Pathology Flashcards
What causes congenital right to left shunts? 5 things
- Tetralogy of Fallot - MCC
- Transposition of great vessels
- Persistent Truncus arteriosus - failure of truncus arteriosus to divide into pulmonary trunk and aorta - most also have VSD
- Tricuspid atresia - characterized by absence of tricuspid valve and hypoplastic RV; requires both ASD and VSD for viability
- Total anomalous pulmonary venous return - pulmonary veins drain into right heart circulation (SVC, coronary sinus, etc); associated with ASD and sometimes PDA to allow for right-to-left shunting to maintain CO
What causes congenital left to right shunts? 3 things
VSD - most common congenital cardiac anomaly
ASD - loud S1; wide, fixed split S2
PDA - closed with indomethacin
Eisenmenger’s syndrome
Uncorrected VSD, ASD, or PDA causes compensatory pulmonary vascular hypertrophy, which results in progressive pulmonary hypertension.
As pulmonary resistance increases, the shunt reverses from left-to-right to right-to-left, which causes late cyanosis, clubbing, and polycythemia
Tetralogy of Fallot Cause: Presents with: How do you correct sx: Tx:
Caused by anterosuperior displacement of the infundibular septum. Pulmonary infundibular stenosis, RVH, Overriding aorta, VSD (PROVe).
Presents with early cyanosis caused by right to left shunt across the VSD bc pulmonary stenosis forces the right to left shunt and causes RVH (VSD alone is usually left to right).
Older patients learn to squat to relieve cyanotic sx (squatting reduced blood flow to the legs, increase peripheral vascular resistance, and decrease the cyanotic right to left shunt across the VSD).
Tx: primary surgical correction - early
D-transposition of great vessels
Cause:
Looks like:
Tx:
Caused by failure of the aorticopulmonary septum to spiral
Aorta leaves RV and pulmonary trunk leaves LV –> separation of systemic and pulmonary circulations –> not compatible with life unless a shunt is present to allow adequate mixing of blood (VSD, PDA, PFO)
Tx with surgical correction and without this most infants die within the first few months of life
Coarctation of the aorta results in:
Infantile type:
Adult type:
Aortic regurgitation
Infantile - aortic stenosis proximal to insertion of ductus arteriosus (Turner’s syndrome) –> check femoral pulses
Adult - stenosis is distal to ligamentum arteriosum. Associated with notching of the ribs (due to collateral circulation), hypertension in upper extremities, weak pulses in lower extremities –> bicuspid aortic valve.
Patent ductus arteriosus Shunting: Murmur: Cause: If uncorrected: Tx:
In fetal period - shunt is right to left then in neonatal –> lung resistance decreases and shunt becomes left to right with subsequent RVH and/or LVH and failure.
Murmur: continuous, “machine like” murmur
Cause: patency is maintained by PGE synthesis and ow O2 tension and if uncorrected can result in late cyanosis in the lower extremities
Tx: indomethacin
*Sometimes necessary to maintain life in conditions like transposition of great vessels
22q11 syndromes associated with
Truncus arteriosus, tetralogy of Fallot
Down syndrome associated with
ASD, VSD, AV septal defect (endocardial cushion defect)
Congenital rubella associated with
Septal defects, PDA, pulmonary artery stenosis
Turner syndrome associated with
Coarctation of aorta (preductal)
Marfan’s syndrome associated with
Aortic insufficiency and dissection (late complication)
Infant of diabetic mother associated with
Transposition of great vessels
Hypertension
RF
Features
Predisposition
- BP greater or equal to 140/90 mmHg
- Increase age, obesity, diabetes, smoking, genetics, black > white > asain
- 90% of HTN is primary and related to increase CO or TPR; other 10% is secondary to renal disease; malignant htn is severe (>180/120) and rapidly progressing
- atherosclerosis, LVH, stroke, CHF, renal failure, retinopathy, aortic dissection
Hyperlipidemia signs: Atheroma Xanthomas Tendinous xanthoma Corneal arcus
Atheromas: plaques in blood vessel walls
Xanthomas: plaques or nodules compsed of lipid-laden histiocytes in the skin, especially eyelids (xanthelasma)
Tendinous xanthoma: lipid deposit in tendon (Achilles)
Corneal arcus: lipid deposit in cornea, nonspecific (arcus senilis)
Atherosclerosis:
Monckeberg
Arterioloscerlosis
Atherosclerosis
Monckeberg: calcification in the media of the arteries, especially radial or ulnar (Usually benign, pipestem arteries) - do not obstruct blood flow and intima not involved
Arteriolosclerosis: hyaline (thickening of small arteries in essential htn or dm) and hyperplastic (onion skinning in malig htn)
Atherosclerosis: fibrous plaques and atheromas form in intima of arteries
Atherosclerosis: RF Progression Complications Location Sx
Dz of elastic arteries and large and medium sized muscular arteries
- RF: smoking, htn, hyperlipidemia, dm; age, gender, + fmhx
