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Congestive Heart Failure
- An end-stage manifestation of many processes that impede the heart’s ability to function.
- Cardiac output insufficient
- Heart first adapts by increasing size/mass, but eventually fails
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Why does the heart fail?
- Failure of the pump.
- An obstruction to flow
- Regurgitant flow
- Disorders of cardiac conduction.
- Disruption of the continuity of the circulatory system
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Congestive Heart Failure
- Usually chronic inability to deliver needs associated with congestion of organs
- Can be acute after fluid overload, MI, acute valvular function, etc
- Characterized by diminished cardiac output, systolic dysfunction (forward failure), or damming back of blood in the venous system, diastolic failure with insufficient filling (backward failure), or both.
- Not a disease
- End stage of many processes
- May be left or right sided, or both
- Degree of dysfunction and whether compensated or not best evaluated outside of heart.
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Heart failure
- Initially heart can compensate by release of catecholamines, myocyte hypertrophy.
- Hypertrophy due to adaptive molecular and cellular remodelling
- Enlargement later pathologic w/ ineffective function/structure. May have arrhythmias
- Later when compensation fails see dilatation
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Left sided failure
- Ischemia due to MI
- Hypertension
- Aortic stenosis
- Mitral regurgitation
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Forward failure
- Systolic dysfunction
- Tissue hypoxia
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Left sided failure
- Heart can’t keep up w/ venous return from lungs
- Increase back pressure into lungs leads to pulmonary congestion and edema
- Lungs heavy/wet (Rales)
- Micro: Lungs show congestion, edema, and hemosiderin-laden alveolar macrophages in alveoli “heart failure cells”
- Decreased renal perfusion activates renin-angiotensin-aldosterone system, leads to retention of salt and water, worsening pulmonary edema
- Hypoxic encephalopathy
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Symptoms
- Dyspnea on exertion
- Paroxysmal nocturnal dyspnea
- Orthopnea - sit up straight
- Hemoptysis - cough up blood
- Rales - sound
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Signs
- Hypoxic encephalopathy
- Renal underperfusion causes Renin-aldosterone to kick in, worsening pulmonary edema
- Azotemia
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Micro
Myocyte hypertrophy w/ boxcar nuclei (enlarged and rectangular in shape)
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Right sided failure
- Usually due to left sided failure.
- Backup from left into lungs overloads right heart
- Heart cannot accept normal systemic return
- “cor pulmonale” - right heart failure due to pulmonary hypertension.
- Pulmonary edema not prominent
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Relaxation failure
- Diastolic dysfunction
- Congestion and stasis of systemic organs
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Right sided failure - manifestations
- Right heart can’t keep up w/systemic return
- Systemic & portal venous congestion and edema
- Chronic passive congestion of the liver (nutmeg liver)
- Splenomegaly
- Distended neck veins
- Deep leg vein thrombosis, stasis dermatitis
- Pedal and pretibial edema, +/- pitting
- Anasarca - total-body edema, most serious
- Pericardial, pleural effusions, ascites
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Cardiomyopathies
- Intrinsically sick heart
- Primary abnormality of myocardium
- Wide array of etiologies: inflammation, immune, genetic, metabolic, idiopathic
- Dilated, hypertrophic, restrictive based on morphology and function
- Traditionally, exclude ischemic, although “ischemic cardiomyopathy” is a term used by some for CHF associated w/ CAD
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Dilated, hypertrophic, restrictive Cardiomyopathies ??
- dilated: LV LA dilated; small portion is genetic
- Hypertrophic: 100% genetic; LV hypertrophic, LA dilated
- Restrictive: LV normal, LA dilated
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Dilated cardiomyopathy
- Most common pattern (90%)
- Heavy, flabby heart w/ dilated chambers.
- Histology - variable myocyte hypertrophy, boxcar nuclei, and interstitial fibrosis
- Poor contractility leads to increased end-systolic, and diastolic volumes, leading to dilatation
- Slow onset CHF
- Poor ejection fraction
- Mitral regurgitation
- Arrhythmias
- Embolism
- Death within 5 years not rare
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Etiologies of DCM:
infection (myocarditis), cardiotoxic drugs/chemicals (ETOH), nutritional disorders, genetic (25-35%), pregnancy (probably multifactorial), autoimmune, idiopathic
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Although dilated and hypertrophic cardiomyopathies differ greatly in subcellular basis and morphologic phenotypes, they share a common set of clinical complications - heart failure, sudden death, atrial fibrilation, stroke.
