Lies in the Thoracic cavity in the middle mediastinum

Weighs 250-350g on average

Left cardiac borders is formed by left ventricle, right cardiac border is formed by right atrium

Atria: .2-.3cm walls; interatrial septum i formed from a primum and secondum that form the fossa ovalis

Ventricles: LV=1.3-1.5cm, RV=.3-.5cm; works overlead in hypertension; Interventricular septum is predominantly muscular with a small membranous portion, abnormalities in it are common

Myocardium: modified striated muscle w/ a single nucleus and abundant mitochondria for aerobic metabolism

Valves: Function depends on integrity of valves and their attachments; disease can be asymptomatic or fatal: factors include valve involved, degree and onset of impairment, subsequent compensatory mechanisms

Pericardium: Covering of the heart with an outer fibrious portion and an inner seious layer, the epicardium; disease is often secondary to primary myocardial conditions or systemic disorders

Proteins and enzymes help aid in management and rule in or out MI

The ideal marker would be: accurate and quick, only found in cardiac tissue and not detectable in healthy patients, be released rapidly then be elevated long enough to be detected but fall quickly, inexpensive and simple and rapidly available

 Creatine Kinase: Found in cardiac and skeletal muscle that transfers high energy phosphate in tissues; CK-MB has the highest concentration in cardiac muscle (but also in other tissues); specificity increased if MB fraction is 5% of total CK w/ serial measurements

CK: Detectable at 3-8 hours, peak at 12-14, back to baseline at 3-4 days

CK-MB: Detectable at 4-6 hours, peak at 12-24 hours, baseline at 2-3 days

Myoglobin: Small oxygen carrying protein in muscle tissue; nonspecific, but released quickly; rarely used; detectable at 1-4 hours, peak at 6-9, baseline at 18-24

Troponins: Respiratory complex that modulates muscle contraction; 3 proteins-C, T, I; I is best for cardiac muscle; may be persistantly elevated in patients with unstable angina; detectable at 4-6 hours, peak at 24-48, baseline at 6-10 days

-CK-MB (widely accepted and understood, inexpensive) and troponin I (most specific, useful for patients that seek attention late, may have prognostic significance) are the typically ordered ones and used diagnostically

IHD=Leading cause of death in developed world; most is caused by atherosclerosis; characterized by a difference between O2 supply and demand

4 clinical syndromes: MI, angina, chronic ischemic heart disease, sudden cardiac death

MI: Results from abrupt change in an atherosclerotic plaque from stable to unstable and thrombus formation

The defects that are compatible with life usually only involve individual chambers or regions

Many are surgically correctable if performed before irreversible secondary pulmonary hypertension develops

May cause hypertrophy/dialation

A few have identifiale genetic abnormalities (Ex: Turner's, Trisomy 21)

Many are related to errors in mesynchemal tissue migration; environmental influences likely play a role

L to R shunts: Increases pulmonary blood flow, not associated w/ cyanosis, often leads to RVH->potential right sides heart failure; includes ASD, VSD, PDA

R to L shunts: Dimineshed pulmonary blood flow w/ less O2 to tissues and cyanosis; Includes Teratology of Fallot, Transposition of Great Arteries, Persistant Truncus Arteriosis, tricuspid atresia, and total anomalous pulmonary venous connection

Obstruction: Coarctation of Aorta (aorta narrows in the area where the ductus arteriosus attaches), Aortic stenosis, and pulmonary stenosis

Normal wall: Veins are thin walled and contain less smooth muscle, arteries are thick-walled to handle higher pressure

Intima: Endothelium and some loose connective tissue

Media: Smooth Muscle

Adentitia: Loose connective tissue

Endothelial Cells: Dynamic and multifunctional w/ many synthetic and metabolic properties

Smooth muscle: responsible for changes in caliber, has migratory and proliferative properties, plays a role in repair

Aneurysm: Localized abnormal dilation of blood vessel/wall of heart; True aneurism: bounded by heart wall structures; includes atherosclerotic, syphilitic, congenital and ventricular wall

Dissection: Blood enters artery wall and splits layers; May or may not be associated w/ aneurysm; Typically occur in thoracic aorta; Associated with HTN and connective tissue disorders

Abdominal Aortic Aneurysm: True aneurysm; Intimal plaque causes medial destruction

Congenital aneurysms frequently involve vessels in the brain

Generally exclude ischemic heart disease, they're primary myocardial disease

Myocarditis: Inflammation of the myocardium that's the cause of disease rather than a result of myocardial injury; Caused by infections, hypersensitivity reactions, and autoimmune disease; Can range from no symptoms to sudden death

Cardiomyopathy: Dilated, hypertrophic and restrictive-the 3 have different etiologies but are related; Uncommon; Often idiopathic

Can have secondary complications including CHF, Ischemia, Arrhythmias

Stenosis or regurgitation may be pure or coexist w/ one predominating

Mitral and aoric valves are most commonly involved

Can be clinically insignificant or fatal depending on degree of disease, rate of development, and compensatory mechanisms

Stenosis: Almost always due to primary cusp abnormalities, usually chronic

Insufficiency/Regurgitation: Results from intrinsic disease of valve cusps or damage to supporting structures; acute or chronic

Inability of the heart to pump blood at a sufficient rate to meet the O2 demands of the peripheral tissues

Common, can result from many cardiac and pulmonary diseases

Most frequent causes: Ischemic heart disease and hypertension (systolic); Diastolic dysfunction can lead to CHF too

Symptoms result from hypoperfusion of tissues or venous backflow/edema

Frequently a recurrent condition with poor prognosis

Type I: Adaptive thickening of intima, smooth muscle cells infiltrate so HHF-35 stain +, NO macrophages so CD68- and oil-red-O -

Type II: Intimal Xanthoma (fatty streak) with lipid laden macrophages (foam cells) in intima; CD68+ and oil-red-O +