- Progression: inflammation important in pathogenesis; endothelial cell dysfxn –> MO and LDL accum –> foam cell formation –> fatty streaks –> smooth muscle cell migration (PDGF/FGF), proliferation, and ECM deposition –> fibrous plaques, complex atheromas
- Complications: aneurysms, ischemia, infarcts, peripheral vascular dz, thrombus, emobli
Location: Ab aorta > coronary artery > poplitearl artery > carotid artery
Sx: angina, claudication, asymptomatic
Aortic aneurysms
Pathologic dilation of blood vessel
Ab aortic aneurysms
Associated with:
Associated with atherosclerosis
More frequently with HTN males who smoke >50 yoa
Thoracic aortic aneurysms
Associated with:
Associated with htn, cystic medial necrosis (Marfan’s syndrome) and tertiary syphilis
Aortic dissection Associated with: Presents: CXR: Result in:
Longitudinal intraluminal tear forming a false lumen
Associated with htn, bicuspid aortic valve, cystic medial necrosis, inherited connective tissue disorders
Presents: tearing chest pain radiating to back
CXR: mediastinal widening, false lumen (limited to ascending aorta, propagate from ascending aorta, or propagate from descending aorta)
Results in: pericardial tamponade, aortic rupture, death
Angina
Types:
CAD narrowing >75%; no myocyte necrosis
Types:
- Stable: mostly secondary to atherosclerosis, ST depression on ECG (retrosternal chest pain with exertion)
- Prinzmetal’s variant: occurs at rest secondary to coronary artery spasm; ST elevation on ECG
- Unstable: thrombosis with incomplete coronary artery occlusion; ST depression on ECG (worsening chest pain at rest or with minimal exertion)
Coronary steal syndrome
Vasodilator may aggravate ischemia by shunting blood from area of critical stenosis to an area of high perfusion
Myocardial infarction
Most often acute thrombosis due to coronary artery atherosclerosis with complete occlusion of coronary artery and myoctye necrosis; ECG initially shows ST depression progressing to ST elevation with continued ischemia and transmural necrosis
Sudden cardiac death
Death from cardiac causes within 1 hour of onset of symptoms, most commonly due to a lethal arrhythmia (ventricular fibrillation)
Associated with CAD
Chronic ischemic heart disease
Progressive onset of CHF over many years due to chronic ischemic myocardial damage
Coronary artery occlusion: MC to LC
LAD > RCA > circumflex
MI symptoms
diaphoresis, nausea, vomiting, severe retrosternal pain, pain in left arm and/or jaw, shortness of breath, fatigue
0-4 hr of MI
No gross or microscopic signs
Risk: arrhythmia, CHF exacerbation, cardiogenic shock
4-12 hr of MI
Gross: Occluded artery, infarct, dark mottling, pale with tetrazolium stain
Microscopic: Early coagulative necrosis, edema, hemorrhage, wavy fibers
Risk: arrhythmia
12-24 hr of MI
Gross: Occluded artery, infarct, dark mottling, pale with tetrazolium stain
Microscopic: contraction bands from reperfusion injury, release of necrotic cell content into blood, beginning of PMN migration
Risk: arrhythmia
1-3 days of MI
Gross: hyperemia
Microscopic: extensive coagulative necrosis, tissue surrounding infarct shows acute inflammation, PMN migration
Risk: fibrinous pericarditis
3-14 days of MI
Gross: hyperemic border; central yellow-brown softening (maximally yellow and soft by 10 days)
Microscopic: macrophage infiltration followed by granulation tissue at the margins
Risk: free wall rupture leading to tamponade, papillary muscle rupture, ventricular aneurysm, interventricular septal rupture due to macrophages that have degraded important structural components
2 weeks of MI
Gross: recanalized artery, gray-white
Microscopic: contracted scar complete
Risk: Dressler’s syndrome
Diagnosis of MI in first 6 hours
ECG is the gold standard
Diagnosis of MI labs
Cardiac troponin I rises after 4 hours and is elevated for 7-10 days
CK-MB is predominantly found in myocardium but can also be released from skeletal muscle (useful in dx reinfarction following acute MI because levels return to normal after 48 hours)
Diagnosis of MI with ECG changes
ST elevation (transmural infarct), ST depression (subendocardial infarct), pathologic Q waves (transmural infarct)
Transmural infarct characteristics
Increased necrosis
Affects entire wall
ST elevation on ECG, Q waves
Subendocardial infarct characteristics
Due to ischemic necrosis of <50% of ventricle wall
Subendocardium especially vulnerable to ischemia
ST depression on ECG
Leads with Q waves in anterior wall infarction (LAD)
V1-V4
Leads with Q waves in anteroseptal infarction (LAD)
V1-V2
Leads with Q waves in anterolateral infarction (LCX)
V4-V6
Leads with Q waves in lateral wall infarction (LCX)
I, aVL
Leads with Q waves in inferior wall infarction (RCA)
II, III, aVF
MI complications
Cardiac arrhythmia - important cause of death before reaching hospital; common in first few days LV failure and pulmonary edema.
