Pathology - cardiac Flashcards
Right-to-left shunts in early cyanotic babies
5 T's Truncus arteriosus (1 vessel) Transposition (2 switched vessels) Tricuspid atresia (3 = tri) Tetralogy of Fallot (4 = tetra) TAPVR (anomalous pulmonary venous return) (5 letters)
Generally caused by defects in aorticopulmonary septum (derived from neural crest)
Tetralogy of Fallot pathology
Caused by anterosuperior displacement of the infundibular septum
Four defects: pulmonary stenosis, RVH, overriding aorta, VSD
Pulm stenosis causes right-to-left shunting across VSD and RVH
Squatting increases systemic resistance, decr. R-to-L shunt, improves cyanosis
Late cyanosis in babies
VSD > ASD > PDA
ASD pathology
usually ostium secundum defects
distinct from PDA: pathology is missing tissue rather than unfused septa
End-stage effects of Eisenmenger syndrome
Late cyanosis, clubbing**, polycythemia
Associations with coarctation of the aorta
Bicuspid aortic valve, Turner syndrome (45 X)
aortic narrowing near insertion of ductus arteriosis (juxtaductal)
Look for delayed pulses in the lower extremities
Hypertension pathology
Primary (essential) - 90%, related to incr. CO or TPR
Secondary - 10%, related to renal/renovascular dz, primary hyper-aldosterone
Cause of hypertension in young woman
Fibromuscular dysplasia - string of beads in renal artery
Hypertensive nephropathy
Renal arterial hyalinosis
Evidence of end-organ damage in hypertensive emergency
Encephalopathy, stroke, retinal hemorrhages/exudates, papilledema, MI, HF, aortic dissection, kidney injury, MAHA, eclampsia
Hyperlipidemia signs
Xanthoma/xanthelesma - lipid-laden histiocytes in the skin
Tendinous xanthoma - seen particularly in Achilles
Corneal arcus - common in elderly
All appear in familial hypercholesterolemia
Arteriosclerosis
Arterial wall thickening and loss of elasticity
Includes arteriolosclerosis and medial calcific sclerosis
Arteriolosclerosis (two types)
Hyaline - thickening of vessel walls, seen in essential HTN/DM
Hyperplastic - proliferation of smooth muscle cells (onion skinning), seen in severe HTN
Medial calcific sclerosis
calcification of elastic lamina or arteries
Leads to vascular stiffening without obstruction
Intima not involved!
Atherosclerosis pathology progression
endothelial cell dysfunction –> macrophage/LDL accumulation –> foam cell formation –> fatty streaks –> smooth muscle migration (PDGF/FGF), ECM deposition –> fibrous plaque –> complex atheroma –> arterial occlusion or plaque rupture
Look for cholesterol crystals
Aortic aneurysm (abdominal vs. thoracic)
Localized pathologic dilatation of aorta
Abdominal: assoc. with atherosclerosis, older men
Thoracic: cystic medial degeneration, assoc. with bicuspid aortic valve, Marfan, tertiary syphillis (obliterative endarteritis)
Aortic dissection
intimal tear forming a false lumen
end results: rupture, pericardial tamponade, death
Type A: Ascending aorta, proximal, treat with surgery
Type B: involves descending aorta, treat with b-blockers then vasodilators
Coronary steal syndrome
phenomenon where administration of vasodilators (CCBs) dilates normal vessels and shunts blood to well-perfused areas, leading to decreased flow and ischemia in post-stenotic region
Pathologic evolution of MI
4-24 hrs
early coagulative necrosis, neuts appear, contraction bands with reperfusion
Pathologic evolution of MI
1-3 days
Extensive coagulative necrosis
Tissue surrounding infarct shows acute inflammation with neutrophils
Watch for pericarditis
Pathologic evolution of MI
3-14 days
Macrophages, then granulation tissue (thus, mac-mediated structural degradation)
Watch for free wall rupture (tampondade), pap muscle rupture (MR), septal rupture
LV pseudoaneurysm also appears
Pathologic evolution of MI
2 weeks - months
Contracted scar
Dressler syndrome: post-MI pericarditis (pleuritic chest pain, pericardial rub/effusion), autoimmune reaction to myocardial neo-antigens
True ventricular aneurysm (risk of mural thrombus)
MI diagnosis (ECG, markers)
ECG: gold standard in first 6 hours
troponin-I: rises after 4 hours, high for 7-10 days, more specific
CK-MB: rises after 6-12 hours (returns to normal in 48 hrs), found in myocardium, can be released from skeletal muscle, useful for reinfarction
Transmural vs. subendocardial infarcts
Transmural: incr. necrosis, affects entire wall, ST elevation
Subendocardial: less than 50% of wall affected, especially vulnerable to ischemia, ST depression
Dilated cardiomyopathy
Eccentric hypertrophy (cells added in series)
Alcohol, wet Beriberi, Coxsackie b, Cocaine use, Chagas, Doxorubicin tox
Look for S3, balloon appearance on CXR
Tx: Na restriction, ACEis, BBs, diuretics
Hypertrophic cardiomyopathy
Mostly familial (B-myosin heavy chain mutation)
