•
• The Blood VesselsThe Blood Vessels •
• The HeartThe Heart •
• The LungThe Lung
BLOOD VESSELS BLOOD VESSELS HEMOSTASIS and THROMBOSIS:
HEMOSTASIS and THROMBOSIS:
•
• Hemostasis:Hemostasis:The arrest of hemorrhage as a response to vascular injury.The arrest of hemorrhage as a response to vascular injury. •
• BLOOD COAGULATIONBLOOD COAGULATION
o
o Intrinsic PathwayIntrinsic Pathway: Factor XII ---> Factor XI ---> Factor IX: Factor XII ---> Factor XI ---> Factor IX o
o Extrinsic PathwayExtrinsic Pathway:: Tissue Factor (Thromboplastin)Tissue Factor (Thromboplastin) ---> Factor VII + Ca---> Factor VII + Ca+2+2 o
o Common PathwayCommon Pathway: Factor X ---> (Prothrombin ---> Thrombin) ---> (Fibrinogen ---> Fibrin): Factor X ---> (Prothrombin ---> Thrombin) ---> (Fibrinogen ---> Fibrin)
•
• PLATELET AGGREGATION:PLATELET AGGREGATION:
o
o Von Willebrand FactorVon Willebrand Factorfrom endothelial cells enhances aggregation.from endothelial cells enhances aggregation. o
o Thromboxane AThromboxane A22(TXA(TXA22))enhances aggregation.enhances aggregation.
•
• ENDOTHELIAL FACTORS: Injury to the endothelium is the most important precipitating event of thrombosis.ENDOTHELIAL FACTORS: Injury to the endothelium is the most important precipitating event of thrombosis.
o
o Function of endothelial cells:Function of endothelial cells:
Permeability barrier Permeability barrier
Vasoactive factors:Vasoactive factors: NONO
Anti-thrombotic factors:Anti-thrombotic factors: Prostacyclin (PGIProstacyclin (PGI22))
Clotting Factors:Clotting Factors:
Factor VIIIFactor VIII
Von Willebrand FactorVon Willebrand Factor
Anti-Coagulant Factors:Anti-Coagulant Factors: ThrombomodulinThrombomodulin
Fibrinolytic Agents:Fibrinolytic Agents: TPATPA
Plasminogen Activator Inhibitor (PAI)Plasminogen Activator Inhibitor (PAI)inhibits fibrinolysisinhibits fibrinolysis
Inflammatory Mediators: IL-1, Cell adhesion moleculesInflammatory Mediators: IL-1, Cell adhesion molecules
Growth Factors: CSF, FGF, PDGFGrowth Factors: CSF, FGF, PDGF •
• CLOT LYSIS: (Plasminogen ---> Plasmin) ---> (Fibrin ---> Fibrin split products)CLOT LYSIS: (Plasminogen ---> Plasmin) ---> (Fibrin ---> Fibrin split products)
o
o Thrombosis itself results in production of TPA, which converts plasminogen to plasmin to effectThrombosis itself results in production of TPA, which converts plasminogen to plasmin to effect
fibrinolysis. fibrinolysis.
ATHEROSCLEROSIS: Progressive accumulation within the intima of smooth muscle cells and lipids. ATHEROSCLEROSIS: Progressive accumulation within the intima of smooth muscle cells and lipids.
•
• PATHOGENESIS and PATHOLOGY:PATHOGENESIS and PATHOLOGY:
o
o Progression:Progression:
Early on: Proliferation of smooth muscle cells and accumulation of lipid.Early on: Proliferation of smooth muscle cells and accumulation of lipid.
Smooth muscle cellsSmooth muscle cells as well as lipid is required for the atheroma to form.as well as lipid is required for the atheroma to form.
Later: Infiltration of macrophages, lymphocytes, and connective tissue.Later: Infiltration of macrophages, lymphocytes, and connective tissue.
Later: Organized thrombus formed, with canals (Later: Organized thrombus formed, with canals (vaso plaquorumvaso plaquorum) going through the lesion.) going through the lesion.
o
o ELEMENTS of ATHEROSCLEROTIC PLAQUE:ELEMENTS of ATHEROSCLEROTIC PLAQUE:
Vascular EndotheliumVascular Endothelium
Arterial Smooth Muscle CellArterial Smooth Muscle Cell
Mononuclear PhagocyteMononuclear Phagocyte
Lymphocytes and NeutrophilsLymphocytes and Neutrophils •
• ATHEROGENIC PROCESSES: The overall process of atherogenesis is a combination of the theories below.ATHEROGENIC PROCESSES: The overall process of atherogenesis is a combination of the theories below.
o
o Insudation Hypothesis:Insudation Hypothesis: Lipids in the atheroma are derived from plasma lipoproteins (LDL) in the blood.Lipids in the atheroma are derived from plasma lipoproteins (LDL) in the blood. o
o Encrustation Hypothesis:Encrustation Hypothesis: Says that small mural thrombi represent the initial event in atherosclerosis.Says that small mural thrombi represent the initial event in atherosclerosis.
We now know that this isn't true. Mural thrombi are not the initial event, but they are critical to theWe now know that this isn't true. Mural thrombi are not the initial event, but they are critical to the later progression of the atheroma, i.e. toward thrombosis.
later progression of the atheroma, i.e. toward thrombosis.
o
o Reaction to Injury Hypothesis:Reaction to Injury Hypothesis:Smooth muscle cells accumulate as a response to injury,Smooth muscle cells accumulate as a response to injury, as a result of as a result of
release of PDGF and other growth factors release of PDGF and other growth factors ..
This theory explains smooth muscle proliferation but not lipid accumulation.This theory explains smooth muscle proliferation but not lipid accumulation.
o
o Monoclonal Hypothesis:Monoclonal Hypothesis:Points to the fact that many plaques contain cells that are mostly monoclonal.Points to the fact that many plaques contain cells that are mostly monoclonal.
Perhaps their proliferation was due to a virus or cell-specific mutagen. Perhaps their proliferation was due to a virus or cell-specific mutagen.
o
o Intimal Cell Mass Hypothesis:Intimal Cell Mass Hypothesis:This is the initial lesion. Accumulation of smooth muscle cells at junctionsThis is the initial lesion. Accumulation of smooth muscle cells at junctions
and branching points of arteries. and branching points of arteries.
o
o Hemodynamic Hypothesis:Hemodynamic Hypothesis: Atheromas tend to occur at locations of turbulence, pressure, and shear forces.Atheromas tend to occur at locations of turbulence, pressure, and shear forces.
Hypertension predisposes to atheromatous formation. Hypertension predisposes to atheromatous formation.
o
o UNIFYING HYPOTHESIS: Likely order of events in AtherogenesisUNIFYING HYPOTHESIS: Likely order of events in Atherogenesis
Intimal cell mass predisposes at branch points.Intimal cell mass predisposes at branch points.
Lipid accumulation occurs.Lipid accumulation occurs.
Lipid Insudation results in cellular injury, leading to accumulation of macrophages and plateletsLipid Insudation results in cellular injury, leading to accumulation of macrophages and platelets
Macrophages and platelets release growth factorsMacrophages and platelets release growth factors
Smooth muscle proliferation and endothelial injury may result in loss of anticoagulant propertiesSmooth muscle proliferation and endothelial injury may result in loss of anticoagulant properties of endothelia, and a thrombus results.
of endothelia, and a thrombus results. •
• MORPHOLOGY:MORPHOLOGY:
o
o INITIAL LESION of ATHEROSCLEROSISINITIAL LESION of ATHEROSCLEROSIS
FATTY STREAKS:FATTY STREAKS: Can be found in young children as well as adults, and not necessarily atCan be found in young children as well as adults, and not necessarily at branch points.
branch points.
INTIMAL CELL MASSES:INTIMAL CELL MASSES:At branch points, may also be the initial lesion.At branch points, may also be the initial lesion.
o
o CHARACTERISTIC LESION of ATHEROSCLEROSIS:CHARACTERISTIC LESION of ATHEROSCLEROSIS: Fibrous Fatty PlaqueFibrous Fatty Plaque
FIBROUS CAP:FIBROUS CAP:Layer of fibrous connective tissue overlying the atheroma. Contains foam cellsLayer of fibrous connective tissue overlying the atheroma. Contains foam cells (macrophages) and smooth muscle cells, as well as fibroblasts.
(macrophages) and smooth muscle cells, as well as fibroblasts.
ATHEROMA:ATHEROMA:Necrotic, lipid-laden center of the lesion. Term can also be used to describe theNecrotic, lipid-laden center of the lesion. Term can also be used to describe the whole lesion.
whole lesion. •
• COMPLICATIONSCOMPLICATIONS
o
o COMPLICATED LESIONS: Changes in structure of vessel itself.COMPLICATED LESIONS: Changes in structure of vessel itself.
Thrombosis:Thrombosis:Damage to endothelial cells leads to loss of anti-coagulant properties (clottingDamage to endothelial cells leads to loss of anti-coagulant properties (clotting inhibitors), leading to Thrombosis.
inhibitors), leading to Thrombosis.