Cardiogenic shock - large infarct (high mortality)
Ventricular free wall rupture –> cardiac tamponade; papillary muscle rupture –> severe mitral regurg; and interventricular septum rupture –> VSD
Ventricular aneurysm formation –> decrease CO, risk of arrhythmia, embolus from mural thrombus; greatest risk approximately 1 week post-MI
Postinfarction fibrinous pericarditis - friction rub (1-3 days post MI)
Dressler’s syndrome
autoimmune phenomenon resulting in fibrinous pericarditis (several weeks post MI)
Dilated Cardiomyopathy
Causes:
Findings:
Tx:
Most common cardiomyopathy
Causes: idiopathic, up to 50% familial, chronic alcohol abuse, wet beriberi, coxsackie B virus myocarditis, chronic cocaine use, Chaga’s disease, doxorubicin toxicity, hemochromatosis, peripartum cardiomyopathy (ABCCCD)
Findings: S3, dilated heart on US, balloon appearance on CXR, systolic dysfxn ensues, eccentric hypertophy
Tx: Na+ restriction, ACE-I, diuretics, digoxin, heart transplant
Hypertrophic Cardiomyopathy
Causes:
Findings:
Tx:
Hypertrophied interventricular septum is “too close” to mitral valve leaflet, leading to outflow tract obstruction (murmur, syncope)
Causes: 60-70% familial (auto dom - B-myosin heavy chain mutation), Friedreich’s ataxia
Findings: disoriented, tangled, hypertrophied myocardial fibers, normal sized heart, S4, apical impulses, systolic murmur, diastolic dysfxn ensues, asymmetric concentric hypertrophy (sarcomeres added parallel)
Tx: b-blocker, non-dihydropyridine calcium channel blocker
What leads to sudden death in young athletes?
Hypertrophic cardiomyopathy
Restrictive/obliterative cardiomyopathy
Causes:
Findings:
Causes: sarcoidosis, amyloidosis, postradiation fibrosis, endocardial fibroeleastosis (thick fibroelastic tissue in endocardium of young children), Loffler’s, hemochromatosis
Diastolic dysfunction ensues (reduced LV compliance)
Loffler’s sydnrome
endomyocadial fibrosis with a prominent eosinophilic infiltrate
CHF
Inherited or acquired abnormality of cardiac structure or fxn
Sx: dyspnea, fatigue, edema, rales
Right CHF
Most often from left heart failure; isolated heart failure from cor pulmonale
Tx of CHF
ACE-I, B-blocker (not in acute decompensated), angiotensin receptor antag, spironolactone –> reduce mortality
Thiazide or loop diuretics are used mainly for symptomatic relief
Hydralazine with nitrate therapy improves both symptoms and mortality in select patients
Cardiac dilation
Greater ventricular end-diastolic volume
Dyspnea on exertion
Failure of cardiac output to increase during exercise
Signs of left heart failure
Pulmonary edema, paroxysmal nocturnal dyspnea (from increase pulmonary venous pressure –> pulmonary venous distention and transudation of fluid. Presence of hemosiderin-lade MO in the lungs)
Orthopnea –> increase venous return in supine position exacerbates pulmonary vascular congestion
Signs of right heart failure
Hepatomegaly (nutmeg liver) –> increase central venous pressuer –> increase resistance to portal flow –> rarely leads to cardiac cirrhosis
Peripheral edema –> increase venous pressure –> fluid transudation
Jugular venous distention from increase venous pressure
Bacterial endocarditis symptoms
Fever, Roth’s spots (round white spots on retina surrounded by hemorrhage), Osler’s nodes (tender raised lesions on finger or toe pads), new murmur, Janeway lesion (small, painless, erythematous lesions on palm or sole), anemia, splinter hemorrhages on nail bed (FROM JANE)
Bacterial endocarditis acute cause
S. aureus (high virulence)
Large vegetations on previously normal valves (rapid onset)
Bacterial endocarditis subacute
Viridans streptococci (low virulence) Smaller vegetations on congenitally abnormal or diseased valves. Sequela of dental procedures, more insidious onset
Non-bacterial endocarditis causes
Secondary to malignancy, hypercoaguable state, lupus (marantic/thrombotic endocarditis).