Type III: Pathologic Intimal Thickening with pools of extracellular lipid (EL) w/o a well defined lipid core; no symptoms b/c arteries are capable of positive remodeling; Intimal SMC's in a proteoglycan and collagen matrix; Occasional macrophages

Type IV: Fibrous cap atheroma w/ well-formed lipid (necrotic) core of extracellular lipids surrounded by macrophages and lymphocytes with overlying smooth myscle rich fibrous cap

Type V: Thin-cap fibroatheroma is a vulnerable plaque with a large lipid (necrotic) core and thin fibrous cap infiltrated by macrophages (CD68+)

Type VI: Coronary thrombosis via plaque rupture or plaque erosion

75% of coronary thromboses

Thin-cap fibroatheromas

Mostly men and post-menopausal women

Associated w/ high TC, low HDL, DM

Mean area stenosis=80%

Calcification present in 70%

25% of coronary thromboses

SMC and proteoglycan rich plaques

Superficial intimal injury (w/o lipid core exposure)

Associated w/ cigarette smoking

Mean area stenosis=80%

Calcification present in 25%

Fewer SMC's in fibrin caps b/c of loss of integrity->increaed likelihood for rupture

Increaed cap inflammation

Thin cap (<65um)

Larger lipid pool

MMP's: Released by macrophages; matrix degrading enzymes; expression reduced by statins and lipid lowering therapy->increased plaque collagen and stabilization

MMP-1=collagenase

MMP-3=stromolysin

MMP-9=gelatinase B

Myeloperoxidase: reacts w/ hydrogen peroxide to form oxidative molecules and tissue molecules and potentially a rupture

High CRP: Marker of global inflammation, an acute phase reactant made by the liver and regulated by IL-1, IL-6, and TNF; Activate compliment, bind LDL, stimulate macs to produece tissue factor

Calcification: Degenerative change of atherosclerosis that can be seen in stable and unstable plaques

Intraplaque hemorrhages: Result from disruption of microvessels within plaque; Causes plaque to expand in size and induces inflammatory response and increases plaque instability

Increasing age

Family history: 1st degree relative with early onset

Dislipidemia: High TC, LDL, and TG, low HDL; High LDL increases lipid infiltration into arterial wall; Low HDL decreases lipid departure from arterial wall

Cigarette smoking: Causes endothelial injury and dysfunction, leading to a prothrombic state

Hypertension: Allows enhanced lipid infiltration into arterial wall and endothelial injry

DM: Causes endothelial dysfunction, prothrombic state; associated w/ HTN, high LDL, and low HDL; 25% of Americans >60yo

Metabolic syndrome: multiple risks, a combo of several of: type 2 DM, high TG, low HDL, HTN, obesity

Patients w/ diabetes and hypercholesterolemia have increased macrophage infiltrates and increased necrotic core size than patients w/ one or the other (meaning more risk factors multiply your risk for atherosclerosis/CVD)

Chest pain brought on by exertion-the most common symtpom of IHD

Occurs when lumed is occluded >75%

Usually stable

Recurrent episodes of substernal or left sided chest pain possibly radiating to left arm, jaw, or upper abdomen

Pain lasts 5-15 minutes and is relieved by rest or a sublingual nitroglycerine tablet

No irrreversible damage, no enzyme elevation

Chest pain at rest or with minimal exetion, symptoms get progressively worse

ECG starts showing changes: ST depression during pain

Patient may have non-occlusive thrombi in coronary arteries

Coronary thrombosis

Most often caused by plaque rupture

Patient has ECG anormalities, high CK-MB and troponin

Usually physical symptoms, 10-15% asymptomatic

Symptoms: severe constricting chest pain often radiating to arm, left shoulder, jaw; sweating, nausea, shortness of breath

Instant or unexpected death within 24 hours of symptom onset

Usually caused by IHD/coronary atherosclerosis w/ >75% occlusion

Mechanism of death is usually lethal cardiac arrhythmia (V-tach or V-fib)

Acute coronary thrombosis present in 50%

Healed MI present in 50%

Immediately: Begin to lose ATP

1-2 minutes: Loss of contractility

10 minutes: 50% of ATP gone

20-40 minutes: Irreversible Cell Injury

1 day: Infarcted area has pallor

24-72 hours: Pallor with hyperemic borders

3-5 days: Maximum necrosis; Peak neutrophil (PMN) infiltration resulting in hyperemia and a central soft yellow-brown area in the myocardium

7 days: Loss of hyperemia and infarct looks more molted

2-3 weeks: Healing-soft, depressed, refractile, gelatinous (granulation tissue)

4-6 weeks: Scarring complete

Non-Reperfused:

3-6 hours: Wavy fibers

12-24 hours: Hypereosinophilic Myocytes

24 hours: PMN infiltration

3+ days: Myocyte nuclei and cross striations disappear; PMN necrosis

3-5 days: Peak PMN infiltration

5-7 days: Macs, capillaries, lymphocytes appear at infarct periphery

8-10 days: Pigmented macs and fibroblasts appear

10-14 days: Granulation tissue, phagocytosis in infarct core

2-4 weeks: fibroblast infiltration, collagen synthesis

4-6 weeks: Mature scar

Reperfused:

12 hours: Diffuse contraction band necrosis (hypereosinophylic bands within cardiomyocytes)

12-24 hours: diffuse interstitial hemorrhage

24 hours: Peak PMN infiltration

2-3 days: PMN infiltration resolves

4-5 days: Mac and lymphocytes remove necrotic myocytes

6-7 days: Early angiogenesis (granulation tissue)

7-10 days: Angiogenesis and early fibrosis

-Restored blood flow and Ca leads to the diffuse contraction band necrosis

 -Diffuse interstitial hemorrhage indicates restored blood flow

Reperfused peak neutrophil infiltration occurs at 24 hours as opposed to 3-5 days in non-reperfused infarcts; angiogenesis and early scar formation occurs at 7-10 days as opposed to 2-4 weeks