NeovascularizationNeovascularization: Organization of the thrombus: Organization of the thrombus
Thinning of the MediaThinning of the Media: Can lead to aneurysm.: Can lead to aneurysm. CalcificationCalcification UlcerationUlceration o o COMPLICATIONS:COMPLICATIONS:
Acute occlusion leading toAcute occlusion leading to Myocardial InfarctMyocardial Infarct. Usually due to hemorrhage into a plaque, or . Usually due to hemorrhage into a plaque, or thrombosis.
thrombosis.
Chronic narrowing of vascular lumen, causing chronic ischemia and sometimes atrophy to kidney.Chronic narrowing of vascular lumen, causing chronic ischemia and sometimes atrophy to kidney.
AneurysmAneurysmformationformation
Cholesterol EmbolismCholesterol Embolism: Embolism of atheromatous material: Embolism of atheromatous material •
• RISK FACTORS: Any factor with a doubling in the incidence of ischemic heart disease.RISK FACTORS: Any factor with a doubling in the incidence of ischemic heart disease. Atherosclerosis is the most Atherosclerosis is the most common cause of heart disease in the Western Hemisphere
common cause of heart disease in the Western Hemisphere ..
o
o hypertensionhypertension o
o blood cholesterol level blood cholesterol level o
o cigarette smokingcigarette smoking o
o diabetesdiabetes o
o increasing ageincreasing age o
o male sexmale sex o
o physical inactivity physical inactivity o
o stressful life patterns.stressful life patterns.
•
• MECHANISMS of LESION PROGRESSION:MECHANISMS of LESION PROGRESSION:
o
o CytokinesCytokines
PDGF and FGFPDGF and FGF cause proliferation of smooth muscle and endothelial cells.cause proliferation of smooth muscle and endothelial cells.
IFN and TGF-betaIFN and TGF-beta inhibit cell proliferation and thus could account for endothelial cellinhibit cell proliferation and thus could account for endothelial cell discontinuities.
discontinuities.
IL-1 and TNFIL-1 and TNFstimulate activation of PAF, Tissue Factor, and PAI (plasminogen activator stimulate activation of PAF, Tissue Factor, and PAI (plasminogen activator inhibitor) in endothelial cells.
inhibitor) in endothelial cells.
o
o T-LymphocytesT-Lymphocytes o
o Endothelium: Loss of continuity of endothelial layer Endothelium: Loss of continuity of endothelial layer
Increase permeability to lipoproteinsIncrease permeability to lipoproteins
Permit platelet interaction with vessel wall, and subsequent release of growth factorsPermit platelet interaction with vessel wall, and subsequent release of growth factors
Blood may enter the wall, allowing interaction, either through an organized thrombus or through aBlood may enter the wall, allowing interaction, either through an organized thrombus or through a tear or discontinuity in the endothelial surface.
o
o ThrombosisThrombosis
•
• HEREDITARY DISORDERS OF LIPID METABOLISM AND ATHEROSCLEROSISHEREDITARY DISORDERS OF LIPID METABOLISM AND ATHEROSCLEROSIS
o
o FAMILIAL HYPERCHOLESTEROLEMIAFAMILIAL HYPERCHOLESTEROLEMIA: Defect in LDL receptors.: Defect in LDL receptors. o o APO-EAPO-E o o HDL:HDL: o o LIPOPROTEIN(A)LIPOPROTEIN(A)
HYPERTENSIVE VASCULAR DISEASE: HYPERTENSIVE VASCULAR DISEASE:
•
• HYPERTENSIONHYPERTENSION: Defined as systolic blood pressure greater than 160mm, or diastolic blood pressure greater than: Defined as systolic blood pressure greater than 160mm, or diastolic blood pressure greater than 90mm, or both.
90mm, or both.
o
o Subtypes:Subtypes:
BENIGN HYPERTENSION:BENIGN HYPERTENSION:AsymptomaticAsymptomatic
MALIGNANT HYPERTENSION:MALIGNANT HYPERTENSION:Rapidly progressing to end-organ failure.Rapidly progressing to end-organ failure.
MORPHOLOGY: Blood vessels showMORPHOLOGY: Blood vessels show fibrinoid necrosisfibrinoid necrosis or concentric hyperplasia of or concentric hyperplasia of smooth muscle-cells
--smooth muscle-cells -- onion-skinonion-skinchanges).changes).
o
o PATHOGENESISPATHOGENESIS::
ESSENTIAL HYPERTENSIONESSENTIAL HYPERTENSION: Primary hypertension in which there is no single identifiable: Primary hypertension in which there is no single identifiable cause. The majority of cases
cause. The majority of cases
ReninReninfrom kidney is a major player.from kidney is a major player.
Renin converts Angiotensinogen ---> Angiotensin IRenin converts Angiotensinogen ---> Angiotensin I
ACE converts Angiotensin I ---> Angiotensin IIACE converts Angiotensin I ---> Angiotensin II
Angiotensin II effects:Angiotensin II effects:
Vasoconstriction.Vasoconstriction.
Stimulate secrete of Stimulate secrete of aldosteronealdosteronefrom adrenal glands ---> K from adrenal glands ---> K ++ excretion and Na
excretion and Na++retention, fluid retention in kidney ---> higher retention, fluid retention in kidney ---> higher blood volume.
blood volume.
SECONDARY HYPERTENSIONSECONDARY HYPERTENSION: Hypertension secondary to a disease. Minority of cases.: Hypertension secondary to a disease. Minority of cases.
Renal Ischemia:Renal Ischemia: Anything causing ischemia to the kidney (vascular stenosis or Anything causing ischemia to the kidney (vascular stenosis or atherosclerosis of Renal Artery or arterioles) will release renin and probably result in atherosclerosis of Renal Artery or arterioles) will release renin and probably result in hypertension.
hypertension.
Fibromuscular DysplasiaFibromuscular Dysplasiaof Renal Artery is a congenital disorder, withof Renal Artery is a congenital disorder, with
progressive concentric thickening of the Renal Artery. Occurs in young females. progressive concentric thickening of the Renal Artery. Occurs in young females.
Cushing's SyndromeCushing's Syndrome: Primary hypersecretion of cortisol.: Primary hypersecretion of cortisol.
Conn's SyndromeConn's Syndrome: Primary hypersecretion of aldosterone.: Primary hypersecretion of aldosterone.
PheochromocytomaPheochromocytoma: Adrenal medullary tumor.: Adrenal medullary tumor.
Norepinephrine is secreted in spurts, so patient will have wildly fluctuating, Norepinephrine is secreted in spurts, so patient will have wildly fluctuating, paroxysmal elevations in blood pressure.
paroxysmal elevations in blood pressure.
ThyrotoxicosisThyrotoxicosis
RISK FACTORS: Similar as those for atherosclerosis and CAD.RISK FACTORS: Similar as those for atherosclerosis and CAD.
Genetics: often found to be familialGenetics: often found to be familial
DietDiet
Stressful lifestyleStressful lifestyle
ObesityObesity
CLINICAL MANIFESTATIONS: Often asymptomaticCLINICAL MANIFESTATIONS: Often asymptomatic
Early on: Headache, nosebleeds, tinnitus, dizziness, fainting, visual impairment.Early on: Headache, nosebleeds, tinnitus, dizziness, fainting, visual impairment.
Later: End-stage hypertension results in stroke, heart failure (from compensatoryLater: End-stage hypertension results in stroke, heart failure (from compensatory hypertrophy), renal failure
hypertrophy), renal failure •
• ARTERIOSCLEROSISARTERIOSCLEROSIS: Arterial vascular changes characterized by thickening and loss of elasticity of arterial: Arterial vascular changes characterized by thickening and loss of elasticity of arterial walls. It may be seen in patients with chronic hypertension, and, to a lesser degree, as part of the aging process. walls. It may be seen in patients with chronic hypertension, and, to a lesser degree, as part of the aging process.
o
o BENIGN (HYALINE) ARTERIOSCLEROSISBENIGN (HYALINE) ARTERIOSCLEROSIS: Particularly occurs in kidneys. The blood vessel walls: Particularly occurs in kidneys. The blood vessel walls
take on a glassy, "hyaline" appearance. This change reflects mild or "benign" hypertension and are take on a glassy, "hyaline" appearance. This change reflects mild or "benign" hypertension and are particularly seen in kidneys.
particularly seen in kidneys.
o
o MALIGNANT (HYPERPLASTIC) ARTERIOSCLEROSISMALIGNANT (HYPERPLASTIC) ARTERIOSCLEROSIS: This change refers to the concentric rings: This change refers to the concentric rings
of increased connective tissue and smooth muscle giving the arteries an onion-skin appearance. Such of increased connective tissue and smooth muscle giving the arteries an onion-skin appearance. Such changes signify acceleration of the hypertension.
changes signify acceleration of the hypertension.
o
o ARTERIOLOSCLEROSISARTERIOLOSCLEROSIS: Smooth muscle proliferation, thickening, and loss of elasticity of walls: Smooth muscle proliferation, thickening, and loss of elasticity of walls
occurring in the arterioles. occurring in the arterioles.