Other bacterial causes of endocarditis
S. bovis is present in colon cancer
S. epidermidis on prosthetic valves
What valve is most frequently involved in bacterial endocarditis?
Mitral valve
Tricuspid valve endocarditis seen in
IVDA - S. aureus, Pseudomonas, Candida
Complications of endocarditis
chordae rupture, glomerulonephritis, suppurative pericarditis, emboli
Rheumatic fever
Cause:
Early:
Late sequalae:
Consequence of pharyngeal infection with group A beta-hemolytic streptococci. Early deaths due to myocarditis.
Early: mitral valve regurg
Late sequelae: rheumatic heart disease (affects valves mitral > aortic»_space; tricuspid), mitral stenosis
Lab/microscopic findings of Rheumatic fever
Aschoff bodies (granuloma with giant cells), Anitschkow's cells (activated histiocytes), elevated ASO titers Immune mediated (type II hypersensitivity) - not a direct effect of bacteria but Antibodies to M protein
Sx of Rheumatic fever
FEVERSS Fever Erythema marginatum Valvular damage (vegetation/fibrosis) ESR increase Red-hot joints (migratory polyarthritis) Subcutaneous nodules Sydenham's chorea
Acute pericarditis
S/Sx
Three causes
Commonly presents with sharp pain, aggravated by inspiration ad relieved by sitting up and learning forward. Friction rub, ECG changes - widespread ST segment elevation and or PR depression
Causes:
1. Fibrinous: caused by Dressler’s syndrome, uremia, radiation, loud friction rub
2. Serous: viral (resolves spontan), noninfectious inflamm dz (RA, SLE)
3. Suppurative/purulent: usually caused by bacterial infxns (pneumo, strepto)
Cardiac tamponade
Compression of heart by fluid in pericardium, leading to decrease CO
Equilibration of diastolic pressures in all 4 chambers
Findings: hypoTN, increase venous pressure (JVD), distant heart sounds, increase HR, pulsus paradoxus
Pulsus paradoxus
decrease in amplitude of systolic blood pressure by >10 mmHg during inspiration. Seen in severe cardiac tamponade, asthma, obstructive sleep apnea, pericarditis, croup
Syphilitic heart disease
Tertiary syphilis disrupts the vasa vasorum of the aorta with consequent atrophy of the vessel wall and dilation of the aorta and valve ring.
May see calcification of the aortic root and ascending aortic arch –> leads to tree bark appearance of the aorta
Can result in aneurysm of the ascending aorta or aortic arch and aortic insufficiency
Myxoma
most common primary tumor in adults
90% occur in the atria (left atrium)
Ball valve obstruction in the left atrium (multiple syncopal episodes
Rhabdomyomas
most frequent primary cardiac tumor in children (associated with tuberous sclerosis)
Most common heart tumor is
A metastasis from melanoma, lymphoma
Kussmaul’s sign
Increase in JVP on inspiration instead of a normal decrease
Inspiration –> negative intrathoracic pressure not transmitted to heart –> impaired filling of right ventricle –> blood backs up into venae cavae –> JVD
Seen with constrictive pericarditis, restrictive cardiomyopathies, right atrial or ventricular tumors, or cardiac tamponade
Raynaud’s phenomenon
decreased blood flow to the skin due to arteriolar vasospasm in response to cold temperature or emotional stress
most often in fingers and toes
Raynaud’s disease
when the phenomenon is idiopathic or primary
Raynaud’s syndrome
when the phenomenon is secondary to a dz process such as mixed connective tissue disease, SLE, or CREST
Temporal arteritis
Clinical: pt population, can lead to, associated with
Morph:
Tx
Giant cell (large vessel vasculitis)
Clinical: elderly female, unilateral headache and jaw claudication; may lead to irreversible blindness due to ophthalmic artery occlusion
Associated with polymyalgia rheumatica
Morph: branches of carotid artery, focal granulomatous inflammation, increase ESR
Tx with high dose corticosteroids
Takayasu’s arteritis
Clinical: pt population, can lead to
Morph:
Tx
Large-vessel arteritis