Reperfusion: Done with balloon angioplasty or thrombolytic therapy (tPA)

Left Anterior Descending (LAD): Most common (40-50%); infarct of anterior and anterioseptal region of the LV

Right Coronary Artery: (30-40%), proximal occlusion infarcts the posterior wall of LV and posterior 1/3 of ventricular septum

Left circumflex coronary: 15-20%, infarcts the lateral wall of LV

Infarct size depends on duration and severity, collateral blood sources, and metabolic demands of ischemic myocardium

Subendocardial: <50% thickness of ventricular wall, less complications

Transmural: >50-75% thickness of ventricular wall, large MI's with higher risk of complication

Cell death progresses from endocardium to epicardium, reperfusion can safe part of myocardium

Cardiac Arrythmias: Tachyarrhythmias: v-tach and v-fib; increase mortality if they occur >24 hours after MI; 50% of IHD Deaths; Treatment: Automatic Defibrilator; Bradyarrhythmias: Less common, usually in inferior wall MI with sinus bradycardia and AV block; treatment: pacemaker if block is permenant

Heart failure: LV systolic failure: In significant LV dysfunction when LV ejection fraction <40%; Cardiogenic shock: Infarct involves >40% of LV, mortality rate=90%

Mitral Regurgitation: When there's mitral valve annular (annulus is a fibrous ring that's attached to leaflets) dilation or papillary muscle injury; chronic severe mitral valve regurg is associated w/ dialated left atrium and poor prognosis

LV Free Wall Rupture: 10% of post-MI mortality, usually 3-5 days post-MI w/ peak coagulation necrosis and neutrophil infiltration; Sudden death-hemopericardium progressing to cardiac tamponade (signs of tamponade: Beck's triad: Low BP, jugular venous distention, muffled heart sounds); Old, female, HTN, 1st MI, poor collateral circulation increase risk

Interventricular septum rupture: 3-5 days post-MI for same reasons as LV wall rupture; Sudden death or heart failure due to acute shunt

Papillar muscle rupture: 3-5 days for same resons; associated w/ Acute Inferior MI; Posteromedial papillary muscle (only has 1 blood supply); Sudden death/heart failure due to mitral regurgitation

LV Mural Thrombus: Akinetic/diskinetic wals don't contract well->blood flow stasis leads to thrombi; associated w/ large transmural MI's

LV aneurysm: Outpouching of myocardial wall->becomes diskinetic->rarely rupture but are associated w/ heart failure and ventricular arrhythmias

Infarct Extension: MI enlarges to involve adjacent areas

Infarct expansion: LV dilates during infarct extension since LV wall thins->worse prognosis ("Expanding LV")

Pericardial Effusion: 25% of acute MI, normally small and insignificant but can cause hemodynamic problems if bigger; diagnosed w/ echo

Post-MI pericarditis: 24-96 hours post MI->inflammatory response to necrotic tissue; late pericarditis (1-8 weeks) is cause by autoimmmune responses

Comes from bulbus cordus

Goes from being on top of the heart tube to bottom, as ventricle grows faster than atrium and causes heart to rotate

Forms pulmonary trunk and aorta

Endocardial cushions form a primitive valve between atria and ventricles->eventually AV septum forms

Defects higher in trisomy 21

Septum primum forms across the chamber with foramen primum as a small hole in it

A second small hole, the foramen secundum, forms to allow foramen primum to close

Septum Secundum develops on top of the septum primum, leaving a small gap called the foramen ovale, which alows R to L shunting

Great vessels (aorta and pulmonary artery) must form spiral septum to allow ventricular septum to close

If ventricular septum fails to meet spiral septum or spiral septum fails to meet ventricular septum->ventricular septal defect

R to L sunts

Teratology of Fallot

Transposition of great vessels

Truncus arteriosus

Tricuspid atresia

Total anomalous pulmonary venous return 

5 T's

Early Cyanosis: Blue Babies

Late cyanosis: Blue kids

VSD (most common congenital cardiac abnormality)

ASD 

PDA

If untreated, cause RVH

All these can be surgically repaired

No tricuspid valve, hypoplastic right ventricle

Requires ASD and VSD for viability

Decreased caliber of pulmonary artery causes increased resistance, so blood shunts R to L at 1st opportunity (Foramen Ovale)

In utero, the connection allows a R to L shunt from pulmonary artery to aorta

If it fails to close, a L to R shunt occurs due to pressure

Failure to thrive from excess of blood in lungs

Corrected by surgery or indomethacin

Pulmonary Artery Stenosis

RVH

Overriding Aorta

VSD

"PROVe"

Most of the flow goes into the aorta b/c it's overriding and pulmonary artery is small

Blalock-Taussing Shunt: Original treatment; Connects one of the subclavian arteries to the pulmonary artery to allow for a corrective L to R shunt, but compromises circulation to an arm

Current solution: Artificial tube connecting subclavian artery and pulmonary artery, to maintain flow into arm and allows flow into lungs to be more tightly controlled; access otained using lateral thoracotomy during surgery

Squatting improve symptoms

Spiral septum closed corectly, but aorta was attached to RV and pulmonary arteries to RV

Switching them did not work b/c the heart was being fed with deoxygenated blood (coronary arteries were now attached to deoxygenated blood source since they come off aorta)

Mustard procedure: Bafle connects SVC and IVC to mitral valve, bypassing 3 chambers to go into LV; Septum between L and R atria obliterated so blood can mix; LV->pulmonary artery->atrium->RV->aorta->systemic circulation; Causes RVH

Newer approach allows surgeons to reposition great vessels and switch coronary arteries from the pulmonary trunk to the aorta

