Cardiac Pathology Part II Flashcards
what is the most common cause of arrhythmia?
ischemic heart disease aka MI
what are other causes of arrhythmia?
cardiomyopathies
myocarditis
valvular disease
congenital disorders
what is sick sinus syndrome?
damage to the SA node causing bradycardia
*AV node becomes pacemaker of the heart
what is atrial fibrillation?
independent and sporadic atrial myocyte depolarization with variable transmission to the AV node
irregularly irregular rhythm
what risks are associated with Afib?
thrombus formation and subsequent stroke
what is a heart block?
dysfunction at the AV node
what is the most severe heart block?
third degree
complete failure of AV node
what can be seen on ECG with a first degree heart block?
prolonged PR interval
what is the primary cause of hereditary arrhytmias?
ion channel disfunction (channelopathies)
K+ and Na+
what is the typical presentation of hereditary channelopathies?
sudden death after exertion
what is another name for hereditary channelopathies?
Long QT syndrome
what can occur in patients with long QT syndrome?
Torsades de Pointes
what is the most common cause of sudden death due to ischemia induced arrhythmia?
coronary artery disease
what can cause sudden cardiac death in younger patients?
drug abuse (cocaine and meth) hereditary arrhythmias cardiomyopathies myocardial hypertrophy myocarditis mitral valve prolapse
what changes in the heart occur with untreated hypertension?
left ventricular concentric hypertrophy
can lead to diastolic dysfunction –> CHF and Afib
what causes right sided hypertensive heart disease?
pulmonary hypertension as a result of:
pulmonary parenchymal disease
pulmonary vessel disease
disorders of chest movement
what changes occur in the heart as a result of pulmonary hypertension?
right ventricular hypertrophy
hypertrophied trabeculae
tricuspid stenosis
what is the most common valve abnormality?
calcific aortic stenosis
what is the demographic of calcific aortic stenosis?
60+ y/o with HTN, high cholesterol or chronic inflammation
what is the risk associated with bicuspid aortic valves?
accelerated course of calcific aortic stenosis
may cause aortic valve dilation or prolapse
bacterial endocarditis is more frequent
what cardiac changes occur with calcific aortic stenosis?
increased LV pressure resulting in concentric LV hypertrophy
what is the clinical presentation of calcific aortic stenosis?
systolic murmur
angina
syncope
CHF
tx: valve replacement
what risks are associated with mitral annular calcification?
more common in females 60+
regurgitation
stenosis
arrhythmias
prone to thrombus and infective endocarditis
what is mitral valve prolapse?
valve leaflets prolapse back into the left atrium during systole
“floppy valve”
what causes mitral valve prolapse?
female predominance
can occur spontaneously without cause
connective tissue disease
complication of MI or rheumatic fever
what would be seen morphologically in mitral valve prolapse?
thickened, rubbery leaflets
interchordal ballooning/hooding of the leafets
what would be seen on histology in mitral valve prolapse?
proteoglycan deposition
myxomatous degeneration
what is the clinical presentation of mitral valve prolapse?
mid-systolic click +/- murmur
dyspnea due to regurgitation
what are rare complications that can occur with MVP?
infective endocarditis
mitral insufficiency
arrhythmias
thromboembolism
what is rheumatic fever?
multisystem inflammatory disorder caused by group A strep pharyngitis
what is the immunological mechanism of rheumatic fever?
antibodies and CD4 cell reaction against M streptococcal antigen
causes reaction to self antigens in heart, joints, soft tissue, skin and nervous system
what is the clinical presentation of rheumatic fever?
fever migratory polyarthritis pancarditis subcutatneous nodules erythema marginatum syndeham chorea
what is acute rheumatic heart disease?
pericarditis, myocarditis or endocarditis (pancarditis if all affected)
valvulitis with vegetation (verrucae formation)
MacCallum plaques
what would bee seen on histology in rheumatic heart disease?
Aschoff bodies with Anitschkow cells
what valves are primarily affected in rheumatic heart disease?
mitral > aortic > tricuspid
MAT
what is chronic rheumatic heart disease?
valvular thickening, short chordae tendinae, fusion and regurgitation
valvular stenosis
what is the clinical presentation of chronic rheumatic heart disease?
mitral stenosis causing diastolic rumbling murmur
what complications are associated with chronic rheumatic heart disease?
infective endocarditis
mitral stenosis –> left atrial enlargement –> thromboembolism
what causes infective endocarditis?
infectious organism, inflammation or fibrinous debris
where does infective endocarditis typically affect the heart?
valves or structural abnormalities
what risk factors are associated with infective endocarditis?
drug use body piercings male gender (haha) poor dentition invasive dental procedures pre-existing heart conditions
what pre-existing conditions are pre-disposed to infective endocarditis?
valvular disease
prosthetic heart valve
structural heart disease
what causes are associated with left sided infective endocarditis?
structural valve abnormalities
poor dentition/dental procedure
prosthetic valves
what organism is the cause of infective endocarditis in valvular abnormalities?
Strep viridans
what organism is the cause of infective endocarditis in poor dentition/dental procedures?
HACEK group: hemophilus actinobacillus cardiobacterium eikenella kingella
what organism is the cause of infective endocarditis in prosthetic valves?
staph epidermidis
what is the only cause of right sided infective endocarditis?