•
• MONCKEBERG MEDIAL SCLEROSISMONCKEBERG MEDIAL SCLEROSIS: Degenerative calcification of the media of large and medium arteries in: Degenerative calcification of the media of large and medium arteries in old people.
old people.
o
o Usually vessels in the extremities.Usually vessels in the extremities. o
o Usually is benign and subclinical.Usually is benign and subclinical.
VASCULITIS: VASCULITIS:
•
• GENERAL PROPERTIES: Inflammation and necrosis of blood vessels, including arteries, veins and capillaries.GENERAL PROPERTIES: Inflammation and necrosis of blood vessels, including arteries, veins and capillaries. The damage may be due to infectious agents, mechanical trauma, radiation or toxins; often no specific etiologic The damage may be due to infectious agents, mechanical trauma, radiation or toxins; often no specific etiologic factor is identified. The pathogenesis is thought to involve immune mechanisms such as deposition of immune factor is identified. The pathogenesis is thought to involve immune mechanisms such as deposition of immune complexes, direct attack by circulating antibodies etc.
complexes, direct attack by circulating antibodies etc. •
• POLYARTERITIS NODOSAPOLYARTERITIS NODOSA: Systemic vasculitis affecting medium and small muscular arteries.: Systemic vasculitis affecting medium and small muscular arteries.
o
o PATHOLOGY: Multiple organs involved, but the lungs are characteristically not involved. If lungPATHOLOGY: Multiple organs involved, but the lungs are characteristically not involved. If lung
involvement is present, suspect Wegener's Granulomatosis. involvement is present, suspect Wegener's Granulomatosis.
o
o CLINICAL FEATURES:CLINICAL FEATURES:
Condition is associated with Hepatitis-B in about 30% of cases.Condition is associated with Hepatitis-B in about 30% of cases.
Treatment: steroids, cyclophosphamide.Treatment: steroids, cyclophosphamide. •
• HYPERSENSITIVITY ANGIITISHYPERSENSITIVITY ANGIITIS::
o
o PATHOGENESIS: Maybe hypersensitivity to a drug, a bacterial product, or maybe secondary to anPATHOGENESIS: Maybe hypersensitivity to a drug, a bacterial product, or maybe secondary to an
autoimmune disease like SLE. autoimmune disease like SLE.
o
o PATHOLOGY: Affects mainly the small vessels -- arterioles and capillaries. Will see fibrinoid necrosis.PATHOLOGY: Affects mainly the small vessels -- arterioles and capillaries. Will see fibrinoid necrosis.
As opposed to Polyarteritis Nodosa, this disease affects small arteries. Otherwise its similar.As opposed to Polyarteritis Nodosa, this disease affects small arteries. Otherwise its similar.
As opposed to Malignant Hypertension (which can also show petechiae), this disease is associatedAs opposed to Malignant Hypertension (which can also show petechiae), this disease is associated with inflammation whereas malignant hypertension is not.
with inflammation whereas malignant hypertension is not.
o
o CLINICAL:CLINICAL: PetechiaePetechiaewill be present from capillary hemorrhage.will be present from capillary hemorrhage. o
o LEUCO CYTOCLASTIC VASCULITISLEUCO CYTOCLASTIC VASCULITIS: Confined predominantly to the skin and presenting as purpuric: Confined predominantly to the skin and presenting as purpuric
lesions. lesions.
PATHOLOGY: Fibrinoid necrosis of small vessels, extravasated red cells.PATHOLOGY: Fibrinoid necrosis of small vessels, extravasated red cells.
CLINICAL: Clinically presents as purpura.CLINICAL: Clinically presents as purpura. •
• CHURG-STRAUSS SYNDROME, ALLERGIC GRANULOMATOSIS and ANGIITISCHURG-STRAUSS SYNDROME, ALLERGIC GRANULOMATOSIS and ANGIITIS ::
o
o PATHOLOGY: Mainly affects the lungs, it is found in young people, and it is associated with asthma.PATHOLOGY: Mainly affects the lungs, it is found in young people, and it is associated with asthma.
Will find Eosinophilia in the blood.Will find Eosinophilia in the blood. •
• GIANT CELL ARTERITIS, TEMPORAL ARTERITISGIANT CELL ARTERITIS, TEMPORAL ARTERITIS::
o
o PATHOLOGY: Giant cells present in the wall of any of the cranial arteries, usually the Temporal Artery.PATHOLOGY: Giant cells present in the wall of any of the cranial arteries, usually the Temporal Artery.
Thrombus found in lumen of the artery. A granulomatous infiltrate will be found in the artery.Thrombus found in lumen of the artery. A granulomatous infiltrate will be found in the artery.
Giant CellsGiant Cellswill be present in the artery, hence the name./=will be present in the artery, hence the name./=
o
o CLINICAL FEATURES:CLINICAL FEATURES:
Occurs in older population, at least 50 yrs old.Occurs in older population, at least 50 yrs old.
Patient presents with headache and temporal pain. Visual symptoms are common, and blindness isPatient presents with headache and temporal pain. Visual symptoms are common, and blindness is a complication in 50% of cases.
a complication in 50% of cases.
Temporal Artery will feel firm and nodular.Temporal Artery will feel firm and nodular.
Treatment: responds well to steroids.Treatment: responds well to steroids. •
• WEGENER'S GRANULOMATOSISWEGENER'S GRANULOMATOSIS::
o
o PATHOLOGY:PATHOLOGY:
Two key pathological findingsTwo key pathological findings
Systemic vasculitisSystemic vasculitis of small arteries and veins.of small arteries and veins.
GranulomatoGranulomatous inflammation us inflammation of of nose, sinuses, and lung nose, sinuses, and lung ..
Anti Cytoplasmic Nuclear Antibodies (ANCA)Anti Cytoplasmic Nuclear Antibodies (ANCA)are found in serum.are found in serum.
o
o CLINICAL FEATURES: Patient may have persistent sinusitis, pneumonitis, hematuria, proteinuria.CLINICAL FEATURES: Patient may have persistent sinusitis, pneumonitis, hematuria, proteinuria.
•
• TAKAYASU ARTERITISTAKAYASU ARTERITIS: An inflammatory disorder of the aortic arch and its major branches, with localized: An inflammatory disorder of the aortic arch and its major branches, with localized stenosis or occlusion
stenosis or occlusion
o
o PATHOLOGY: Also known asPATHOLOGY: Also known as PULSELESS DISEASE,PULSELESS DISEASE, as the obliteration and thickening of the Aorticas the obliteration and thickening of the Aortic
Arch may result in no pulse in the wrist. Arch may result in no pulse in the wrist.
o
o PATHOGENESIS: An auto-immune basis has been proposed.PATHOGENESIS: An auto-immune basis has been proposed. o
o CLINICAL FEATURES: It is most common in young women, similar to other auto-immune disorders.CLINICAL FEATURES: It is most common in young women, similar to other auto-immune disorders.
•
• KAWASAKI DISEASE (MUCOCUTANEOUS LYMPH NODE KAWASAKI DISEASE (MUCOCUTANEOUS LYMPH NODE SYNDROME)SYNDROME)::
o
o PATHOGENESIS: Thought to be viral etiology.PATHOGENESIS: Thought to be viral etiology. o
Coronary AneurysmsCoronary Aneurysmsare found in 70% of patients -- an unusual finding.are found in 70% of patients -- an unusual finding.
o
o CLINICAL:CLINICAL:
Infancy and early childhoodInfancy and early childhood
Fever, rash, conjunctival and oral lesionsFever, rash, conjunctival and oral lesions
LymphadenitisLymphadenitis •
• THROMBOANGIITIS OBLITERANS (BUERGER'S DISEASE)THROMBOANGIITIS OBLITERANS (BUERGER'S DISEASE): An occlusive, inflammatory disease of the: An occlusive, inflammatory disease of the medium-sized and small arteries in the distal arms and legs.
medium-sized and small arteries in the distal arms and legs.
o
o PATHOLOGY: Microscopically, there is acute inflammation of arteries with thrombosis and obliteration of PATHOLOGY: Microscopically, there is acute inflammation of arteries with thrombosis and obliteration of
lumen. lumen.
o
o CLINICAL FEATURES:CLINICAL FEATURES:
Intermittent ClaudicationIntermittent Claudication(weakness relieved by rest) is seen in legs.(weakness relieved by rest) is seen in legs.
Strongly associated with smoking. Young or middle-aged males.Strongly associated with smoking. Young or middle-aged males. RAYNAUD'S PHENOMENON
RAYNAUD'S PHENOMENON: Intermittent attacks of ischemia of fingers or toes due to intense arterial vasospasm, often: Intermittent attacks of ischemia of fingers or toes due to intense arterial vasospasm, often precipitated by cold or emotional stimuli.
precipitated by cold or emotional stimuli. ANEURYSMS:
ANEURYSMS:
•
• SHAPES OF ANEURYSMS:SHAPES OF ANEURYSMS:
o
o Fusiform:Fusiform:Most common form.Most common form. o
o Saccular:Saccular:Most common form.Most common form. o
o Dissecting:Dissecting:Actually a hematoma, splitting apart of media.Actually a hematoma, splitting apart of media. o
o Arteriovenous Fistula:Arteriovenous Fistula:Direct connection of arterioles to venules, bypassing the tissue and causingDirect connection of arterioles to venules, bypassing the tissue and causing
necrosis. necrosis. •
• ATHEROSCLEROTIC ANEURYSMSATHEROSCLEROTIC ANEURYSMS: Most common type of aneurysm.: Most common type of aneurysm.
o
o PATHOLOGY: Usually found inPATHOLOGY: Usually found in abdominal aortaabdominal aorta.. Lumen may only be slightly enlarged, but you will seeLumen may only be slightly enlarged, but you will see
lots of atherosclerosis and thrombosis. lots of atherosclerosis and thrombosis.
o
o CLINICAL FEATURES: May be asymptomatic, or may be found coincident to some other X-ray, surgery,CLINICAL FEATURES: May be asymptomatic, or may be found coincident to some other X-ray, surgery,
or medical procedure. or medical procedure.