W/o a shunt to allow mixing, incompatible with life

RVH can be managed acutely by removing or adding fluid

If resistance increaed, RV increases preload by increasing venous return->RV collapses if this fails

Chronically, fluid management doesnt work; RVH can increase wall stress and pressure on LV->decreased LV volume->diastolic dysfunction->increased energy demands->ventricular ischemia

Infantile: proximal to insertion of ductus arteriosis ("IN close to heart")

Adult: Stenosis distal to ductus arteriosis: Distal to Ductus; HTN in upper extremeties; weak pulse in lower

Associated w/ turners

Associated w/ bicuspid aortic valve

After ruling out CAD and valvular disease, since these usually result in contractile dysfunction rather than the problem being a primary disease of the myocardium

CAD: Diagnosed by angiography

Valvular disease: Diagnosed by auscultation and echo

Myocarditis: Inflammatory diseases of the myocardium; caused by infection, toxins, autoimmunity, or allergy; classified by histological appearance; sometimes idiopathic

Cardiomyopathy: Non-inflammatory diseases of the myocardium

Most common form

Cause: Autoimmune damage after Enterovirus (especially Coxsackievirus B3), but likelihood of a positive culture is low (infection is fleeting)

Viral replication (often asymptomatic)->autoimmune injury (immunosuppresives used at this stage)->dilated cardiomyopathy (Beta blockers and ACE Inhibitors)

Clinical: Heart failure, arrhythmias, sudden death (similar to dilated cardiomopathy); Most recover, 10-25% progress to dilated cardiomyopathy (some after latency period of months to years)

Pathology: Myocyte necosis and lymphocytic inflammation

Gross: Ventricular dilation and flabby appearance

Pericarditis common

Allergic reaction to meds, esp antibiotics (cephalosporins)

Rare, Generally asymptomatic

Symptoms: Heart failure, arrhythmias

Pathology: Eosinophilic infiltrates, little myocyte necrosis so little scarring

Unknown cause, may be autoimmune

Myocyte necrosis and destruction by macrophage Giant cells

Rapidly fatal

Young to middle age caucasians

Toxins: catecholamines, chemo, adriamycin, etc

Histologic findings: NeuTrophils, edema, conTracTion bands

Chronic disease, less than 5% with sarcoid have cardiac involvement

Symptoms: Death, arrhythmias, constrictive and dilated cardiomyopathy

Histologic findings: Nonnecrotizing granulomas with scarring

Often involves LV free wall, you see fibrosis in myocardium

Immunocompromised (may occur in immunocompetent)

Viral, Protazoal, fungal, or bacterial

Viral organisms: CMV most common

Protozoal: Toxoplasmosis most common

Chagas: Cardiomyopathy in S. America

Primary disease of the heart muscle

Dilated: Mild/Moderate Ventricular Hypertrophy; Dilated cavity, Systolic Dysfunction

Hypertrophic: Severe Ventricular Hypertrophy; Small Ventricular Cavity; Diastolic Dysfunction (decreased compliance)

Restrictive: No Ventricular Hypertrophy; Normal cavity; Diastolic Dysfunction (decreased compliance)

Right Ventricular: No Ventricular Hypertrophy; Dilated cavity; Systolic Dysfunction/Arrhythmias

Idiopathic-unknown etiology, not well understood

Rare, typically 30-40yo males

35% 5-year-survival, 15% 10 year survival

Symptoms: Heart failure, Dyspnea, arrhythmias

Complications: Systemic, pulmonary emboli

Associated with anormalities of cytoskeletal proteins

Pathology: Non-specific, 4 chamber dilation, Mural thrombi, Normal arteries and valves, Myocyte atrophy/hypertrophy, fibrosis

May be 2nd to viral myocarditis (10%), Association with autoimmune diseases, heavy alcohol use, familial autosomal dominan X linked, Peripartal (pregnancy)

In contrast to LVH caused by increased afterload, ventricle is assymetric b/c of an ill-defines mass of disorganized myocytes frequently located at ventricular septum, instead of concentric

Subaortic stenosis: muscle mass obstructs left ventricle outflow in 1/2 to 1/3 of patients

Rare, 20-50yo males

Symptoms: Chest pain, dyspnea, syncope, sudden death (exercise-related)

Echo: Systolic anterior motion of mitral valve

70% 10 year survival

Pathology: Dilated left atrium; LV outflow tract plaque; Myofiber disarray; Thickened intramural coronary arteries

Associations: 50% familial from B-myosin heavy chain

Abnormalities of Sarcometric proteins

Ventricles have normal thickness and cavities, but lack compliance

Result in dilated atria, congested lungs and liver

Pathologic conditions: Diseases of myocyte, interstitum (infiltrative), and endocardium

Clinically: Similar to Constrictive Pericarditis

Diseases: Infiltrative: Amyloidosis (gives heart waxy/glistening appearance; Most common cause in US), Radiation, Sarcoidosis; Endocardial (Most common cause worldwide): Eosinophilic (Loeffler's), Idiopathic (Davies); Idiopathic; Myocardial: Storage diseases, Anthracycline toxicity, End-stage hypertrophic cardiomyopathy

Right ventricular aneurysms, Thinning of RV wall, arrhythmias

Sudden death (exercise), right sided heart failure

Thinning of RV with fat and fibrous tissue replacement

30% familial (autosomal dominant)

Bulge/Ballooning out of the wall of a vessel

Typically occurs in arteries

Saccular, Small Saccular (berry), Fusiform, Giant; Congenital or acquired

Saccular: Discreet outpouching of medial layer-True aneurysm

Mycotic aneurysm: Underlyin infection w/ bacteria or fungus, usually saccular

Fusiform: Gradual enlargement of medial layer-True aneurysm

Psuedoaneurysm: Body's reaction of wall off aneurysm with a wall of fibrous tissue; Generally traumatic-False aneurysm

Dissecting Aneurysm: Pane of blood develops between medial and aventitial layer with a wall of fibrous tissue