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Hypertrophic cardiomyopathy
- Marked LVH without dilatation leads to resistence to diastolic filling with hypercontracted heart and contracted slit-like ventricle (“banana-like”)
- 50% familial (autosomal dominant), 50% sporadic
- Asymmetrical thickening, often in subaortic region of septum, even thicker than the wall.
- Classic young athlete sudden death
- Histological characteristics - disarray (dilated CM is more organized), extreme hypertrophy (boxcar), and branching of myocytes; interstitial fibrosis
- Manifestations
- •Angina
- •Atrial fibrillation w/ possible thromboembolism
- •Mitral infective endocarditis
- •Ventricular arrhythmias
- •Sudden death
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Restrictive Cardiomyopathy
- Essentially normal sized heart, dilated atria
- Reduced ventricular compliance due to stiffening of wall and failure of relaxation leads to resistance to diastolic filling.
- Systolic function unaffected, hence no hypertrophy
- Etiologies - Radiation injury/fibrosis, amyloidosis, sarcoidosis, idiopathic, inborn errors of metabolism, metastatic tumors
- If not idiopathic, see specific pathology of the causative disease
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Amyloid Restrictive Cardiomyopathy
- red stain -> apple green
- Amyloid infiltration around myocytes
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Myocarditis
- Myocardial Inflammation
- Cause but not a response to myocardial injury (ie in MI)
- Coxackieviruses A and B and other enteroviruses probably account for most of the cases.
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Active phase of Myocarditis
- Normal or dilated; some hypertrophy may be present.
- Ventricular myocardium - flabby, mottled by pale foci or minute hemorrhagic lesions.
- Interstitial inflammatory infiltrate and focal necrosis of myocytes adjacent to the inflammatory cells - diagnosis
- The lesions may be diffuse or patchy.
- Mural thrombi may be present in any chamber.
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Congenital Heart Disease
- Abnormalities of the heart/great vessels present at birth
- Most common heart disease in children
- May be associated with genetic syndromes such as trisomy 13,18, 21, XO
- May be caused by a genetic abnormality
- May be environmental, ex Rubella syndrome
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Two basic functional categories
- Shunt (L->R, R->L, or both)
- Obstruction to flow
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Non-cyanotic shunt
- L->R, not bypassing lungs, overloading instead, more common
- Increased pulmonary flow -> increased pulmonary pressure/volume -> pulmonary hypertension -> RVH -> heart failure, cor pulmonale
- Not initially cyanotic, but can become later
- When pulmonary pressure equals systemic, can have reversal of shunt from right to left, leading to cyanosis
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Most Common L to R shunt
- Atrial septal defect (ASD)
- Ventricular septal defect (VSD)
- Patent ductus arteriosus (PDA)
- {Atrioventricular canal (endocardial cushion defect)
- Coarctation of the aorta}
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ASD
- Usually asymptomatic until adulthood
- Complications include
- • Pulmonary hypertension
- • Cardiac failure
- • Paradoxical embolization - thrombus crossing over; right atrial clot going to systemic circulation instead of pulmonary
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VSD
- Most common congenital heart lesion
- If small may be asymptomatic and close spontaneously
- If large, generally presents from birth
- Membranous VSD and Muscular VSD (towards the bottom of the heart)
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AV canals
- Endocardial cushion defect
- A combination of ASD and VSD
- One giant AV valve, not normal
- Most common in trisomy 21, down syndrome
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Patent Ductus Arteriosus
- In fetus, blood goes from pulmonary artery to aorta, bypassing the lungs
- Should close off and become a fibrous band soon after birth
- If stays open after birth, since the systemic pressure is higher, the flow goes from aorta to pulmonary, overloading the lungs, causing pulmonary hypertension.
- Long term risk of CHF (small channel)
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Cyanotic shunt
- Deoxygenated blood from right bypasses lungs, goes to left, hence early cyanosis
- •Tetralogy of Fallot
- •Transposition of the great vessels
- •Truncus arteriosus
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Tetralogy of Fallot
- Right ventricular outlet obstruction (subpulmonic stenosis)
- Ventricular septal defect
- Right ventricular hypertrophy
- Big overriding aorta - due to ventricular hypertrophy
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Transposition of the great vessels
- Two closed systems.