Ruptured AneurysmRuptured Aneurysmis the most dramatic presentation, surgical emergency.is the most dramatic presentation, surgical emergency.
TREATMENT: Put a synthetic graft over the aneurysm.TREATMENT: Put a synthetic graft over the aneurysm. •
• CONGENITAL (BERRY) ANEURYSMSCONGENITAL (BERRY) ANEURYSMS: Usually occurs at a main branching point of the basilar artery.: Usually occurs at a main branching point of the basilar artery.
o
o PATHOLOGY: Fairly small aneurysm, saccular in shape.PATHOLOGY: Fairly small aneurysm, saccular in shape.
•
• DISSECTING ANEURYSMSDISSECTING ANEURYSMS: Not really an aneurysm, actually it's a hematoma in the muscular media of the: Not really an aneurysm, actually it's a hematoma in the muscular media of the Aorta.
Aorta.
o
o PATHOGENESIS: Results from Marfan's Syndrome or from longstanding hypertension in elderly males.PATHOGENESIS: Results from Marfan's Syndrome or from longstanding hypertension in elderly males.
Dissection splits the aortic wall in two. Multiple types have been described.Dissection splits the aortic wall in two. Multiple types have been described.
TYPE-A: Start at Aortic Arch and extend proximally to include the Aortic valves,TYPE-A: Start at Aortic Arch and extend proximally to include the Aortic valves, resulting in hemopericardium. Grave prognosis.
resulting in hemopericardium. Grave prognosis.
TYPE-B: Start Aortic Arch and extend distally, down through the abdominal Aorta.TYPE-B: Start Aortic Arch and extend distally, down through the abdominal Aorta.
Double-Barrel Aorta:Double-Barrel Aorta:Dissection penetrates back into the true Aortic lumen, temporarilyDissection penetrates back into the true Aortic lumen, temporarily relieving the pressure and biding some time.
relieving the pressure and biding some time.
o
o PATHOLOGY:PATHOLOGY: Cystic Medial NecrosisCystic Medial Necrosisis the characteristic finding -- cystic changes in media of aorta.is the characteristic finding -- cystic changes in media of aorta.
Stains positive for mucopolysaccharide.Stains positive for mucopolysaccharide.
o
o CLINICAL FEATURES: Patient describes a severeCLINICAL FEATURES: Patient describes a severe tearingtearingtype of pain.type of pain.
•
• SYPHILITIC ANEURYSMSSYPHILITIC ANEURYSMS: Aneurysms in: Aneurysms in Thoracic AortaThoracic Aortaresulting fromresulting from Obliterative EndarteritisObliterative Endarteritisof theof the vaso vasorum
vaso vasorum supplying the Aorta.supplying the Aorta.
o
o PATHOLOGY:PATHOLOGY: CharacteristicCharacteristic Tree-Bark AppearanceTree-Bark Appearance of linear and patchy scars is seen on the luminalof linear and patchy scars is seen on the luminal
aspect of the aorta. aspect of the aorta.
o
o CLINICAL: Aortic calcification,CLINICAL: Aortic calcification, Aortic Insufficiency,Aortic Insufficiency,andand hemopericardiumhemopericardiumare frequent complications.are frequent complications.
•
• MYCOTIC ANEURYSMS:MYCOTIC ANEURYSMS:These lesions are caused by significant weakening of the blood vessel wall byThese lesions are caused by significant weakening of the blood vessel wall by infection.
VEINS: VEINS:
•
• VARICOSE VEINSVARICOSE VEINS: Enlarged and tortuous blood vessels. Occurs most commonly in the legs.: Enlarged and tortuous blood vessels. Occurs most commonly in the legs.
o
o RISK-FACTORS:RISK-FACTORS:
older ageolder age
female sexfemale sex heredityheredity posture posture obesity.obesity. o
o PATHOLOGY: Dilation and elongation of the veins, incompetence of venous valves.PATHOLOGY: Dilation and elongation of the veins, incompetence of venous valves. o
o SITES:SITES:
Varicose Veins of LegsVaricose Veins of Legs is most common.is most common.
HemorrhoidsHemorrhoids
Esophageal VaricesEsophageal Varices
Varicocele:Varicocele:Varicose veins of pampiniform plexus of scrotum.Varicose veins of pampiniform plexus of scrotum. •
• DEEP VENOUS THROMBOSISDEEP VENOUS THROMBOSIS: Associated with prolonged bed-rest, blood stasis, and reduced cardiac output.: Associated with prolonged bed-rest, blood stasis, and reduced cardiac output. Risk for pulmonary embolism.
Risk for pulmonary embolism.
o
o ThrombophlebitisThrombophlebitis: Inflammation, commonly from a bacterial infection, with secondary thrombosis of : Inflammation, commonly from a bacterial infection, with secondary thrombosis of
deep leg veins. deep leg veins.
o
o PhlebothrombosisPhlebothrombosis: Thrombosis of deep leg veins without initial inflammation.: Thrombosis of deep leg veins without initial inflammation.
LYMPHATIC VESSELS: LYMPHATIC VESSELS:
•
• LYMPHANGIITIS:LYMPHANGIITIS: •
• LYMPHATIC OBSTRUCTIONLYMPHATIC OBSTRUCTION
o o Lymphedema:Lymphedema: o o Lymphangiectasia:Lymphangiectasia: o o Elephantiasis:Elephantiasis: o
o Milroy Disease:Milroy Disease:Inherited form of lymphedema present at birth.Inherited form of lymphedema present at birth.
BENIGN BLOOD-VESSEL TUMORS: BENIGN BLOOD-VESSEL TUMORS:
•
• HEMANGIOMASHEMANGIOMAS: Benign tumors of blood vessels. They don't know whether it's a true neoplasia or just a: Benign tumors of blood vessels. They don't know whether it's a true neoplasia or just a hamartoma.
hamartoma.
o
o Types:Types:
CAPILLARY HEMANGIOMACAPILLARY HEMANGIOMA: Containing capillaries.: Containing capillaries.
Juvenile Hemangioma:Juvenile Hemangioma:
CAVERNOUS HEMANGIOMACAVERNOUS HEMANGIOMA: Containing open vascular spaces.: Containing open vascular spaces.
Multiple Hemangiomatous Syndromes:Multiple Hemangiomatous Syndromes:
o
o CLINICAL: Truly benign and not important, unless they are a problem cosmetically.CLINICAL: Truly benign and not important, unless they are a problem cosmetically.
•
• GRANULOMA PYOGENICUMGRANULOMA PYOGENICUM: Mass of granulation tissue resembling a hemangioma. They are truly benign and: Mass of granulation tissue resembling a hemangioma. They are truly benign and occur after some injury
occur after some injury •
• VASCULAR ECTASIAVASCULAR ECTASIA: Local Dilatation and growth of blood vessels, not a tumor.: Local Dilatation and growth of blood vessels, not a tumor.
o
o Spider AngiomataSpider Angiomatais an example.is an example.
•
• GLOMUS TUMOR (GLOMANGIOMA)GLOMUS TUMOR (GLOMANGIOMA): A benign, painful tumor of the: A benign, painful tumor of the glomus bodyglomus body-- neuromyoarterial-- neuromyoarterial receptor which is sensitive to temperature and regulates arterial flow.
receptor which is sensitive to temperature and regulates arterial flow.
o
o PATHOLOGY: Small, reddish blue spots occur most commonly in the distal fingers and toes.PATHOLOGY: Small, reddish blue spots occur most commonly in the distal fingers and toes.
Histologically, there is a mixture of branching vascular channels and nests of glomus cells.Histologically, there is a mixture of branching vascular channels and nests of glomus cells. •
• HEMANGIOENDOTHELIOMAHEMANGIOENDOTHELIOMA: A vascular tumor composed of endothelial cells, considered to be intermediate: A vascular tumor composed of endothelial cells, considered to be intermediate between benign hemangiomas and frankly malignant angiosarcomas. Histologically, several variants are described, between benign hemangiomas and frankly malignant angiosarcomas. Histologically, several variants are described, based on the predominant cell type - spindle cell, epithelioid etc.
based on the predominant cell type - spindle cell, epithelioid etc.
o
o Epithelioid HemangioendotheliomaEpithelioid Hemangioendothelioma o
o Spindle-Cell HemangioendotheliomaSpindle-Cell Hemangioendothelioma o
o CLINICAL: In general, surgical excision is curative. Rarely do these tumors metastasize.CLINICAL: In general, surgical excision is curative. Rarely do these tumors metastasize.