True: Has all 3 normal layers

False: Walls formed by fibrous tiissue

Cerebral: Berry aneurysm, most common

Marfan: Usually causes aneurysm of aorta occasional peripheral arteries; Autosomal dominant, chromosome 15, fibrillin-1 defect; Includes medial degeneration; Effects elastic part of arteries

Ehler's Danlos Type IV: Collagen mutation, causing easy bruising, thin skin, saccular aneurysms, dissetions, or arterial rupture

Fibromuscular Dysplasia: Weakly familial; Characterized by malformed blood vessels and renal vascular hypertension

Ascending: Syphilis, aortic root dilating, Marfan's

Syphilitic: Inflammatory destruction of aortic wall; May present with hemopericardium; wrinkled appearance of intima (tree-barking); necrosis and inflammation of media (gummas)

Dissecting: Seen in ascending and thoracic aorta; Hypertension, Marfan's, and bicuspid aortic valve are risk factors; Also called medial degenerating aneurysms; High mortality due to rupture of the false lumen

Descending: Trauma, Infection, Atherosclerosis

Traumatic: Distal to left subclavian near ligamentum arteriosum

Infectious: Mycotic aneurysms

Abdominal: Typically seen with atherosclerosis, smoking, HTN; distal to renal arteries

Inflammation of the blood vessels

Any vessel may be involved

Lumen is often compromised and tissues supplied by the blood vessel are damaged

Vessels involved by a disorder could be local or systemic

Noninfectious immunologic mechanisms: immune complex deposition, anti-neutrophil cytoplasmic antibodies, and anti-endothelial cell antibodies

Cause is undetermined

Antigen is rarely identified

Immune complexes do not need to be deposited in the vessel wall to cause vasculitis

Antigen-antibody complex deposited->C5a compliment activated->attracts neutrophils that phagocytoze complex and damage tissue

Macrophages and lymphocytes infiltrate and may cause further damage

Directed against proteins withtin the cytoplasm (primary granules) of neutrophils

Heterogeneous group of antibodies 

cANCA (cytoplasmic): Diffuse, granular, cytoplasmic staining; Target: proteinase 3 (PR3)

pANCA (perinuclear): Localized pattern of staining within or around nucleus; Target: myeloperoxidase (MPO)

No causal role in Vasculitic syndroms, but serve as quantitative markers

Directed nonspecifically to endothelial cells

May predispose to some vasculitic syndromes

Most common type in US

Large vessel inflammatory disorder involving one of more branches of the carotid, usually the temporal artery

Opthalmic and vertbral arteries may be involved

No exact mechanism, propably against arterial wall component like elastin

Patients >50, associated w/ polymalgia rheumatica

Non-specific symptoms (fever, weight loss, fatigues) or headache or facial pain; Artery may be tender to touch; Visual symptoms

Diagnose from biopsy

Morphology: Segmental involvement of artery, granulomatous inflammation in inner half of media, Fragmentation (destruction) of internal elastic intima

Treatment: Glucocorticoids

Prognosis: Good

Inflammatory disease of medium and large-size vessels with preference for aortic arch and its branches

Granulomatous Inflammation and Stenosis, wrinkled aorta

No known cause, Women <40

Symptoms: Low BP, Weak upper extremity pulse, coldness and numbness of fingers; Ocular distrubances

Clinical assessment and angiography to diagnose

Morphology: Irregular thickening, mononuclear infiltrate often involving vaso vasorum, Granulomatous inflammation of media and adventitia with scarring, Intimal thickening and fibrosis

Treatment: Glucocorticoids with angioplasty for stenosed vessels

Prognosis: Variable

Complications: Cerebrovascular accidents, MI, Pulmonary HTN

Systemic necrotizing vasculitis involving small and medium-sized muscular arteries

Segmental lesions involving areas of bifurcation

Renal and visceral arteries, sparing of pulmonary arteries; Muscular arteries, not arterioles, venules, or capillaries

Likely immunologic, 20-30% have Hepatitis B antigenemia; Associated w/ Hep C and Hairy Cell Leukemia

Middle aged Males

Symptoms: Non-specific, symptoms from ischemia and infarction of affected organs; abdominal pain, multiple aneurysms, skin lesions

Patient usually has elevated WBC (neutrophilia) and ESR

Morphology: Transmural necrotizing inflammation, early fibroid necrosis, late fibrosis; All morphology can coexist

Treatment: Glucocorticoids, plasma exchange

Prognosis: Variable, relentless flare-ups

Complications: Bowel infarcts and perforation, hypertension, peripheral neuritis, death from renal failure

Mucocutaneous lymph node syndrome: an acute, febrile illness in children <4

Arteritis involves coronary arteries

Viral infection?

Symptoms: Cervical lymph node enlargement, fever, conjunctival and oral erythema, skin rash, swelling of hands and feet

Morphology: Necrotizing inflammation, Intimal proliferation, beadlike aneurysms

Treatment: Gamma globulin and aspirin

Prognosis: Good, <3% develop fatal cardio consequences

Called Wegener's if it involves URT lesions, lung lesions, and glomerulonephritis; MPA if only small vessel vasculitis present

Affects arterioles, capilaries, and venules

Renal and pulmonary capillaries; If renal->necrotizing glomerulonephritis

Immunologic response to antigen like a drug, microbe or tumor 

Clinical: Hemoptysis (lung lesions); arthralgias, abdominal pain, hematuria, proteinuria, muscle pain or weakness

Diagnosis: Biopsy, pANCA (70% of patients)

Treatment:  Glucocorticoids, cyclophosphamide

Prognosis: Variable, Death from renal failure or pulmonary hemorrhage

Segmental, thrombosing inflammation of small and medium-sized vessels, especially tibial and radial arteries

