Diseases Flashcards
Stable Angina
Chest pain that arises with exertion or emotional stress
Due to atherosclerosis of coronary arteries with >70% stenosis
Decreases blood flow is not enough to meet the metabolic demands of the myocardium during exertion
Reversible injury to myocytes-swelling not necrosis
Chest pain lasts less than 20 minutes and radiates to left arm or jaw, diaphoresis, and shortness of breath
EKG shows ST segment depression due to subendocardial ischemia
Relieved by rest or nitroglycerin
Unstable Angina
Chest pain that occurs at rest
Usually due to rupture of atherosclerotic plaque with thrombosis and incomplete occlusion of coronary artery
Represents reversible injury to myocytes-no necrosis
EKG show ST segment deprsesion due to subendocardial ischemia
Relieved by nitroglycerin
high risk of progression to myocaridal infarction
Prinzmetal angina
Episodic chest pain unrelated to exertion
Due to coronary artery vasospasm
Caused by tobacco, cocaine, and triptans
Represents reversible injury to myocytes (no necrosis)
EKG show ST segment elevation due to transmural ischemia
Relieved by nitroglycerin and calcium channel blockers
Ergonovine stress test
Myocardial Infarction
Necrosis of mycoytes-irreversible
Diagnosis:ST elevations on ECG if transmural
St depression if subendocardial
First 6 hours: ECG is gold standard
Cardiac troponin rises after 4 hours and is increased for 7-10 days-more specific
CK-MB diagnosis reinfarction following acute MI levels return to normal after 48 hours
Pathologic Q waves demonstrate evolving or old trasmural infarct
Due to atherosclerotic plaque rupture with thrombisis and complete occlusion of coronary artery
Other causes: vasospasm, emboli and vasculitis
Clinical: severe, crushing chest pain (>20 mins), that radiates to left arm or jaw, diaphoressis, and dyspnea
ATP depletion and loss of contractile function within 60 seconds
Loss of contractility in s syndrome), Ventricular rupture-pericardil tamponade , aneurysm, rupture of papilllary muscle (mitral regurgitation)
Complications: arrhythmia (cause of death before reaching hospital)
LV failure and pulmonary edema (Rapid onset)
Cardiogenic shock-high risk of mortality
Ventricular free wall rupture leads to cardiac tamponade, papillary muscle rupture-mitral regurgitation, interventricuular septum rupture leads to VSD (6-14 days post infarct)
Ventricular pseduoanerysm: decreased CO, risk of arrhytmia, emobolis from mural thrombus (1 week post MI)
Posinfarction fibrionus pericarditis (1 day post MI)
Dressler syndrome
Treatment: Asprin/heparin, O2, Nitrates, B-blocker, Ace inhibitor, Fibrinolysis or angioplasty,
Left Sided heart failure
Causes: ischemia, hypertension, dilated cardiomyopathy, myocardial infarction and restrictive cardiomyopathy
clinical: due to decreased forward perfusion and pulmonary congestion
Pulmonary congestion leads to pulmonary edema: dyspnea, paroxysmal nocturnal dyspnea, orthopnea, and crackles
small congested capillaries may burst causing intraalveolar hemorrhage marked by hemosiderin-laden macrophages
Decreased flow to kidneys leads to activation of renin-angiotensin system exacerbating CHF
Treatment is ACE inhibitor
Right sided heart failure
Most commonly due to left sided heart failure
other causes are left to right shunt and chronic lung disease (cor pulmonale)
Clinical features due to congestion: jugular venous distension, painful hepatosplenomegaly (nutmeg liver) can lead to cardiac cirrhosis, dependent pitting edema (increased hydrostatic pressure)
Ventricular Septal Defect (VSD)
Defect in septum that divides right and left ventricles
Membranous just below aortic valve (more common)
Muscular
Most common congenital heart defect