•
• MULTIPLE HEMANGIOMATOUS SYNDROMESMULTIPLE HEMANGIOMATOUS SYNDROMES: Angiomatous lesions present in two or more tissues.: Angiomatous lesions present in two or more tissues.
o
o Von Hippel-Lindau SyndromeVon Hippel-Lindau Syndrome: Hemangiomas in brain and retina.: Hemangiomas in brain and retina. o
MALIGNANT BLOOD-VESSEL TUMORS: MALIGNANT BLOOD-VESSEL TUMORS:
•
• ANGIOSARCOMAANGIOSARCOMA: Malignant neoplasm arising from blood vessels.: Malignant neoplasm arising from blood vessels.
o
o CARCINOGENS have implicated as causes:CARCINOGENS have implicated as causes:
ThorotrastThorotrast-- radio-opaque dye.-- radio-opaque dye.
PVCPVC
ArsenicArsenic..
o
o PATHOLOGY: Most commonly found on scalp, breast, other soft tissues, or liver.PATHOLOGY: Most commonly found on scalp, breast, other soft tissues, or liver.
Resembles hemangiomas, except the lining endothelial cells are malignant.Resembles hemangiomas, except the lining endothelial cells are malignant.
o
o CLINICAL: Variable prognosis, from indolent to high malignant.CLINICAL: Variable prognosis, from indolent to high malignant. o
o LYMPHEDEMA-ASSOCIATED ANGIOSARCOMA:LYMPHEDEMA-ASSOCIATED ANGIOSARCOMA: Occurs in 1% of cases, some 20 years after anOccurs in 1% of cases, some 20 years after an
axillary-node dissection for breast cancer. Occurs in associated with lymphedema of the upper extremity. axillary-node dissection for breast cancer. Occurs in associated with lymphedema of the upper extremity.
Also known Stewart-Treves Syndrome.Also known Stewart-Treves Syndrome. •
• HEMANGIOPERICYTOMAHEMANGIOPERICYTOMA: Rare malignant neoplasm thought to arise from pericytes, smooth muscle cells: Rare malignant neoplasm thought to arise from pericytes, smooth muscle cells external to the walls of capillaries and arterioles.
external to the walls of capillaries and arterioles. •
• KAPOSI SARCOMAKAPOSI SARCOMA::
o
o PATHOGENESIS: In AIDS patients, caused by infection of PATHOGENESIS: In AIDS patients, caused by infection of HHV-8HHV-8 o
o FOUR TYPESFOUR TYPES
Classical / EuropeanClassical / European: Found in older men of Poland, Italy. Chronic course and rarely fatal.: Found in older men of Poland, Italy. Chronic course and rarely fatal.
AfricanAfrican: Also chronic, more malignant than above, but still not usually fatal.: Also chronic, more malignant than above, but still not usually fatal.
Transplant-associatedTransplant-associated: Immunosuppression, particularly with renal transplants.: Immunosuppression, particularly with renal transplants.
AIDS-ASSOCIATEDAIDS-ASSOCIATED: Immunosuppression.: Immunosuppression.
Found in highest incidence inFound in highest incidence in homosexual at-risk grouphomosexual at-risk group, as compared to other groups,, as compared to other groups, perhaps because of sexual transmission of HHV-8 virus.
perhaps because of sexual transmission of HHV-8 virus.
o
o PATHOLOGY: Two stagesPATHOLOGY: Two stages
Patch Stage:Patch Stage:Early on.Early on.
Plaque, Nodular StagePlaque, Nodular Stage: Later progression.: Later progression. •
• BACILLARY ANGIOMATOSISBACILLARY ANGIOMATOSIS: Vascular infection appearing like Kaposi Sarcoma, and caused by: Vascular infection appearing like Kaposi Sarcoma, and caused by Bartonella Bartonella Henselae
Henselae(same causal agent as Cat Scratch Fever).(same causal agent as Cat Scratch Fever).
o
THE HEART THE HEART CORONARY VESSELS:
CORONARY VESSELS:
•
• LEFT ANTERIOR DESCENDING (LAD) CORONARY ARTERYLEFT ANTERIOR DESCENDING (LAD) CORONARY ARTERY: Most common artery to occlude. Results in: Most common artery to occlude. Results in an
an anterior infarctanterior infarct::
o
o Anterior wall.Anterior wall. o
o Anterior two thirds of septum.Anterior two thirds of septum. o
o Entire apex of heart, circumferentially.Entire apex of heart, circumferentially.
•
• LEFT CIRCUMFLEX LEFT CIRCUMFLEX CORONARY ARTERYCORONARY ARTERY: Occlusion gives you a: Occlusion gives you a posterolateral infarctposterolateral infarct-- posterior, lateral-- posterior, lateral left aspect of heart.
left aspect of heart. •
• RIGHT CORONARY ARTERYRIGHT CORONARY ARTERY: Results in a: Results in a posterior septal infarctposterior septal infarct-- posterior one third of septum, inferior -- posterior one third of septum, inferior aspect, and posterior wall of heart.
aspect, and posterior wall of heart.
o
o Infarction of the Right Ventricle is Infarction of the Right Ventricle is rarerare, because the right side has far less demand for oxygen. Right, because the right side has far less demand for oxygen. Right
Ventricular infarcts are usually extensions of posterior septal infarcts caused by occlusion of the Right Ventricular infarcts are usually extensions of posterior septal infarcts caused by occlusion of the Right Coronary Artery.
Coronary Artery.
MYOCARDIAL HYPERTROPHY and CONGESTIVE HEART FAILURE: MYOCARDIAL HYPERTROPHY and CONGESTIVE HEART FAILURE:
•
• CAUSES of HEART FAILURECAUSES of HEART FAILURE
o
o Pump FailurePump Failure: Failure that is intrinsic to the myocardium.: Failure that is intrinsic to the myocardium.
Two types:Two types:
Systolic Failure:Systolic Failure:Failure to pump blood out of heart. Low ejection fraction.Failure to pump blood out of heart. Low ejection fraction.
Diastolic FailureDiastolic Failure: Failure to distend the heart to fill the ventricles, as in: Failure to distend the heart to fill the ventricles, as in constrictiveconstrictive pericarditis
pericarditis..
Most common reason for pump failure is fromMost common reason for pump failure is from myocardial hypertrophymyocardial hypertrophy, usually secondary to, usually secondary to hypertension. hypertension. MyocarditisMyocarditis CardiomyopathyCardiomyopathy o
o Conduction System FailureConduction System Failure: Secondary to MI: Secondary to MI o
o Valvular Failure: Inflammatory (endocarditis), autoimmune, or congenital.Valvular Failure: Inflammatory (endocarditis), autoimmune, or congenital. o
o Cardiac MalformationsCardiac Malformations: Congenital: Congenital o
o Blood Loss / Obstruction of Blood FlowBlood Loss / Obstruction of Blood Flow: Extracardiac causes. Pulmonary emboli or bleeding.: Extracardiac causes. Pulmonary emboli or bleeding.
•
• HEART'S RESPONSE TO INJURYHEART'S RESPONSE TO INJURY
o
o HYPERTROPHYHYPERTROPHY: Normal heart is 250-350g. myocardial cells can hypertrophy to about 3X size, or about: Normal heart is 250-350g. myocardial cells can hypertrophy to about 3X size, or about
900g. 900g.
Box Car NucleiBox Car Nucleiare the characteristic histological appearance of hypertrophied cells.are the characteristic histological appearance of hypertrophied cells.
o
o DILATATIONDILATATION: Could be caused by pump failure (filled ventricles that can't empty), Aortic Insufficiency,: Could be caused by pump failure (filled ventricles that can't empty), Aortic Insufficiency,
or many other causes. or many other causes.
o
o NECROSISNECROSIS::
Ischemic NecrosisIschemic Necrosis
Contraction Band Necrosis.Contraction Band Necrosis.
o o DEGENERATIONDEGENERATION o o INFLAMMATIONINFLAMMATION o o RESOLUTIONRESOLUTION o o FIBROSISFIBROSIS o
o CALCIFICATION: Dystrophic calcificationCALCIFICATION: Dystrophic calcification
•
• PATHOGENESISPATHOGENESIS
o
o HUMORAL RESPONSESHUMORAL RESPONSES o
o CELLULAR HYPERTROPHYCELLULAR HYPERTROPHY o
o ABNORMAL PROTEIN ISOFORMSABNORMAL PROTEIN ISOFORMS o
o ALTERATIONS in CALCIUM HOMEOSTASISALTERATIONS in CALCIUM HOMEOSTASIS o
o PROTO-ONCOGENESPROTO-ONCOGENES o
o EXTRACELLULAR MATRIXEXTRACELLULAR MATRIX o
o ADRENERGIC DESENSITIZATIONADRENERGIC DESENSITIZATION
•
• PATHOLOGYPATHOLOGY •
CONGENITAL HEART DISEASE: CONGENITAL HEART DISEASE:
•
• HEART EMBRYOLOGY:HEART EMBRYOLOGY:
o
o Openings generally allow blood to pass from right heart to left heart, bypassing the lungs.Openings generally allow blood to pass from right heart to left heart, bypassing the lungs.