Assoc. with Friedrich ataxia
Look for S4, mitral regurg (impaired valve closure)
Tx: BBs, CCBs (verapimil), ICD if high-risk
Path: myofibrillar disarray and fibrosis
can be obstructive
Restrictive/infiltrative cardiomyopathy
Sarcoid/amyloid, postradiation fibrosis, hemochromatosis
Loffler syndrome (endomyocardial fibrosis with a prominent eosinophilic infiltrate)
Leads to diastolic dysfunction
Systolic vs. diastolic dysfunction
systolic: reduced EF, incr. EDV, decr. contractility
diastolic: preserved EF, normal EDV, decr. compliance
Decreased mortality in HF
ACEis/ARBs
BBs
Spironolactone
Hemosiderin-laden macrophages in the lungs
Sign of pulmonary edema
Four types of shock
Hypovolemic (decr. preload)
Cardiogenic (decr. CO)
Obstructive (decr. CO)
Distributive (decr. SVR)
Bacterial endocarditis findings
Fever
Roth spots (white spots on retina)
Osler nodes (finger/toe pads),
Mitral valve/murmur
Janeway lesions (red lesions on palms/soles)
Anemia
Nail splinter hemorrhages
Emboli
acute endocarditis etiology
S. aureus (high virulence)
Rapid onset, large vegetations on normal valves
chronic endocarditis etiology
viridans streptococci
Smaller vegetations on damaged valves
Dental procedure sequela
tricuspid valve endocarditis
S. aureus, Pseudomonas, Candida
culture negative endocarditis
Coxiella, Bartonella, HACEK (h. flu, actinobacillus, cardiobacterium, eikenella, kingella)
Rheumatic fever
Consequence of pharyngeal infection with GABHS, type II hypersensitivity (Abs to M protein cross-react)
Early: MVR, progresses to MS
Joints (migratory polyarthritis) Heart sign Nodules Erythema marginatum Sydenham chorea
Tx: penicillin
Acute pericarditis
Sharp pain, aggravated by inspiration
Causes: coxsackie, idiopathic, autoimmune (Dressler, SLE, RA), radiation therapy
Tamponade findings
Equilibration of diastolic pressure in all four chambers
Beck triad: hypotension, distended neck veins, distant heart sounds
low-voltage QRS and electrical alternans
Cardiac tumors
Myxomas - ball valve obstruction in left atrium, early diastolic tumor plop sound
Rhabdomyomas - Most frequent primary tumor in children (assoc. with tuberous sclerosis)
Kussmaul sign
- increase in JVP with inspiration (normally there’s a decrease)
Impaired filling of RV with inspiration due to constrictive pericarditis, restrictive cardiomyopathies, right atrial/ventricular tumors
Vascular tumors assoc. with elderly
Angiosarcoma - rare, malignancy, if hepatic then assoc. with vinyl chloride/arsenic, very aggressive
Cherry hemangioma - benign, capillary, does not regress
Vascular tumors assoc. with AIDS
Bacillary angiomatosis - capillary skin papules, caused by Bartonella henselae, neutrophilic infiltrate
Kaposi sarcoma - endothelial malignancy, assoc. with HHV-8, HIV, lymphocytic infiltrate
Vascular tumors assoc. with pregnancy or childhood
Pyogenic granuloma - pregnancy, can ulcerate and bleed
Strawberry hemangioma - grows rapidly, regresses spontaneously by 5-8 yo
Raynaud phenomenon
decr. blood flow to skin due to arteriolar vasospasm in response to cold/stress
syndrome occurs when 2ry to SLE, CREST, mixed connective tissue dz
Tx: CCBs
Large-vessel vasculitis
Temporal arteritis - elderly females, can lead to blindness due to ophthalmic artery occlusion, assoc. w/ polymyalgia rheumatica
Takayasu arteritis - aortic arch syndrome, weak UE pulses, granulomatous thickening of aortic arch
Medium-vessel vasculitis
Polyarteritis nodosa - young adults, Hep B, const. sx, renal/visceral vessels (microaneurysms), immune complex mediated, tx: cyclophosphamide
Kawasaki dz - CRASH and burn (conjunctiva, rash, adenopathy, strawberry tongue, hand-foot changes, fever, can develop coronary artery aneurysms
Beurger dz - heavy smokers under 40, male, intermittent claudication, loss of digits, often seen w/ Raynaud, tx = quit smoking!
ANCA assoc vasculitidies
GPA (c-ANCA, anti PR3) - nasal septum perforation, chronic sinusitis, hemoptysis, hematuria, red cell casts, large pulmonary nodules on CXR
Microscopic polyangiitis (p-ANCA, anti MPO) - necrotizing vasculitis involving lungs, kidneys, skin. No nasal involvement.
EGPA (p-ANCA, anti MPO) - asthma, sinusitis, peripheral neuropathy, look for eosinophilia
Henoch-Schonlein Purpura
Follows URI, triad: skin (palpable purpura), arthralgias, GI (ab pain)
secondary to IgA complex deposition, assoc with IgA nephropathy
Familial chylomicronemia syndrome
Type 1
defect in lipoprotein lipase and ApoC-II
risk of pancreatitis
Familial hypercholesterolemia
Type 2a
defect in LDL receptor, ApoB-100
(lack of uptake by liver –> incr. LDL serum)
premature coronary artery disease
Familial dysbetalipoproteinemia
Type 3
ApoE defect
premature coronary artery disease
Familial hypertriglyceridemia
Type 4
ApoA-V defect