- Need communication to survive - ASD, VSD, PDA
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Truncus arteriosus
- One vessel coming off the heart, different patterns
- Always accompanied by VSD
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Obstruction to flow
- Abnormal narrowing of chambers, valves or blood vessels.
- - Coarctation of aorta - Higher association with turner’s syndrome, 45 XO
- - Pulmonary stenosis or atresia
- - Aortic stenosis or atresia
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Obstruction to flow
- Early life - Depending on severity of obstruction and associated cardiac lesions, patients may present with congestive heart failure, severe acidosis, or poor perfusion to the lower body.
- Beyond infancy - usually asymptomatic. may present with hypertension, headache, nosebleed, leg cramps, muscle weakness, cold feet, or neurological changes. Also differential of pulses - Collaterals
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Blood vessels
- All vessels except capillaries share a three-layered architecture, inner to outer:
- - Endothelium lined intima
- - Surrounding smooth muscle media
- - Supportive adventitia - some capillaries
- Composition of a vessel (smooth muscle cell and matrix content) vary according to hemodynamic demands (e.g., pressure, pulsatility) and functional requirements.
- Composition influences the nature and consequences of pathologic injuries.
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Response of Vascular Wall Cells to Injury
- All vessels are lined by endothelium
- Endothelial cells in specific vascular beds have special features that allow for tissue-specific functions (e.g., fenestrated endothelial cells in renal glomeruli).
- Injury (of almost any type) to the vessel wall results in a stereotyped healing response involving smooth muscle cell proliferation, extracellular matrix deposition, and intimal expansion.
- Excessive thickening of the intima may result in luminal stenosis and vascular obstruction.
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Aneurysms
- A dilatation
- Localized abnormal dilatation of vessel or heart
- May be saccular (outpouching) or fusiform (pointy) in shape
- May be after trauma, congenital (eg berry aneurysm), infection (eg syphilis, mycotic), or relating to systemic diseases
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Berry Aneurysm
- Intracranial saccular aneurysm
- Develop at branch points of Circle of Willis
- If rupture
- - Most common cause of significant subarachnoid hemorrhage
- - 25-50% mortality
- - SEVERE SUDDEN headache
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The most common aneurysm are the aortic aneurysm, below the renal arteries.
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Clinical effects
- Compress nerves, bones, viscera
- Rupture - massive hemorrhage, fatal
- Obstruct renal arteries
- Thromboembolism to leg arteries
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False aneurysm
- Hematoma covered by extravascular connective tissue, extravasation of blood.
- More likely to burst than other types of aneurysm.
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Dissection
tear in intima + extravasation of blood
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Abdominal Aortic Aneurysm - Risk Factors
- Hypertension
- Connective tissue abnormality (eg Marfan)
- Atherosclerosis - weakening the vessel
- Cystic medial degeneration (sometimes w/ Marfan, sometimes independent; w/ snapped elastic fibers)
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Abdominal Aortic Aneurysm - Outcomes
- Rupture into peritoneal cavity or retroperitoneum
- Obstruction w/ ischemia - usually w/ smaller vessels
- Embolism of atheroma or mural thrombus - Stroke, loss of limb possible
- Impingement on adjacent structure, eg ureter or vertebra
- Abdominal “tumor”
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Outcomes of Aortic Dissection
- AD can occur de novo or as consequence of aneurysm
- Creation of a false channel within the muscular wall of the aorta. May not have aneurysmal dilatation
- Expanding hematoma
- Often starts near ascending aorta, near valve
- Often ruptures outwards w/ catastrophic hemorrhage
- Ripping or tearing chest pain as lesion extends
- Can develop hemopericardium with cardiac tamponade
- Can rupture inwards (“double barrel aorta”, “chronic dissection”)
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Vasculitis Syndromes (vasculitides)
- Inflammation of vessel walls
- Variety of causes
- Most common:
- • Direct injury to vessels by infectious organisms
- • Immune-related inflammation
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Immune-related vasculitis - mechanisms
- Immune complex formation with damage
- Anti-neutrophil cytoplasmic autoantibodies (ANCAs) directed against enzymes in neutrophil granules
- - p-ANCA against myeloperoxidase (p=perinuclear)
- - c-ANCA against proteinase (c=cytoplasmic)
- Antiendothelial cell antibodies
- Autoreactive T-cells
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Large vessel vasculitis
- Giant cell arteritis
- Takayasu arteritis
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Giant Cell (Temporal) Arteritis
- Most common vasculitis syndrome
- Cause unknown, possibly a T cell mediated immune response to an unknown antigen in vessel wall
- Genetic. Increased in HLA-DR4 branches of the carotid, esp. temporal & opthalmic
- Destructive granulomatous inflammation
- of the vessel wall
- CD4+ T cells
- Fragmentation of internal elastic lamina
- Usually giant cells
- Inflammation -> intimal thickening -> stenosis +/- thrombosis
- Classic female >50 w/ severe headache and radiating facial pain
- 50% ocular sx due to involvement of opthalmic artery. Treat early to prevent blindness
- About 50% w/ flu-like syndrome + joint stiffness: polymyalgia rheumatica
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Takayasu’s Arteritis
- Granulomatous inflammation of large arteries branching from aortic arch
- Luminal occlusion
- Blindness, neurological impairment, undetectable upper extremity pulses
- Young females
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Medium-sized vessel vasculitis
- • Polyarteritis nodosa (PAN)
- • Kawasaki disease
- • Thromboangiitis obliterans (Buerger disease)
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PAN
- • Segmental necrotizing inflammation of vessels, most often of kidney, heart, liver.