FORAMEN OVALEFORAMEN OVALE: An opening at the midpoint of the interatrial septum. It is open during fetal: An opening at the midpoint of the interatrial septum. It is open during fetal life allowing the passage of blood from the right to the left atrium. This natural fetal shunt allows life allowing the passage of blood from the right to the left atrium. This natural fetal shunt allows the blood to bypass the fetal lungs.
the blood to bypass the fetal lungs.
DUCTUS ARTERIOSUSDUCTUS ARTERIOSUS: Fetal blood vessel that connects the pulmonary artery with the aorta: Fetal blood vessel that connects the pulmonary artery with the aorta allowing the oxygenated blood from the fetal pulmonary artery to bypass the lungs and enter allowing the oxygenated blood from the fetal pulmonary artery to bypass the lungs and enter directly into the aorta.
directly into the aorta. •
• INITIAL LEFT-to-RIGHT SHUNT (Late Cyanotic or Non-Cyanotic)INITIAL LEFT-to-RIGHT SHUNT (Late Cyanotic or Non-Cyanotic)
o
o PATHOGENESIS: LATE CYANOSIS is Initial left-to-right shunt will lead to Pulmonary Congestion ---PATHOGENESIS: LATE CYANOSIS is Initial left-to-right shunt will lead to Pulmonary Congestion
---> Right Ventricular Hypertrophy plus
> Right Ventricular Hypertrophy plus pulmonary hypertension.pulmonary hypertension.In the baby, the pulmonary hypertensionIn the baby, the pulmonary hypertension eventually (over months or years) get so bad as to surpass systemic blood pressure yielding a
eventually (over months or years) get so bad as to surpass systemic blood pressure yielding alate Right-to-late Right-to-Left shunt ---> cyanosis
Left shunt ---> cyanosis..
o
o VENTRICULAR SEPTAL DEFECT (ROGER DISEASE)VENTRICULAR SEPTAL DEFECT (ROGER DISEASE): Most commonly diagnosed congenital heart: Most commonly diagnosed congenital heart
disease. disease.
PATHOGENESIS: Maybe membranous or muscular, depending on where it occurs.PATHOGENESIS: Maybe membranous or muscular, depending on where it occurs.
MEMBRANOUS VSDMEMBRANOUS VSD: Most common, 85% of cases.: Most common, 85% of cases.
MUSCULAR VSDMUSCULAR VSD: Less common.: Less common.
MURMUR:MURMUR: Holosystolic MurmurHolosystolic Murmur can be heard.can be heard.
CLINICAL: Most commonly diagnosed disorder, and often associated with other defects, likeCLINICAL: Most commonly diagnosed disorder, and often associated with other defects, like Tetralogy of Fellot.
Tetralogy of Fellot.
o
o ATRIAL SEPTAL DEFECTSATRIAL SEPTAL DEFECTS::
PATENT FORAMEN OVALE: Most common locale of atrial septal defect.PATENT FORAMEN OVALE: Most common locale of atrial septal defect.
OSTIUM SECONDUM DEFECT:OSTIUM SECONDUM DEFECT:
OSTIUM PRIMUM DEFECT:OSTIUM PRIMUM DEFECT:
CLINICAL: Small defects are usually asymptomatic, whereas the larger ones may cause shuntingCLINICAL: Small defects are usually asymptomatic, whereas the larger ones may cause shunting of the blood from left to right.
of the blood from left to right.
o
o PATENT DUCTUS ARTERIOSUSPATENT DUCTUS ARTERIOSUS: Incomplete involution of the ductus arteriosus.: Incomplete involution of the ductus arteriosus.
TREATMENT: Sometimes the ductus will close naturally. Otherwise, two ways to get a patentTREATMENT: Sometimes the ductus will close naturally. Otherwise, two ways to get a patent ductus arteriosus to close:
ductus arteriosus to close:
Indomethacin:Indomethacin: Inhibits prostaglandin synthesis in ductus, which forces its closing. TheInhibits prostaglandin synthesis in ductus, which forces its closing. The opening is mediated by prostaglandins (PGE
opening is mediated by prostaglandins (PGE22).).
Surgically tie it off.Surgically tie it off.
o
o PERSISTENT TRUNCUS ARTERIOSUSPERSISTENT TRUNCUS ARTERIOSUS: Pulmonary artery and aorta are not separated one from: Pulmonary artery and aorta are not separated one from
another but remain a common vessel. another but remain a common vessel. •
• INITIAL RIGHT-to-LEFT SHUNT (Cyanotic)INITIAL RIGHT-to-LEFT SHUNT (Cyanotic)
o
o TETRALOGY OF FELLOTTETRALOGY OF FELLOT
Classical Diagnostic Symptoms:Classical Diagnostic Symptoms:
Pulmonary Artery StenosisPulmonary Artery Stenosis: The principle etiology responsible for the pulmonary: The principle etiology responsible for the pulmonary hypertension and right-to-left shunt.
hypertension and right-to-left shunt.
Ventricular Septal DefectVentricular Septal Defect
Dextroposition of Aorta (overriding)Dextroposition of Aorta (overriding): Aorta originates from the septal area, such that it: Aorta originates from the septal area, such that it receives blood originating from both right and left ventricle.
receives blood originating from both right and left ventricle.
Right Ventricular HypertrophyRight Ventricular Hypertrophy
Patent Ductus ArteriosusPatent Ductus Arteriosusoften co-occurs with Tetralogy, although it isn't part of the syndrome.often co-occurs with Tetralogy, although it isn't part of the syndrome. It is helpful in Tetralogy, as it provides a channel for shunted blood to get back into the pulmonary It is helpful in Tetralogy, as it provides a channel for shunted blood to get back into the pulmonary circulation where it belongs.
circulation where it belongs.
(Right Atrium ---> Right Ventricle ---> VSD ---> Left Ventricle ---> Aorta ---(Right Atrium -> Right Ventricle -> VSD -> Left Ventricle -> Aorta ---> through the Ductus Arteriosus ---> Pulmonary Arteries)
-> through the Ductus Arteriosus ---> Pulmonary Arteries) •
• NO SHUNT NO SHUNT
o
o TRANSPOSITION of the GREAT ARTERIES (Cyanotic)TRANSPOSITION of the GREAT ARTERIES (Cyanotic): Aorta and the pulmonary artery are: Aorta and the pulmonary artery are
transposed. The aorta arises from the right ventricle and the pulmonary artery from the left ventricle. It transposed. The aorta arises from the right ventricle and the pulmonary artery from the left ventricle. It presents with cyanosis of early onset.
presents with cyanosis of early onset.
o
o COARCTATION of the AORTACOARCTATION of the AORTA: Congenital constriction of the aorta.: Congenital constriction of the aorta.
PATHOGENESIS: Occurs usually just proximal or distal to the ductus arteriosus (connection of PATHOGENESIS: Occurs usually just proximal or distal to the ductus arteriosus (connection of aorta and pulmonary artery).
PRE-DUCTAL COARCTATIONPRE-DUCTAL COARCTATION: Occurs in infants. Considered to be incompatible: Occurs in infants. Considered to be incompatible with life.
with life.
POST-DUCTAL COARCTATIONPOST-DUCTAL COARCTATION: Occurs in adults. Post-ductal coarctation is: Occurs in adults. Post-ductal coarctation is associated with a discrepancy in blood pressure between upper and lower extremities associated with a discrepancy in blood pressure between upper and lower extremities --arms much higher than legs, as --arms get all the blood.
arms much higher than legs, as arms get all the blood.
Such patients also develop extensive arterial anastomoses between theSuch patients also develop extensive arterial anastomoses between the subclavian artery and the aorta distal to the constriction.
subclavian artery and the aorta distal to the constriction.
Internal Mammary ArteryInternal Mammary Artery
Intercostal ArteriesIntercostal Arteries-- notching on chest wall-- notching on chest wall
Cerebral hemorrhage is common because of high blood pressure in brain.Cerebral hemorrhage is common because of high blood pressure in brain.
o
o PULMONARY STENOSISPULMONARY STENOSIS o
o CONGENITAL AORTIC STENOSISCONGENITAL AORTIC STENOSIS o
o DEXTROCARDIADEXTROCARDIA: Characterized by inversion of the cardiac chambers. In this condition the left atrium: Characterized by inversion of the cardiac chambers. In this condition the left atrium
and ventricle are on the right side and the right atrium and ventricle on the left. It may be associated with and ventricle are on the right side and the right atrium and ventricle on the left. It may be associated with situs inversus
situs inversus..
o
o EBSTEIN MALFORMATIONEBSTEIN MALFORMATION: Congenital Tricuspid Valve Insufficiency.: Congenital Tricuspid Valve Insufficiency.