Asian female <40, pulseless disease (weak upper extremity pulses), fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances
Granulomatous thickening of aortic arch, proximal great vessels, increase ESR
Tx with corticosteroids
Polyarteritis nodosa
Clinical: pt population, can lead to, associated with
Morph:
Tx
Medium-vessel vasculitis
Young adults; Fever, weight loss, malaise, headache, ab pain, melena, HTN, neurologic dysfxn, cutaneous eruptions, renal damage; Hepatitis B seropositivity in 30% of patients
Renal and visceral vessels, immune complex mediated, transmural inflammation of the arterial wall with fibrinoid necrosis, lesions of diff ages, many aneurysms and constrictions on arteriogram
Tx with corticosteroids, cyclophosphamide
Kawasaki disease
Clinical: pt population, can lead to, associated with
Morph:
Tx
Medium vessel vasculitis
Asian children MI, rupture
Tx with IV Ig and aspirin
Buerger’s disease (thromboangiitis obliterans)
Clinical:
Morph:
Tx:
Heavy smokers, males s phenomenon often present
Segmental thrombosing vasculitis
Tx with smoking cessation
Microscopic polyangiitis
Clinical:
Morph:
Tx:
Necrotizing vasculitis commonly involving lung, kidneys, and skin with pauci-immune glomerulonephritis and palpable purpura
No granulomas, p-ANCA
Tx with cyclophosphamide and corticosteroids
Wegener’s granulomatosis (granulomatosis with polyangiitis)
Clinical:
Morph:
Tx:
Upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis; Lower respiratory tract: hemoptysis, cough, dyspnea; Renal: hematuria, red cell casts
Triad of: focal necrotizing vasculitis, necrotizing granulomas in the lung and upper airway, necrotizing glomerulonephritis
c-ANCA; CXR shoes large nodular densities
Tx cyclophosphamide, corticosteroids
Churg Strauss syndrome
Clinical:
Morph:
Tx:
Asthma, sinusitis, palpable purpura, peripheral neuropathy (wrist/foot drop)
Can also involve heart, GI, kidneys (pauci-immune glomerulonephritis)
Granuloamtous, necrotizing vasculitis with eosinophilia
p-ANCA, elevated IgE
Henoch-Schonlein purpura
Clinical:
Morph:
Tx:
MC childhood systemic vasculitis
Follows URI
Triad: skin palpable purpura on buttocks/legs, arthralgia, ab pain/melena/multiple lesions of same age
Vasculitis secondary to IgA complex deposition
Associated with IgA nephropathy
Strawberry hemangioma
Benign capillary hemangioma of infancy. Appears in first few weeks of life (1/200 births); grows rapidly and regresses spontaneously in 5-8 years of age
Cherry hemangioma
Benign capillary hemangioma of the eldery; does not regress; frequency increases with age
Pyogenic granuloma
Polypoid capillary hemangioma that can ulcerate and bleed. Associated with trauma and pregnancy
Cystic hygroma
Cavernous lymphangioma of the neck. Associated with Turner syndrome
Glomus tumor
Benign, painful, red-blue tumor under fingernails. Ariese from modified smooth muscle cells of glomus body
Bacillary angiomatosis
Benign capillary skin papules found in AIDS patients. Caused by Bartonella henselae indfections. Frequently mistaken for Kaposi’s sarcoma.
Angiosarcoma
Rare blood vessel malignancy typically occurring in the head, neck, breast areas. Associated with patient receiving radiation thearpy, especially for breast cancer and Hodgkin’s lymphoma. Very aggressive and difficult to resect due to delay in dx
Lymphangiosarcoma
Lymphatic malignancy associated with persistent lymphedema (post-radical masectomy)
Kaposi’s sarcoma
Endothelial malig most commonly of the skin but also mouth/GI tract/respiratory tract. Associated with HHV-8 and HIV. Frequently mistaken for bacillary angiomatosis
Sturge-Weber disease
Congenital vascular disorder that affects capillary sized blood vessels. Manifests with port-wine stain (nevus flammeus) on face, ipsilateral leptomeningeal angiomatosis (intracerebral AVM), seizures, and early-onset glaucoma
Affects small vessels