Associated with cigarette smoking, also likely has a genetic predisposition, <35

Severe pain in extremeties even at rest

Diagnosis: Clinical

Morphology: Segmental inflammation both acute and chronic, thrombosis with microabscesses, granulomatous component, late fibrosis

Treatment: Don't smoke, arterial bypass surgery as necessary

Most commonly seen in the skin

Histology the same as MPA

Reaction to drugs or other antigens

Tricuspid: 3 leaflets tethered by chordae tendinae

Chordae: Vary in lengthy but arise from a large anterior papillary muscle; Chordae+Papillary=Tensor Apparatus

Tensor Apparatus: Papillary muscles contract->bring down chordae->valve opens

Mitral: 2 leaflets (anterior/aortic and posterior) tethered by chordae tendinae; Aortic leaflet has variable continuity w/ aortic valve->part of outflow tract of blood from ventricle through aorta

Pulmonary: 3 cusps (anterior, right, left)

Cusp: U shaped (semilunar) shallow pocket 

Aortic: 3 semilunary cusps (right, left, and posterior)(sometimes less); right->right coronary artery origin, left->left coronary artery origin

Stenosis: Failure of a valve to open completely

Regurgitation: Failure of a valve to close completely->blood leaks back into previous chamber

Stenosis and Regurgitation usually occur alone but can occur together

Influences on disease: Which valve involved, rate of disease development (quick=bad), quality of compensatory mechanisms (LVH=most common and most likely from aortic stenosis)

Often from post-inflammatory scarring from rheumatic heart disease

A result of abnormalities in the leaflets or commissures (space between eaflets), tensor apparatus,  or valve annulus

Leaflets/Commissures: Post-inflammatory scarring secondary to rheumatic heart disease, infective endocarditis (usually bacterial), or mitral valve prolapse

Tensor Apparatus: Ruptured papillary muscle or chodae tendinae (most likely from acute MI: Ischemic papillary dies->floppy valve)

Valve anulus: Valvular base where it attaches to the heart; Can be from LV enlargement or calcification of mitral annulus

The most common cause of heart disease

Due to post-inflammatory scarring from rheumatic heart disease or calcium deposition which makes nodules to obstruct blood flow (calcific aortic stenosis)

Puts you at risk of valvular disease, aortic aneurysm, and dissections

Most common cause of congenital heart disease (2-3%)

Valve is subjected to abnormal shear and pressure forces->shear forces cause calcification of valve

Most common acquired valve abnormality (2%), caused by aging and normal wear+tear

Can be superimposed on a congenital bicuspid valve, can occur with atherosclerosis or independently->patient may not have systemic atherosclerosis

Instead of smooth muscle cell proliferation (normal atherosclerosis), we have proliferation of osteoblast-like cells that lay down Calcium

Free edges are not involved (center of valves are), commissural fusion isn't seen (like in rheumatic heart disease)

Seen more in 3rd world (lack of antibiotics)

Young person w/ strep pyogenes

Pharyngitis dissapears->heart disease a few weeks later manifesting as valvular abnormalities and causing edema and fluid back up into lungs

Latent phase: Body directs humoral immunity against M protein of strep that can cross react with patient's myocardium

Acute: Pericarditis (all layers), anitschkow cell

Chronic: Mitral valve thickening and commissures fuse->fish mouth appearance; shotening, fusion and thickening of chordae tendinae; fibrosis, calcification, and neurovascularization

Mitral valve always involved, sometimes others (pulmonary=least likely)

Marked by small, warty vegetations along the lines of closure of valve leaflets

Anterior, posterior, or once in a while both mitral valves become floppy and prolapse back into left atrium during systole->valve becomes larger than normal and creates a hood during prolapse

Mostly asymptomatic, could cause sudden death

Path: Leaflets-enlarged, thick, and rubbery with ballooning posterior mitral leaflet; chordae-elongated and thinned; mucoid material is deposited into the valve spongiosa matrix making the valve floppy; fibrosis of LV or atrium can occur due to snapping of elongated chordae tendae against cardiac wall

Sterile thrombi can occur on atrial surfaces of floppy mitral valve-> go to coronary arteries, cerebral arteries, renal arteries

Microclots can break off and embolize

Infectious (acute or subacute), non-bacterial thombotic related, or Libman-Sacks (disease state)

Most important cause of aortic regurgitation

Caused by colonization of heart valves or surface endocardium by microorganisms (usually bacteria)

Usually effects mitral valve (very rarely pulmonic)

Acute/Native Valve Endocarditis: Normal valve-highly virulent organism; ONLY S. Aureus; Post-infection the valve displays necrosis, vascularization, and destruction; Can invade myocardium and cause myocardial abscess (abcess+embolization of bacterial colonies->septic infarct)

Subacute: Previously injured valve, less virulent organism (strep), less destructive; Can be due to myoxamous degeneration, calcific aortic stenosis, bicuspid valve, artificial valves, or congenital defects; Most commonly after dental procedures w/o prophylaxis (S. Viridans) or IV drug use (S. Aureus); Usually affects left sided valves except in drug users-right sided (usually tricuspid->can lead to Septic PE); Abscess affects annulus of tricuspid and aoric valve (usually w/ S. aureus in IV drug users)

Marked by large irregular masses on the valve cusps that can extend into chordae

MIM: Transient bacteria that results from opportunistic colonization after endothelial injury (from turbulent blood flow from an acquired or congenital cardiac abnormality); You get localized inflammation near the valves as a response to endothelial trauma->damaged tissue attracts platelets, fibrinonectin, and fibrin and thrombus develops->bacteria see damaged heart valves and form a mass on the heart valve complicated by the thrombus->mass can evade the immune system and some antibiotics->requires long term IV antibiotic treatment

Can result in MVP-most likely to develop in patients with regurgitant murmers

Most IE infections: Strep Viridans

If patient has underlying adenocarcinoma of colon: S. Bovis (group D)