Congenital heart disease characterized by downward displacement of abnormal tricuspid valveCongenital heart disease characterized by downward displacement of abnormal tricuspid valve into an underdeveloped right ventricle.
into an underdeveloped right ventricle.
o
o ENDOCARDIAL FIBROELASTOSIS (EFE)ENDOCARDIAL FIBROELASTOSIS (EFE): Thickening of the endocardium of the heart chambers and: Thickening of the endocardium of the heart chambers and
valves most prominent in the left ventricle. valves most prominent in the left ventricle.
PRIMARY EFEPRIMARY EFE: Congenital disease of unknown etiology.: Congenital disease of unknown etiology.
SECONDARY EFE:SECONDARY EFE:Complication of various other cardiac disease characterized byComplication of various other cardiac disease characterized by interventricular hypertension or turbulent blood flow.
interventricular hypertension or turbulent blood flow. ISCHEMIC HEART DISEASE:
ISCHEMIC HEART DISEASE:
•
• ANGINA PECTORIS: Pain originating in chest and radiating to left should, typically. But there are variants.ANGINA PECTORIS: Pain originating in chest and radiating to left should, typically. But there are variants.
o
o STABLE ANGINASTABLE ANGINA: Exertional angina.: Exertional angina. o
o UNSTABLE ANGINAUNSTABLE ANGINA: Late angina carrying poor prognosis. Angina even at rest.: Late angina carrying poor prognosis. Angina even at rest. o
o VARIANT (PRINZMETAL) ANGINAVARIANT (PRINZMETAL) ANGINA: Episodic angina occurring at rest, and due to coronary artery: Episodic angina occurring at rest, and due to coronary artery
spasm. spasm. •
• LAB INDICATORS:LAB INDICATORS:
o
o TROPONIN-T:TROPONIN-T:Most recent blood protein has been shown to have very good predictive value for an earlyMost recent blood protein has been shown to have very good predictive value for an early
MI. Very specific to MI. MI. Very specific to MI.
Cardiac troponin is its own isotype and is different from troponin of the skeletal muscle.Cardiac troponin is its own isotype and is different from troponin of the skeletal muscle.
o
o CAL (Coronary Artery Lesion)CAL (Coronary Artery Lesion): Mnemonic indicates the time course of elevated blood enzymes.: Mnemonic indicates the time course of elevated blood enzymes.
CPK (Creatine Phosphokinase)CPK (Creatine Phosphokinase)will go up first (around same time as Troponin). Again, it's thewill go up first (around same time as Troponin). Again, it's the cardiac isoform that does up. Peaks a few hours after the event.
cardiac isoform that does up. Peaks a few hours after the event.
ASTAST((Aspartate aminotransferaseAspartate aminotransferase) goes up next. Peaks next.) goes up next. Peaks next.
LDH (Lactate Dehydrogenase)LDH (Lactate Dehydrogenase) is the third enzyme to peak, and lasts the longest.is the third enzyme to peak, and lasts the longest.
LDH-1LDH-1 becomes elevated in 6-12 hrs. There is no proportional increase in LDH-2, so thebecomes elevated in 6-12 hrs. There is no proportional increase in LDH-2, so the LDH-1:LDH-2 ratio goes up.
LDH-1:LDH-2 ratio goes up. •
• PATHOGENESIS / PATHOLOGY: Coronary atherosclerosis, thrombosis, and coronary artery spasm.PATHOGENESIS / PATHOLOGY: Coronary atherosclerosis, thrombosis, and coronary artery spasm.
o
o Two types of infarctsTwo types of infarcts
TRANSMURAL INFARCTTRANSMURAL INFARCT: Infarct going from epicardium to endocardium, caused by coronary: Infarct going from epicardium to endocardium, caused by coronary artery occlusion.
artery occlusion.
Stunned MyocardiumStunned Myocardiumis present in the periphery of an infarct zone -- edematous andis present in the periphery of an infarct zone -- edematous and dysfunctional muscle that is not yet dead and may be recovered.
dysfunctional muscle that is not yet dead and may be recovered.
SUBENDOCARDIAL INFARCT:SUBENDOCARDIAL INFARCT:Diffuse, circumferential infarct, around theDiffuse, circumferential infarct, around the subendocardium subendocardium (just inside the endocardial layer). Caused by shock, CHF, hypotension, or anything that results in (just inside the endocardial layer). Caused by shock, CHF, hypotension, or anything that results in inadequate blood supply to the coronary arteries.
inadequate blood supply to the coronary arteries.
Arteries perfuse the myocardium from the outside in. Thus with inadequate blood, theArteries perfuse the myocardium from the outside in. Thus with inadequate blood, the outer layers of muscle will get the blood first, and the inner layers may become ischemic. outer layers of muscle will get the blood first, and the inner layers may become ischemic.
The endocardium can gets its blood from the heart chambers itself -- hence the lesion isThe endocardium can gets its blood from the heart chambers itself -- hence the lesion is described as subendocardial.
described as subendocardial.
o
o GRADES: Coronary atherosclerosis or stenosis is graded as follows.GRADES: Coronary atherosclerosis or stenosis is graded as follows.
GRADE 1: 0-25% OCCLUSION -- asymptomatic and commonGRADE 1: 0-25% OCCLUSION -- asymptomatic and common
GRADE 3: 50-75% OCCLUSION -- stable anginaGRADE 3: 50-75% OCCLUSION -- stable angina
GRADE 4: 75%+ OCCLUSION -- unstable angina, impending MI and thrombosis.GRADE 4: 75%+ OCCLUSION -- unstable angina, impending MI and thrombosis.
o
o ENDOTHELIAL INJURYENDOTHELIAL INJURY: Early grade atherosclerosis may show paroxysmal coronary artery: Early grade atherosclerosis may show paroxysmal coronary artery
contractions resulting from endothelial injury. contractions resulting from endothelial injury.
The substances that were supposed to stimulate release of NO leading to vasodilatation, insteadThe substances that were supposed to stimulate release of NO leading to vasodilatation, instead wind up stimulating vasoconstriction, because there are no endothelial cells present to release the wind up stimulating vasoconstriction, because there are no endothelial cells present to release the NO.
NO.
This vasoconstriction can occur with Grades 1 and 2This vasoconstriction can occur with Grades 1 and 2
Can also cause thrombosis in more advanced lesions.Can also cause thrombosis in more advanced lesions.
o
o TIME COURSE of CHANGE: Time after infarct.TIME COURSE of CHANGE: Time after infarct.The myocardium can survive up to one hour of absoluteThe myocardium can survive up to one hour of absolute
ischemia (center of the infarct) and up to four hours of relative ischemia (periphery of the infarcted area) ischemia (center of the infarct) and up to four hours of relative ischemia (periphery of the infarcted area) ..
1-5 min1-5 min: Swelling of mitochondria and ER; reversible changes. Glycogen depletion and depletion: Swelling of mitochondria and ER; reversible changes. Glycogen depletion and depletion of ATP are noticeable, but no morphological changes grossly.
of ATP are noticeable, but no morphological changes grossly.
20-40 min20-40 min: Irreversible changes microscopically. Cell necrosis, nuclear changes, plasma: Irreversible changes microscopically. Cell necrosis, nuclear changes, plasma membrane rupture.
membrane rupture.
4-12 hrs4-12 hrs: Onset of irreversible coagulative necrosis. Myocardial cells lose striations. Thrombus is: Onset of irreversible coagulative necrosis. Myocardial cells lose striations. Thrombus is still present.
still present.
18-24 hrs18-24 hrs: Pyknosis, karyolysis, karyohexis.: Pyknosis, karyolysis, karyohexis. MyocytolysisMyocytolysisoccurs -- loss of cytoplasm.occurs -- loss of cytoplasm.
1-31-3ddaysays: Widespread necrosis. Infiltration of PMN's begins. Thrombus has often been lysed: Widespread necrosis. Infiltration of PMN's begins. Thrombus has often been lysed naturally by now. Infarct can be recognized on gross examination -- tissue is mottled, red, and naturally by now. Infarct can be recognized on gross examination -- tissue is mottled, red, and congested.
congested.
4-10 days4-10 days: PMN's move out, and macrophages take over to clean up the debris.: PMN's move out, and macrophages take over to clean up the debris. Period of greatest Period of greatest weakness
weakness, during which, during which ruptureruptureand cardiac tamponade may occur. 50% of ruptures occur withinand cardiac tamponade may occur. 50% of ruptures occur within first 5 days and 87% within 14 days. Grossly, necrotic tissue is yellow, soft, and pus-like.
first 5 days and 87% within 14 days. Grossly, necrotic tissue is yellow, soft, and pus-like.
1 month1 month: Granulation tissue has developed. The infarct heals from the outside in, with the central: Granulation tissue has developed. The infarct heals from the outside in, with the central area of necrosis being the last to fibrose.
area of necrosis being the last to fibrose.
3 months3 months: Scar formation is complete.: Scar formation is complete.
o
o CONTRACTION BAND NECROSISCONTRACTION BAND NECROSIS: Hyper contraction of myofilaments. Typically found at the: Hyper contraction of myofilaments. Typically found at the
margins of infarcts or in reperfusion injury. margins of infarcts or in reperfusion injury. •
• CLINICAL:CLINICAL:
o
o SYMPTOMS: Either excessive sympathetic or parasympathetic symptoms may be seen.SYMPTOMS: Either excessive sympathetic or parasympathetic symptoms may be seen.