- Lungs spared.
- • Acute inflammation, necrosis and thickening of vessels
- • Can get aneurysms, ruptures
- • Young adults
- • Fever, ischemia of involved organs.
- • Can die of acute renal failure
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Kawasaki disease (mucocutaneous lymph node disease)
- • Involves coronary arteries
- • Usually children under 4
- • Systemic symptoms, fever, eye and mouth mucous membrane redness/erosion, red palms, soles, skin rash, cervical adenopathy
- • Coronary aneurysms (beading) and death possible
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Buerger disease (Thromboangiitis obliterans)
- • Acute & chronic inflammation of arm and leg vessels
- • Can spread from arteries to veins, nerves
- • Big association w/ cigarettes
- • Ulcers/gangrene of extremities
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Small vessel vasculitis
- • Wegner’s granulomatosis
- • Churg Strauss syndrome (allergic granulomatous angiitis)
- • Leukocytoclastic vasculitis
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Wegener’s Granulomatosis
- small to medium-sized vessel disease
- Cause unknown, c-ANCA in 95% of patients
- Classic Triad:
- • Necrotizing/granulomatous inflammation of small to medium sized vessels
- • Necrotizing granulomas of upper/lower respiratory tract including ear, sinuses, oral cavity
- • Necrotizing glomerulonephritis
- Male > Female
- 5th decade
- Persistent pneumonitis w/ bilat cavitary and nodular infiltrates
- Chronic sinusitis, mucosal ulcerations of nasopharynx, renal disease
- Variety of other sx.
- Strawberry Gingiva
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Churg-Strauss Syndrome
- Allergic angiitis and granulomatosis.
- Nose, sinuses, lungs, heart, intestine and nerves.
- May also affect the kidneys, muscles, or joints.
- Associated with asthma and eosinophils.
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Leukocytoclastic vasculitis
Inflammation of the small vessels of the skin
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Vasculitis associated w/systemic disorder - Henoch Schönlein purpura
- • Purpura of extensor surfaces of arms, legs, buttocks
- • Abdominal pain, vomitting, intestinal bleeding
- • Nonmigratory arthralgia
- • Renal abnormalities
- • Necrotizing vasculitis of small dermal vessels
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Vasculitis in summary
- Inflammation of vessel walls.
- Frequently associated with systemic manifestations (including fever, malaise, myalgias, and arthralgias) and organ dysfunction that depends on the pattern of vascular involvement.
- Can result from infections; more commonly has immunologic basis, including immune complex deposition, formation of antineutrophil antibodies (ANCA), or T cell responses to vascular wall antigens.
- Different forms tend to specifically affect vessels of a particular caliber and location, and the clinical manifestations depend on the pattern of vessel involvement.
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Reynaud’s phenomenon
- Exaggerated vasoconstriction of arteries and arterioles in the extremities, particularly the fingers and toes, but also occasionally the nose, earlobes, or lips.
- Primary exaggerated central and local vasomotor responses to cold or emotion; it affects 3% to 5% of the general population and has a predilection for young women.
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Varicose Veins
- Abnormally dilated, elongated, tortuous veins.
- Prolonged increased intraluminal pressure predisposes, eg. long periods of standing, pregnancy.
- Can occur anywhere, 3 important clinical sites:
- - superficial veins of legs;
- - anorectal venous plexus;
- - esophagus.