Associated with fetal alcohol syndrome
Results in left-right shunt
Small defects often asymptomatic, large defects can lead to Eisenmenger syndrome
harsh systolic murmur-large VSDs may not have murmur heard over L. sternal border in 3/4 intercostal space
Accentuated by hand grip leading to increased afterload
Increase in O2 saturation from RA to the Rv
Treatment: resole spontaneously or surgical closure for larger defects
Atrial septal Defect
Defect in septum that divides right and left atria-most common is ostium secundum due to excessive septum primum resorption
Ostium primum type is associated with Down Syndrome failure of septum primum and endocardial cushion to fuse
results in L-R shunt and split S2 on auscultation (Increased blood in right heart delays closure of pulmonary valve)
paradoxical emboli are an important complication
Distinct from patent foramen ovale due to missing tissue rather than just unfused
Patent Ductus Arteriousus (PDA)
Failure of ductus arteriousus to close-Associated with congenital rubella, prematurity, perinatal distress, fetal alcohol syndrome
Present with: dyspnea, cyanosis and frequent respiratory infections
results in L-R shunt between aorta and pulmonary artery
Left heart enlargement and/or RVH, dilated pulmonary arteries and veins, dilated descending aorta
Drop in diastolic pressure
asymptomaticc at birth with continuous “machine like” murmur at left subclavicular region also a palpable thrill may be present
May lead to Eisenmerger syndrome resulting in lower extremity CYANOSIS
Treatment: Indomethacin which decreases PGE resulting in PDA closure
Tetraology of Fallot
Failure of conal truncal septation-Failure of neural crest cells migrating into truncus arteriousus and bulbis cordis
Caused by anterosuperior displacement of the infundibular septum
1. Stenosis of right ventricular outflow tract (pulmonary valve)-determines degree of R-L shunting
2. Right ventricular hypertrophy-boot shaped heart
3. VSD
4. overriding aorta
R-L shunt leads to early cyanosis associated with degree of stenosis also tachypnea
Clinical: fainting, acute onset of fatigue, Blood gas shows low arterial pO2
Can lead to polycythemia
Patients squat in response to cyanotic spell increasing arterial resistance and decreasing shunting allowing more blood to reach the lungs
Boot shaped heart on X-Ray
Transposition of Great Vessels
Pulmonary artery arising from left ventricle (posterior) and aorta arising from right ventricle (anterior)
Due to failure of aorticopulmonary septum to spiral during septation of truncus arteriosus
Associated with maternal diabetes
Presents with early cyanosis
Creation of shunt is required for survival
PGE can be administered to maintain PDA until surgery
Results in hypertrophy of right ventricle and atrophy of the left ventricle
Truncus Arteriousus
Single large vessel arising from both ventricles
Truncus fails to divide
Presents with early cyanosis; deoxygenated blood from right ventricle mixes with oxygenated blood from left ventricle before pulmonary and aortic circulations separate
Most have accompanying VSD
Tricuspid Atresia
Tricuspid valve orifice fails to develop
Right ventricle is hypoplastic
Often associated with ASD or VSD resulting in R-L shunt
Presents with early cyanosis
Coarctation of the aorta
Narrowing of the aorta
Infantile-associated with a PDA
Coaractation lies after the aortic arch but before the PDA
Presents as lower extremity cyanosis in infants, often at birth
Associated with Turner syndrome
Adult-not associated with PDA, coaractation lies after the aortic arch
Presents as hypertension in the upper extremities and hypotension with weak pulses in the lower extremities (NOT CYANOSIS!!)