Sympathetic Symptoms: tachycardia, sweating, pallor Sympathetic Symptoms: tachycardia, sweating, pallor
Parasympathetic Symptoms (Vagal discharge): bradycardia, vomiting.Parasympathetic Symptoms (Vagal discharge): bradycardia, vomiting.
FeverFevermay be seen. Myocardial cells may release endogenous pyrogens such as IL-1 when theymay be seen. Myocardial cells may release endogenous pyrogens such as IL-1 when they rupture.
rupture.
Cardiogenic Shock: Oliguria, hypotension, pulmonary edema, pale and cold extremities.Cardiogenic Shock: Oliguria, hypotension, pulmonary edema, pale and cold extremities.
o
o Leukocytosis with left shift will be seen.Leukocytosis with left shift will be seen. o
o Arrhythmias and other EKG changes may be seen.Arrhythmias and other EKG changes may be seen.
•
• COMPLICATIONSCOMPLICATIONS
o
o ARRHYTHMIASARRHYTHMIAS: 85% of cases. Most important cause of death from MI, and can occur at any time,: 85% of cases. Most important cause of death from MI, and can occur at any time,
either acute or chronic, after the MI. either acute or chronic, after the MI.
The arrhythmias are often reversible -- they go away when edema and inflammation disappear.The arrhythmias are often reversible -- they go away when edema and inflammation disappear.
o
o CARDIOGENIC SHOCK CARDIOGENIC SHOCK : 15% of cases, relatively rare. Systemic hypotension due to pump failure.: 15% of cases, relatively rare. Systemic hypotension due to pump failure. o
o EXTENSION of the INFARCTEXTENSION of the INFARCT o
o RUPTURE, CARDIAC TAMPONADERUPTURE, CARDIAC TAMPONADE: 1-2% of cases. Rare.: 1-2% of cases. Rare. o
o ANEURYSMANEURYSM o
o MURAL THROMBOSIS and EMBOLISMMURAL THROMBOSIS and EMBOLISM: Arterial embolism may go to distal artery and cause tissue: Arterial embolism may go to distal artery and cause tissue
necrosis (kidney), petechiae, or gangrene. necrosis (kidney), petechiae, or gangrene.
o
o PERICARDITISPERICARDITIS:: DRESSLER SYNDROMEDRESSLER SYNDROME is a delayed pericarditis that occurs following cardiacis a delayed pericarditis that occurs following cardiac
surgery or myocardial infarction. surgery or myocardial infarction. COR PULMONALE
COR PULMONALE: Right ventricular failure.: Right ventricular failure.
•
• ACUTE COR PULMONALEACUTE COR PULMONALE: May occur from a saddle pulmonary embolus.: May occur from a saddle pulmonary embolus. •
• CHRONIC COR PULMONALECHRONIC COR PULMONALE: From COPD or from left ventricular failure.: From COPD or from left ventricular failure. •
ACQUIRED VALVULAR and ENDOCARDIAL DISEASES: ACQUIRED VALVULAR and ENDOCARDIAL DISEASES:
•
• RHEUMATIC HEART DISEASE:RHEUMATIC HEART DISEASE:
o
o ACUTE RHEUMATIC FEVER ACUTE RHEUMATIC FEVER : A hypersensitivity disease typically caused by an abnormal immune: A hypersensitivity disease typically caused by an abnormal immune
response to Strep-A antigens. response to Strep-A antigens.
PATHOGENESIS: Immune mechanism involves both B and T cells.PATHOGENESIS: Immune mechanism involves both B and T cells.
CLINICAL: Patient must have at least one of the major symptoms, and two of the minor CLINICAL: Patient must have at least one of the major symptoms, and two of the minor symptoms of the
symptoms of the JONES'S CRITERIAJONES'S CRITERIA::
MAJOR JONES'S SYMPTOMS:MAJOR JONES'S SYMPTOMS:
CarditisCarditis: Found in 35% of patients.: Found in 35% of patients.
PolyarthritisPolyarthritis: found in 75% of patients.: found in 75% of patients.
ChoreaChorea: Rapid jerky, dyskinetic involuntary movements.: Rapid jerky, dyskinetic involuntary movements.
Erythema MarginatumErythema Marginatum
Subcutaneous nodulesSubcutaneous nodules
MINOR JONES'S SYMPTOMS:MINOR JONES'S SYMPTOMS:
Fever Fever
ArthralgiaArthralgia
History of Rheumatic Fever or Rheumatic Heart DiseaseHistory of Rheumatic Fever or Rheumatic Heart Disease
Acute-Phase Reactants: high sed-rate, or positive C-Reactive Protein, which areAcute-Phase Reactants: high sed-rate, or positive C-Reactive Protein, which are evidence of inflammation.
evidence of inflammation.
Prolonged PR interval by ECG.Prolonged PR interval by ECG.
RISK-FACTORS: The more severe the initial Streptococcal infection, the more likely itRISK-FACTORS: The more severe the initial Streptococcal infection, the more likely it is for patients to develop Rheumatic Fever.
is for patients to develop Rheumatic Fever.
o
o CHRONIC RHEUMATIC HEART DISEASECHRONIC RHEUMATIC HEART DISEASE::
PANCARDITISPANCARDITIS: Rheumatic heart disease is typically a pancarditis, affecting all three layers of : Rheumatic heart disease is typically a pancarditis, affecting all three layers of the heart.
the heart.
VALVULITISVALVULITIS: Endocarditis affecting Mitral and Aortic Valves.: Endocarditis affecting Mitral and Aortic Valves.
MITRAL VALVE is most common: Chordae Tendineae get fused together, resulting inMITRAL VALVE is most common: Chordae Tendineae get fused together, resulting in MITRAL STENOSIS
MITRAL STENOSIS.. Rheumatic Fever is the most common cause of mitral stenosis Rheumatic Fever is the most common cause of mitral stenosis ..
Stenosis has a fishmouth appearance.Stenosis has a fishmouth appearance.
You may also at the same time seeYou may also at the same time see mitral insufficiencymitral insufficiencyin the same patient.in the same patient.
AORTIC VALVE is second most common, which can lead toAORTIC VALVE is second most common, which can lead to AORTIC STENOSISAORTIC STENOSIS..
PERICARDITIS:PERICARDITIS: Often seen, but described asOften seen, but described as Bread-and-Butter PericarditisBread-and-Butter Pericarditis. This is an. This is an inflammatory process, but it is
inflammatory process, but it is not Constrictive not Constrictive PericardPericarditisitis..
HISTOPATHOLOGY: It is aseptic so no bacteria are present.HISTOPATHOLOGY: It is aseptic so no bacteria are present.
ASCHOFF BODYASCHOFF BODYis the characteristic finding. A granuloma composed of a centralis the characteristic finding. A granuloma composed of a central triangular area of fibrinoid necrosis surrounded by histocytes. They heal by scarring. triangular area of fibrinoid necrosis surrounded by histocytes. They heal by scarring.
ANITCHKOFF CELLSANITCHKOFF CELLS: Found around the perimeter of the Aschoff bodies. They are: Found around the perimeter of the Aschoff bodies. They are also called
also called Caterpillar cellsCaterpillar cells. Round-to-ovoid nuclei have chromatin disposed in a wavy. Round-to-ovoid nuclei have chromatin disposed in a wavy ribbon resembling a caterpillar.
ribbon resembling a caterpillar. •
• BACTERIAL ENDOCARDITISBACTERIAL ENDOCARDITIS::
o
o CAUSES:CAUSES:
Congenital Heart DefectsCongenital Heart Defects lead to stasis of blood in the heart and therefore predispose to them.lead to stasis of blood in the heart and therefore predispose to them.
Bicuspid Aortic ValveBicuspid Aortic Valve predisposes to bacterial endocarditis resulting in Aortic Stenosis.predisposes to bacterial endocarditis resulting in Aortic Stenosis.
SepsisSepsis
o
o Types:Types:
ACUTE ENDOCARDITISACUTE ENDOCARDITIS
SUBACUTE ENDOCARDITISSUBACUTE ENDOCARDITIS
o
o CONSEQUENCESCONSEQUENCES
Valvular insufficiency or stenosisValvular insufficiency or stenosis
Septic EmboliSeptic Emboliare thrombo-emboli plus bacteria, which may be thrown to a distal artery, causingare thrombo-emboli plus bacteria, which may be thrown to a distal artery, causing gangrene.
gangrene.
Heart Failure secondary to valvular insufficiency.Heart Failure secondary to valvular insufficiency.
Immune complex glomerulonephritis.Immune complex glomerulonephritis. •
• MARANTIC (NON-BACTERIAL THROMBOTIC) ENDOCARDITISMARANTIC (NON-BACTERIAL THROMBOTIC) ENDOCARDITIS: Occurs in people suffering from: Occurs in people suffering from malnutrition, marasmus. They cannot repair the little foci of damage that occur with normal wear and tear of the malnutrition, marasmus. They cannot repair the little foci of damage that occur with normal wear and tear of the heart (constant contraction and what not).