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Varicose veins of legs
- Upper & lower superficial veins.
- Most common location.
- Increase w/age, obesity, female, pregnancy.
- Venous dilatation, valve incompetence, stasis, congestion/edema, thrombosis (not the one in the deep vein), stasis dermatitis and varicose ulcers.
- Resultant impaired circulation leads to risk of infection and impaired wound healing.
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Anorectal venous plexus
- A K A hemorrhoids.
- Type depends on the place of origin relative to the dentate line.
- Above - internal hemorrhoids.
- Below - external hemorrhoids.
- Above and below - mixed hemorrhoids.
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Esophageal varices
- A complication of portal hypertension in cirrhosis.
- Rupture may cause fatal hematemesis.
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Caput Medusae
Abdominal varicose capillaries.
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Thrombophlebitis & Phlebothrombosis.
- 90% deep leg veins;
- Risk factors include cardiac failure, neoplasia (secretion induce clotting; paraneoplastic syndrome), pregnancy, obesity, postoperative status, prolonged bedrest or immobilization, genetic hypercoagulability;
- Trousseau sign - migratory thrombophlebitis as a paraneoplastic syndrome, esp with pancreas, colon, lung cancers.
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Signs and Symptoms of deep vein thrombosis.
- Early, none;
- Edema, cyanosis, dilated superficial veins, heat, redness, tenderness, swelling, pain, possible;
- Homan sign - pain on forced dorsiflexion of foot.
- Phlegmasia alba dolens - iliofemoral thrombosis with pregnancy;
- Risk of pulmonary embolism.
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Superior vena cava syndrome;
- Neoplasms that compress or invade the superior vena cava;
- Bronchogenic carcinoma, mediastinal lymphoma, or other space-occupying lesions in the mediastinum such as aortic aneurysms.
- Marked dilation of the veins of the head, neck, and arms with cyanosis.
- Pulmonary vessels can also be compressed, inducing respiratory distress.
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Lymphangitis;
- Acute inflammation elicited by the spread of bacterial infections into lymphatics; Group A β-hemolytic streptococci is the most common agent, although can be any microbe.
- Lymphatics are dilated and filled with an exudate of neutrophils and monocytes; the infiltrates can extend through the vessel wall, and in severe cases, can produce cellulitis or focal abscesses.
- Red, painful subcutaneous streaks (the inflamed lymphatics), and painful enlargement of the draining lymph nodes (lymphadenitis).
- If bacteria are not successfully contained within the lymph nodes, subsequent escape into the venous circulation can result in bacteremia or sepsis.
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Lymphedema;
- Primary lymphedema - isolated congenital defect (simple congenital lymphedema) or familial Milroy disease (heredofamilial congenital lymphedema), which results in lymphatic agenesis or hypoplasia.
- Secondary or obstructive lymphedema - blockage of lymphatic by tumors, node-removing surgery, radiation, filariasis (Elephantiasis), postinflammatory.
- Increases hydrostatic pressure in distal lymphatics and causes increased interstitial fluid accumulation.
- Peau d'orange (orange peel) look skin - Persistent edema and subsequent deposition of interstitial connective tissue thus affect the overlying skin, typically the skin overlying breast cancers after tumor cells clog the draining lymphatics.
- Ulcers - may develop due to inadequate tissue perfusion.
- Rupture of dilated lymphatics (e.g., secondary to obstruction from a tumor) leads to milky accumulations of lymph, eg. chylous ascites (abdomen), chylothorax, and chylopericardium.
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Vascular neoplasms can be endothelial-derived (e.g., hemangioma, lymphangioma, angiosarcoma) or can arise from cells that support or surround blood vessels (e.g., glomus tumor, hemangiopericytoma).
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Vascular tumor summary;
- Vascular ectasias are not neoplasms, but rather dilations of existing vessels.
- Vessel neoplasms can derive from either blood vessels or lymphatics, and can be composed of endothelial cells (hemangioma, lymphangioma, angiosarcoma) or other components of vascular wall cells.
- Mostly benign (e.g., hemangiomas), some intermediate, locally aggressive (e.g., Kaposi sarcoma), and others are highly malignant (e.g., angiosarcoma).
- Benign tumors typically form obvious vascular channels lined by normal-appearing endothelial cells.
- Malignant tumors are more often solid and cellular, exhibit cytologic atypia, and lack well-defined vessels.