Collateral circulation develops across the intercostal arteries, engorged arteries cause notching of ribs on Xray
Delayed pulses in lower extremities
Hypertension-headaches and epitaxis
Lower extremity muscle weakness and fatigue due to inadequate muscle supply
Associated with berry aneurysm
Also can die of left ventricular failure and dissecting aortic aneurysm
Associated with bicuspid aortic valve
Chronic Rheumatic Fever
Valve scarring that arises as a consequence of rheumatic fever (mitral regurgitation progresses to mitral stenosis)
Results in stenosis: almost always mitral valve with thickened chordae tendineae and cusps
Occasionally involves aortic valves leading to fusion of commissures
Thickened, fused and shortened chordae
Subendothelial collections of Aschoff nodules in L. atrium forming irregular thickenings called MacCallum plaques
Death due to myocarditis
Complications include infectious endocarditis
Infectious Endocarditis
New onset murmur in young
Causes:
Strep Viridans: most common overall-infects previously damaged valves
Staph. aureus: IV drug abusers affect tricuspid valve (acute)-necrotizing, ulcerative and invasive infections abscesses, spleneomegaly, rapid onset of fever, more risk for emboli
Staph. epidermidis: prosthetic valves
Strep. bovis: underlying colorectal cancer
Haemophilus, Actnobacillus, cardiobacerium, Eikenella, Kingella (HACEK) associated with negative blood cultures
Blood borne pathogens
Contributed by neutropenia or immunosuppression
Clinical: fever, murmur, Janeway lesions (nontender lesions on palms and soles due to thrombi), Osler nodes (tender lesions on fingers and toes due to immune complexes), splinter hemorrhages, Roth spots-white spots on retina (embolization of septic vegetations), anemia of chronic disease
Lab findings: positive blood cultures, anemia of chronic disease,
Large irregular masses on valve cusps extension onto chordae
Copmlications: chordae rupture, diffuse proliferated glomerulonephritis, suppurative pericarditis, emboli
Dilated Cardiomyopathy
Dilatation of all four chambers of the heart
Results in systolic dysfunction-leads to biventricular CHF
Complications include mitral and tricuspid valve regurgitation and arrhythmia
Gross: flabby heart with cardiomegaly
Poor contractile function and stasis-mural thrombi
Findings: heart failure, S3 heart sound, balloon appearance on CXR
Most commonly idiopathic
Swelling of SR is an early sign of doxorubicin associated cardiomyopathy
Genetic mutation-Dystrophin, enzymes of fatty acid beta oxidation
Myocarditis-coxsackie A or B, echovirus-lymphocytic infiltrate in the myocardium
Alcohol abuse
Doxorubicin
Pregnancy
Hemochromatosis
Treatment is Heart Transplant
Hypertrophic Cardiomyopathy
Massive hypertrophy of left ventricle
Usually due to sarcomere proteins (B-myosin heavy chain)
Clinical: decreased CO (SV decreased)-diastolic dysfunction
Sudden death due to ventricular arrhytmias
Syncope with exercise-subaortic hypertrophy of the ventricular septum results in functional aortic stenosis (systolic ejection murmur)
Hypertrophied septum contact the anterior mitral leaflet
myofiber hypertrophy with disarray
Patchy interstitial and replacement fibrosis
Complications: A. Fib-emboli, infective endocarditis, CHF, Ventricular arrhythmias and sudden death
Findings: S4
Decreased preload increases sound of murmur (decreased seperation between mitral valve and IV septum leading to increased obstruction
Maneuvers that increase: sudden standing, valsalva, nitroglycerin
Restrictive Cardiomyopathy
Decreased compliance of the ventricular endomyocardium that restricts filling during diastole-decreased CO
Normal ventricle size but biatrial dilation
Causes: amyloidosis, sarcoidosis, endocardial fibroelastosis (children) and Loeffler syndrome (endomyocardial fibrosis with an eosinophilic infiltrate and peripheral eosinophilia of many organs often with mural thrombus)
microscopic:
Patchy or diffuse interstitial myocardial fibrosis
Presents as Congestive Heart Failure, classic finding is low-voltage EKG with diminished QRS amplitude
Cardiac changes probably due to Major basic protein by eosinophils leading to endomyocardial necrosis, scarring, layering by thrombosis and subsequent organization
Can be associated with myoproliferative disorder with eosinophilia and rearrangements of PDGFR1a or PDGFR
Giant Cell Arteritis
Granulomatous vasculitis that involves branches of carotid artery (large arteries)
Most common vasculitis in adults (>50) usually affects females
Presents as: headache, visual disturbances-acute onset (opthalmic artery), and jaw claudication
Flu-like symptoms with joint and muscle pain (polymyalgia rheumatica)
ESR elevated
Biopsy reveals inflamed vessel wall with giant cells and intimal fibrosis with medial scarring-granulomatous inflammation
lesions are segmental
Treatment is corticosteroids (high risk of blindness without treatment)
Diagnosis with temporal artery biopsy
Takayasu Arteritis
Granulomatous vasculitis that involves the aortic arch at branch points (medium to large arteries)
Presents in asian females less than 40 years old
Pulseless disease-weak upper extremity pulses
Fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances, cold or numb fingers
Granulomatous thickening and narrowing of aortic arch and proximal great vessels
Increased ESR
Treat with corticosteroids
Polyarteritis Nodosa
necrotiizing vasculitis (small to medium arteries) involving multiple organs; lungs are spared
Presents: hypertension (renal artery), abdominal pain with melena (mesenteric artery), neurologic disturbances and skin lesions-livedo reticularis (purplish network patterned discoloration and palpable purpura)
Associated with serum HBsAg
Lesions of varying stages are present due to immune cmoplex deposition
Can induce thrombosis causing distal ischemic injury
Early lesion consists of transmural inflammation with fibrinoid necrosis (hyaline protenaceous depositions in a degenerating vessel wall) -neutrophilic infitrates can extend into adventitia
Eventually heals with fibrosis producing a “string-of-pearls”
Treatment: croticosteroids and cyclophosphamide-fatal if not treated
Kawasaki disease
Affects asian children less than 4 years old
Fever, cervical lymphadenitis, conjunctival injection, changes in lips/oral mucosa, hand foot erythema, desquamating rash
May develop coronary artery aneurysms, thrombosis leading to MI and rupture
Treat with IV immunoglobin and aspirin
Buerger Disease
Thrombangiitis Obliterans
necrotizing vasculitis involving digits
Heavy smoking males less than 40
acute lesions: neutrophilic infiltrates with mural thrombi containing microabscesses with giant cell formation and secondary involvement of adjacent vein and nerve
usually tibial and radial artery
Due to T cell hypersensitivity to smoke modified self Ags
segmental, thrombosing, acute and chronic inflammation of small arteries and veins
Highly associated with heavy smoking-treatment is smoking cessation
Clinical:
nodular phlebitis, Raynaud-like cold sensitivity, leg claudication
vascular insufficiency can lead to excruciating pain ulceration, gangrene, and autoamputation of fingers and toes
Wegener Granulomatosis
necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys
Due to T cell hypersentitivity to inhaled microbe
Granulomas occur with geographic necrosis and accompanying vasculitis
Granulomas can coalesce to produce nodules that cavitate
Classic presentation: middle aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates and hematuria due to rapidly progressive glomerulonephritis (crescenteric)
Serum: c-ANCA levels
Treatment: cyclophosphamide and steroids, relapses common, TNF antagonists
Microscopic polyangiitis
necrotizing vasculitis (small vessels) involving multiple organs, especially lung and kidney !No nasopharyngeal involvement and granulomas are absent!