MRSA: If patient has underlying hematologic malignancies and catheters placed

Enterococcus Infections: Can result from recurrent GU infetions and history of prosthetitis

Prosthetic valves: Encocarditis caused by coagulase negative staph (S. epi)

Culture negative endocarditis: Patients with low grade fever, lines, and recent dental procedures after prior antibiotic group; fastidious and grow 2-4 weeks after incubation; all of these organisms live in mouth; HACEK bacteria, legionella, chlamydia, brucella, bartonella

Clinical: IE presents within 2 weeks of infection (low grade fever/nonspecific fatigue/malaise can go on longer in strep); Valvular destructions (esp S. Aureus b/c it seeds along electrical conduction pathway)->CHF, pulmonary edema; The transient bacteria that led to infection can become a continuous bacteremia

Physical exam: New murmur, fever; local thrombosis in microvasculature, Janeway lesion-painless macular lesion on palms or soles of feet from pieces of vegetation in microcirculation; Osler's node-immune complex deposit in microvasculature->local vasculitis (presents as raised nodules painful to palpatation: Osler's=ouch); Roth's spots-red inclusions in retina around optic disk blood vessels from circulating immune complexes as a result of local thrombosis and vasculitis

All symptoms: Small vascular beds in periphery

Must treat rapidly: 1/3 of patients have septic emboli to liver, kidney, or brain; 20% have a CNS event

Diagnosis: 2 major criteria, 5 minor, or a combo; + blood culture (3 sets over 24 hours, 10 ml blood per culure) and echocardiographic change in valve movement is sufficient

Transesophageal echocardiogram is more sensitive to IE than transtrachial

Duke's criteria for endocarditis: Focus on oscillating masses in echo, + blood cultures

Treatment: IV antibiotics (not oral) for 4-6 weeks; Aminoglycosides like gentamycin for S. Viridans, Vancomycin for S. Aureus

Deposition of smaller sterile thrombi on valve leaflets along the lines of closure->thrombi may produce emboli, w/ or w/o infarcts

Associated with MALT (mucin-producing adenocarcinomas) in SLOP (stomach, lung, ovary, or pancreas)

Non-infectious, non-invasive

Marked by small bland vegetations, usually along line of closure

Patients with SLE: Anti-phospholipid antibodies deposit on heart valve

Tricuspid, Mitral, or both

Sterile and small thrombi on either side of the valve, on the chordae, or on the mural endocardium

Can be valve necrosis (so you could mistake it for bacterial)

Marked by small to medium-sized vegitations on either or both sides of valve leaflets

Right side valves (tricuspid and pulmonic) are exposed to a low pressure system

Left side valves (mitral and aortic) are exposed to a high-pressure system and are more likely to present with pathology

Echo: The tool for valvular heart disease

Regurgitant disorders: Happen in combos-reversal of blood flow when valve is supposed to be closed

Blood flow apparati are dynamic->compensate to meet its own and the body's nutritional needs

Normally during diastole, aortic leaflets open to meet at the commisure and block blood flow back into LV

Bicuspid aortic valve: Leads to irreversible fibrosis after inflammatory response (most often after rheumatic disease), calcification in the elderly and in response to mechanical sress (dystrophic change around collagen fibers), myxoid degeneration (overproduction of extracellular proteins and connective tissue->leaflets can't function normally), and destruction-associated with a concurrent pathology (secondary, not primary)

Related to physical exertion->decreased exercise tolerance

SOB and chest pressure

CHF as disease progresses

Orthopenia-shortness of breath while lying down, from increased pulmonary venous pressure

Echo

Murmurs: Indicate contitions like prolapse; can do provocative maneuvers to enhance the murmurs

Increased LV volume: Leg raise, squatting; Makes aortic stenosis, mitral regur, and tricuspid regug louder, mitral prolapse click later

Decreased LV volume: Valsalva; Makes Tricuspid Regurg louder, mitral prolapse click earlier

Increased TPR: Hand Grip, makes aortic stenosis softer, mitral regur louder

Decreased TPR: Nitrates; makes mitral regurg softer

Mitral valve prolapse: works better and has less prolapse when the ventricle is full

Aortic Stenosis and Mitral regurg: Heard in systole, more likely with increased ventricular volume

Hypertrophy in concentric fashion

Originally a diastolic dysfunction, then progresses

Symptoms: Dyspnea and angina, may or may not be related to CAD

Thickened calcified leaflets that only open a little->valve is always closed so you can't tell the difference between systole and diastole on Echo

Heard as a late systolic click, loudest along left sternal border, also heard at apex (earlier with valsalva)

Young women

Echo: Leaflets prolapse past the annular plane (normally they stop there)

Symptoms: Nonspecific-fatigue, palpitations, chest pain

Older men

Related to MVP, but more serious

Secondary-caused by connecive tissue diseases or hypertrophic cardiomyopathy

Prognosis: Determined by Left Ventricular Ejection Fraction

Ventricle must pump blood twice

To increase CO, the heart increases stroke volume and contractility while getting larger

Myocyte stretch: early compensation to pump more out of ventricle

When myocyte stretch fails->heart increses filling pressure->overcapacity in cardiac cycle leads to pulmonary edema->decreased performance during exertion, then at rest

Results in a thin, passive flow of blood through the right heart

Heterogenicity and thickening of leaflets

Bacteria and clot susceptible to mobilization and embolization

Symptoms: Incresed systolic wave, holosystolic murmur along left lower sternal border, murmur louder upon inspiration (indicates right side of heart), increaed systolic wave in JVP, Hepatomegaly

Younger patients

Can result from altered blood flow, annular dilation from pulmonary HTN, and catheter/pacer wire adherence to tricuspid valve structures

Mitral valve moves independently from the rest of the system

Left side endocaditis is most dangerous b/c emboli go into systemic circulation and occlude aorta or carotids