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Vascular Tumors and Tumor-Like Conditions - Benign Developmental and Acquired Conditions;
- Hemangioma;
- - Capillary hemangioma ;
- - Cavernous hemangioma (large);
- - Pyogenic granuloma (in mouth);
- Lymphangioma ;
- - Simple (capillary) lymphangioma;
- - Cavernous lymphangioma (cystic hygroma);
- Glomus tumor;
- Vascular ectasias;
- - Nevus flammeus ;
- - Spider telangiectasia (arterial spider) ;
- - Hereditary hemorrhagic telangiectasis (Osler-Weber-Rendu disease);
- Reactive vascular proliferations;
- Bacillary angiomatosis.
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Intermediate Grade Neoplasms;
- Kaposi sarcoma;
- Hemangioendothelioma.
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Malignant Neoplasms;
- Angiosarcoma;
- Hemangiopericytoma.
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Hemangiomas;
- Capillary - congenital ones can regress spontaneously;
- Cavernous;
- Pyogenic granulomas - capillary hemangiomas; rapidly growing, red, pedunculated, lesions on skin, gingival, or oral mucosa. They bleed easily and are often ulcerated.
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Lymphangiomas;
- Capillary;
- Cavernous lymphangiomas (cystic hygromas)
- - typically in the neck (common in Turner syndrome) or axilla of children; more rarely in the retroperitoneum.
- - occasionally enormous (up to 15 cm in diameter) and may fill the axilla or produce gross deformities about the neck (disfiguring webbed neck in Turner).
- Margins are indistinct and unencapsulated even though not malignant, making definitive resection difficult.
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Glomus tumor;
- Painful;
- Arising from modified smooth muscle cells of the glomus bodies, arteriovenous structures involved in thermoregulation.
- Distal portion of the digits, especially under the fingernails.
- Excision is curative.
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Nevus flammeus
- “birthmark”, the most common form of vascular ectasias; light pink to deep purple, flat, head or neck, composed of dilated vessels. Most ultimately regress spontaneously.
- "Port wine stain" - a special form. Tends to grow during childhood, thickens the skin surface, and does not fade with time.
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Bacillary angiomatosis;
- Vascular proliferation in immunocompromised hosts caused by opportunistic gram-negative bacilli of the Bartonella family.
- Lesions can involve the skin, bone, brain, and other organs.
- Two species are implicated:
- - B. henselae - lives on domestic cat; causes cat-scratch disease (a necrotizing granulomatous disorder of lymph nodes) in immunocompetent hosts.
- - B. Quintana - transmitted by human body lice; the cause of “trench fever” in World War I.
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Morphology Bacillary angiomatosis.
- Characteristic cutaneous lesion.
- Acute neutrophilic inflammation and vascular (capillary) proliferation. Silver stain demonstrates clusters of tangled bacilli.
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Kaposi sarcoma (KS);
- Intermediate grade vascular neoplasm caused by human herpes virus 8, highly associated with AIDS.
- Classic KS - found in older men of Mediterranean, Middle Eastern, or Eastern European descent (especially Ashkenazic Jews); uncommon in U.S. It can be associated with malignancy or altered immunity, but not AIDS.
- Endemic African KS - HIV-negative individuals.
- Transplant-associated KS.
- AIDS-associated (epidemic) KS is an AIDS-defining illness, and worldwide, it represents the most common HIV-related malignancy.
- Show spindle cells.
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Epithelioid hemangioendothelioma;
- Adults;
- Around medium- and large-sized veins.
- Extremely variable clinical behavior
- Mostly cured by excision, but up to 40% recur, 20% to 30% eventually metastasize, and perhaps 15% of patients die of their tumor.
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Angiosarcoma;
- Older adults;
- May occur at any site, but mostly skin, soft tissue, breast, and liver.
- Hepatic angiosarcoma - associated with carcinogenic exposures, including arsenic (e.g., in pesticides), Thorotrast (a radioactive contrast agent formerly used for radiologic imaging), and polyvinyl chloride (a widely used plastic); Long latency.
- Can arise in the setting of lymphedema, classically in the ipsilateral upper extremity several years after radical mastectomy (with lymph node resection); the tumor presumably arises from lymphatic vessels (lymphangiosarcoma).
- Can be induced by radiation.
- Locally invasive and can readily metastasize; 5-year survival rates approach 30%.
- Histology - Atypia of endothelial cells; hyperchromatic.
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