Fibrinoid necrosis with fragmented neutrophils nuclei within and around vessel walls (leukocytoclastic vsculitis)
Clinical:
Hemoptysis, hematuria/proteinuria, purpura, bowel pain and bleeding
Ab response to drugs, microbes, or tumor proteins with IC deposition
Serum-p-ANCA (anti myeloperoxidase)
Treatment: corticosteroids and cyclophosphamide (cyclosprine)- relapses are common
Churg Strauss syndrome
Necrotizing granulomatous (small vessels) inflammation with eosinophils involving multiple organs especially lungs and heart Pauci immune glomerulonephritis
Migratory pulmonary infiltrates
peripheral neuropathy-wrist/foot drop
adult onset Asthma and peripheral eosinophilia are often present
Eosinophilia can lead to cardiomyopathy
Serum p-ANCA
Henoch Schonlein Purpura
Vasculitis due to IgA immune complex deposition, most common vasculitis in children
Presents with palpable prupura on buttocs and legs, GI pain and bleeding and hematuria (IgA nephropathy) usually occurs after an upper respiratory tract infection
arthralgias
lesions of same age
vasculitis due to IgA and C3 complex deposition
Self limiting but treated with steroids if severe
Primary/Essential Hypertension
BP: systolic greater than 140 diastolic greater than 90
Unknown etiology
Risk factors: age, race (increased african Americans, decreased in asians), obesity, stress, lack of physical activity and high salt diet
Positive family history
Normal Plasma K+
Predisposes to: atherosclerosis, LVH, stroke, CHF, renal failure, retinopathy, and aortic dissection
Isolated systolic hypertension common greater than 50 y.o
Caused by age related decrease in aortic compliance
Secondary Hypertension
Renal artery stenosis
Aterhosclerosis
Fibromuscular dysplasia
Hypertension accelerates development of atherosclerosis and causes arteriolar structural changes that potentiate aortic dissection and cerebrovascular hemorrhage
Risk factors: Smoking-Increases BP Obesity Alcohol lack of Exercise activity Sodium-elderly and AAs particularly sensitive Low potassium Low calcium Low Mg Stress-acutely
Pathophys:
BP=CO x TPR
Most common hemodynamic cause of hypertension is increased TPR
Malignant Hypertension
Severe elevation of BP (>200/120) or just diastolic above 130
may arise from preexisting benign HTN or de novo
Presents as acute end-organ damage (acute renal failure (increased GFR), headache, and papilledema, retinal hemorrhages) medical emergency
Hypertensive encephalopathy: headache, irritability alterations in consciousness
Hyperplastic rterosclerosis: onion like concentric thickening of arterioles
Atherosclerosis
Intimal plaque that obstructs blood flow -necrotic lipid core with a fibromuscular cap also proliferating SMC, inflammatory cells, and increased ECM
Chronic inflammation leads to endothelial cell injury
Risk factors:
Hypertension, hypercholestrolemia, smoking, diabetes
Age, males and postmenopausal females, genetics (most significant)
Additional: inflammation (C-reactive protein), hyperhomocystinemia, metabolic syndrome (adipose tissue cytokines), lipoprotein a, hemostatic factors
Pathogenesis:
Damage to endothelium allows lipids to leak into the intima as well as monocytes and SMCs
Lipids are oxidized and then consumed by macrophages via scavenger receptors resulting in foam cells
Inflammation and healing leads to deposition of ECM and proliferation of smooth muscle from media into the intima(due to platelets releasing PDGF and FGF)
Stages: fatty streaks-flat yellow lesions of the intima consisting of lipid laden macrophages arise early in life
Progresses to plaque
Complications: stenosis of medium sized vessels impairs blood flow and leads to ischemia
Peripheral vascular disease
Angina
Ischemic Bowel Disease
Plaque rupture with thrombosis results in myocardial infarction and stroke
Plaque rupture with embolization results in atherosclerotic emboli characterized by cholesterol crystals within embolus (macrophages contribute to plaque instability due to collagen degradation by metalloproteinases)
Weakening of vessel wall results in aneurysm
Slow developing plaques allow for compensation of arterial collaterals which provide flow to hypoperfused areas
Symptoms: angina, claudication, difficulty with erection (pudendal)
Location: abdominal aorta>coronary artery>poplitieal>carotid>circle of willis
