Arteries
Aorta off RV andPulmonary artery offLV
AV connectionsnormal
Incompatible withpost natal life, unlessa shunt (VSD or PDA)is present
Truncus Arteriosus
Failure of partitioning of embryologictruncus into aorta and pulmonary arte
Single great artery gets blood from b
ventricles
Underlying VSD
Blood from both ventricles mixes
Cyanotic
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193
Truncus Arteriosus Left-to right shunts: The D
Atrial Septal Defect
Ventricular Septal Defect
Patent Ductus Arteriosus
195
Remember
Ds are red (acyanotic, left-to-right shunt) and Ts are blue(cyanotic, right-to-left shunt).
Os
S
197
Ventricular Septal Defect
Most common congenital heart lesion Associated with Trisomy 21 (DS), 13, 18 Most commonly (90%) membranous Most VSDs close spontaneously in childhood
Clinical features: Pulmonary hypertension; CHF,pansystolic murmur,
Shunt reversal leads to cyanosis -EISENMENGER COMPLEX
Atrial Septal Defect
10% of CHD
Age at presentation variable, may be
asymptomatic into adult
Most common congenital cardiac
malformation diagnosed in adults
Pulmonary vascularity increased if sigleft-to-right shunt
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e typese typese typese types
Ostium secundumOstium secundum Ostium secundumOstium secundum15% Ostium primum15% Ostium primum15% Ostium primum15% Ostium primum
10% Sinus venosus10% Sinus venosus10% Sinus venosus10% Sinus venosus
Patent Ductus Arteriosus
7% of cardiac malformations
90% isolated anomaly
Females more common than males
Continuous, machinery-like murmu
May need to keep open with prostag
E if associated with other malformatio Shunt may reverse leading to cyanos
201
PDA Pathology
Connect left pulmonary artery to aortic
arch
Closes with high oxygen tension
Higher incidence in maternal rubella
infection
Associated with polycythemia
Patent Ductus Arteriosus
203
Obstructive Congenital Anomalies
Coarctation of the Aorta
Pulmonary Stenosis and Atresia
Aortic Stenosis and Atresia (HypoplasticLeft Heart Syndrome)
Coarctation of the Aorta
Narrowed aortic lumen
Associated with Turners Syndrome
50% cases isolated cardiac anoma
Remaining cases associated with PVSD, ASD
Two types
Preductal (infantile)
Postductal (adult)
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THOGENESIS
Clinical features
Preductal - Presents in infancy as Congestive heart failure Selective cyanosis of lower extremities Femoral pulses are weaker than those of the u
extremities
Postductal presents in older children and no selective cyanosis is seen Hypertension of the upper extremities
Blood pressure is low and pulses are weak in extremities Notching of ribs due to collaterals Intermittent claudication arterial insufficiency
207
TUMORS OF THE HEART
Primary tumors are rare
Most common are metastatic neoplasms
Clinical presentation
Sudden onset of severe, rapidly progressiveheart failure without apparent cause and/orarrhythmia
Silent till impair function
Cardiac Myxomas
Most common primary tumor
Female preponderance
Age 30 60 years
Often calcify and can, at times, bseen on X- ray
209
Morphology
Gross lobulated pedunculated mass
Most common location - Left atrium
Histologically multinucleated
stellate cells suspended in anedematous mucopolysaccharide rich
stroma
Clinical features
Most are asymptomatic
Some may fragment and embolize
Ball-valve obstruction of atrioventricuvalve syncopal episodes, sudden d
Associated with diastolic murmur
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Secondary Tumors
Direct extension of lung cancer
Breast
Lymphoma
Malignant melanoma
Pericardial diseases
Effusions - Serous
Congestive heart failure Hypoalbuminemia
Serosanguineous Trauma malignancy
Chylous mediastinal lymphatic obstructi
Hemopericardium cardiac tamponade Ruptured aortic aneurysm or myocardial infarc Penetrating traumatic injury
213
Pericarditis
Primary uncommon; usually infectious in
origin; virus most commonly
Secondary acute MI, cardiac surgery,
radiation, rheumatic fever, SLE,
Dresslers Syndrome
Uremia most common systemic disorderassociated with pericarditis
Clinical features
Chest pain; worsens on reclining
High-pitched friction rub
Cardiac tamponade
Faint distant heart sounds
Distended neck veins
Declining cardiac output
Shock
Morphology
Acute pericarditis fibrinous pericarditis
Chronic pericarditis
Delicate adhesions to dense, fibrotic scarsthat obliterate the pericardial space
Extreme cases heart is completely encasedith d ti t d