Caused by bacteria and other material rom the heart that travels from the lungs

Comes from right heart, except in patients with an ASD or VSD

Bacteremic infections: sustained IV antibiotic treatment is needed

Surgical tretment

Class 1: Clear benefit to patient; surgical treatment of patients with stenosis/regurg resulting in HF, patients with severe pulmonary hypertension, infective endocarditis by highly resistant organisms or in the presence of heart block, annular, or aortic abcesses; No class I indications for antibiotic prophylaxis

Class II: surgical treatment for patients with infective endocarditis with recurrent emboli and mobile vegitations; Class II indications for antibiotic prophylaxis are patients with prosthetic valves, previous endocarditis, congenital heart disease, heart transplant, or dental procedures

State in which the heart can't produce sufficien CO to meet the metabolic needs of the body

Heart failure->decreased CO->increased HR and SV, maintain MAP through RAAS and SNS

To increase SV: Heart dilates to allow increased preload (Frank Starling method of increasing EDV to increase SV)

Congestion: Heart reaches a point where dilating to increase preload no longer changes SV

Healthy heart: Can go into heart failure if you increase preload (ex: inject saline)

Failing heart: CHF occurs at lower preload (lower blood volume), because dilating the ventricle can't increase SV as much as it would in a healthy heart

Chronicaly increased SV and blood volume: Lead to left venricular heart failure; increased MAP means larger afterload for the heart to work against->cardiac remodeling and worsening of heart failure

Myocardial injury from CAD with or w/o infarction, Chronic HTN, or Idiopathic (40%) (or many other less common etiologies)

Abnormal thickening of heart walls

Muscle fibers are in disarray->increased stiffness so it's harder to fill the heart with blood

Diastolic Heart Failure/Heart Failure with Preserved Ejection Fraction (HFPEF): Ventricular filling is reduced->CO decreases even though ventricle can contract well (aka normal EF)

No treatment

Usually caused by left sided heart failure

Blood gets backed up from LV to LA and increases pressure->pulmonary pressure increases->strain on right ventricle

Also caused by pulmonary HTN, congenital defects

Heart failure is the largest expenditure of medicare

10% of the population over 75 have it

Younger people tend to have systolic heart failure

Cost=30 billion/year

50% of patients return to hospital in 6 mo, 20% in 30 days

1 year mortality=33%

ACC/AHA:

A. At risk for HF but w/o structural heart disease or symptoms

B. Structural heart disease w/o HF

C. Structural heart disease w/ prior or current HF 

D. Refractory HF requiring specialized interventions

NYHA:

I. Asymptomatic

II. Symptomatic w moderate exertion

III. Symptomatic with minimal exertion

IV. Symptomatic at rest

When the patient presents with hypotension, murmur on exam, or LVH/LAE on EKG, order a surface echo

Transesophageal echo: additional imaging, but more invasive and carries greater risk->proximity of LA and aorta to the esophagus make it particularly dangerous if involvement of these areas is suspected

All walls moving

Some regurgitation is normal, EXCEPT in aortic regurgitation

Examine the valves: Vavle thickening can be caused by carcinoid syndrome-cardiac symptoms with advanced carcinoid cancer, among many other things

Tricuspid should appear closer to the apex than the mitral valve: If not, RA might be expanded compared to RV (Estein's anomaly)

Red flow is towards transducer, blue is away

Increased turbulant flow->hypertrophic cardiomyopathy

MVP: Can be diagnosed via parasternal and apical long axis views on echo

Ex: Apex and sepum akinetic due to mid-LAD obstruction

VSD: Heard as a holostolic murmur

3 causes or holostolic murmur: MR, TR, VSD

Rupture of papillary (only one source of blood)

Disruption of wall motion in area of apex b/c of enlarging of pericardial space following free wall rupture

Immediate Pericardiocentesis necessary

Common in cancer, but occurs more gradually then after a cardiac procedure

Classic: Male with uncontrolled HTN, chest pain (10/10) radiating to back

Patients with Marfan's or connective tissue disorder

Wide pulse pressure

Holodiastolic murmur

Ascending AA=surgical emergency

Descending can be treated medically (B Blocker) until renal involvement or loss of distal pulse

Flap of an aortic dissection can obstruct coronary ostium->patient can present w/ ST segment elevation

Tricuspid regurgitation and right-sided volume overload

LV appears flat instead of rounded on echo

Sometimes you can see clot on Echo

Able to visualize the prolapse and turbulant flow as well as flail segment (from ruptured chordae) on echo

Surgically reparing valve is preferred, b/c mechanical valves last 7-10 years and require lifetime warfarin therapy

Class 1: Always do it; Benefit>Risk

Class IIa: Benefit>Risk so it's reasonable, but more studies needed

Class IIb: Benefit may be greater than risk, but more studies needed

Class III: Don't do it! Risk>Benefits

Level A: Tons of RCT to support recommendation

Level B: Some RCT data

Level C: Recommended based on standard of care, expert opinion, case studies, or limited studies of population

IF LVEF <40%, prescirbe ACE-I or ARB, access LV function, counsel patient to stop smoking

B-Blocker: Class I level A recommendation; Bisoprolol, Carvedilol, and sustained-release metoprolol reduct mortality

Aldosterone antagonists: currently no recommendation, seem to improve survial

Implanted Cardioverter Defibrillator (ICD): Primary preventio for sudden cardiac death in patients with dilated cardiomyopathy or ischemic heart disease

Cardiac Resynchronization Therapy: for biventricular pacing (to pace both RV apex and LV free wall so they can contract together); corrects widened QRS

Cardiac Resynchronization+ICD (most CR patients get ICD)

Left ventricular assist device (LVAD): Causes low pulse pressure but not a big deal; Used in severe HF

No specific drugs to target diastolic function

We try to control HTN, ventricular rhythm, and get rid of fluid