IV drug abuse
what organism is the cause of infective endocarditis in IV drug abuse?
staph aureus
what is the clinical presentation of acute infective endocarditis?
rapid onset fever chills and weakness subungual splinter hemorrhages (nails) Janeway lesions (palms and soles) osler nodes (hands) roth spots (eyes)
what is nonbacterial thrombotic endocarditis?
sterile, non-inflammatory valvular thrombi that is asymptomatic until embolization occurs
what causes nonbacterial thrombotic endocarditis?
sepsis
cancer
antiphospholipid syndrome
SLE (Libman-Sacks endocarditis)
what is carcinoid heart disease?
compounds secreted by carcinoid tumors induce plaque-like endocardial and valvular thickening
what part of the heart is affected by carcinoid heart disease?
right sided valves and endocardial tissue
left side is protected by pulmonary vascular degradation of compounds
what is the clinical presentation of carcinoid heart disease?
flushing
diarrhea
dermatitis
bronchoconstriction
what can be seen on histology in carcinoid heart disease?
plaque-like thickening with mucopolysaccharide deposition
what is a potential complication in someone with a prosthetic valve (mechanical or biotic)?
infective endocarditis
anticoagulant related hemorrhage
dysfunction or exuberant healing
hemolytic anemia
what condition is associated with a harsh systolic murmur?
calcific aortic stenosis
what conditions are associated with a holosystolic murmur?
mitral regurgitation
VSD
what condition is associated with a diastolic decrescendo murmur?
aortic regurgitation
what condition is associated with a diastolic rumbling murmur?
rheumatic heart disease (mitral valve stenosis)
what condition is associated with a continuous machine-like murmur?
PDA
what does cardiomyopathy mean in general?
heart muscle disease
what are the three major types of cardiomyopathy?
- dilated (most common)
- hypertrophic
- restricted (least common)
what causes dilated cardiomyopathy?
AD hereditary TTN gene peripartum cardiomyopathy alcoholism with wet beri-beri myocarditis cardiotoxic drugs hemochromatosis
what morphology is associated with dilated cardiomyopathy?
dilation of all heart chambers
hypertrophy without wall thickening
functional valve regurgitation
what is the clinical presentation of dilated cardiomyopathy?
20-50 y/o progressive CHF with decreased EF systolic dysfunction arrhythmias thromboembolism
what is takotsubo cardiomyopathy?
broken heart syndrome :(
associated with sudden surge of catecholamines due to emotional distress
most likely to occur in women because we are emotional AF
what would be seen on CXR in someone with takotsubo cardiomyopathy?
apical ballooning of left ventricle
looks like Japanese fishing pot!
what is arrhythmogenic right ventricular cardiomyopathy (ARVC)?
AD hereditary disorder with defective cell adhesion proteins in the desmosomes that link adjacent cardiac myocytes
what morphological changes are associated with ARVC?
right ventribular wall is replaced by adipose and fibrosis causing right ventricular failure and arrhythmia
V tach or V fib causes sudden cardiac death
what is Naxos syndrome?
ARVC with plantar and palmar keratosis and wooly hair
caused by mutation in the desmosome associated protein plakoglobin
what is hypertrophic cardiomyopathy?
GENETIC ONLY
myocyte hypertrophy and disarray with septal prominence
what mutation is most common in hypertrophic cardiomyopathy?
B-MHC
what can be seen morphologically in hypertrophic cardiomyopathy?
septal hypertrophy > ventricular wall hypertrophy
septum and anterior mitral valve obstruct blood flow
what is the classical presentation of hypertrophic cardiomyopathy?
sudden unexplained death in athlete during exercise
what can be seen clinically in hypertrophic cardiomyopathy?
ventricular arrhythmia systolic ejection murmur exertional dyspnea exertional CP palpitations
what is restrictive cardiomyopathy?
decreased ventricular compliance resulting in diastolic dysfunction
what causes restrictive cardiomyopathy?
deposition of material within cardiac walls
amyloid due to amyloidosis
OR
fibrosis due to radiation
what histological findigs are associated with amyloidosis?
apple green birefringence on congo red stain
what is the most common cause of myocarditis?
VIRAL:
coxsackievirus B
what is the clinical presentation of myocarditis?
ranges from asymptomatic to heart failure
may present as arrhythmia causing sudden death
+/- fever
what are other infectious causes of myocarditis?
Chagas disease (trypanosoma cruzi) Trichinosis (pork helminth) Lyme disease (spirochete)
what is more common: lymphocytic or eosinophilic myocarditis?
lymphocytic (viral, autoimmune or idiopathic cause)
what is the prognosis of idiopathic giant cell myocarditis?
very poor prognosis
survival typically less than 3 monst from onset
what are the classic cardiotoxic drugs?
doxorubicin and daunorubicin
cause dilated cardiomyopathy and HF
what is pericardial effusion?
acute or chronic accumulation of fluid within the pericardium
when is pericardial effusion dangerous?
acute onset (<1 week) causes cardiac tamponade
what are the subtypes of pericardial effusions?
hemopericardium
serous effusion
purulent pericarditis
what causes hemopericardium?
trauma
ruptured MI
aortic dissection
what causes serous effusion?
CHF
what causes purulent pericarditis?
necroinflammatory debris
acute inflammation
secondary to infectious process
what is pericarditis?
inflammation of the pericardial sac
what is the clinical presentation of pericarditis?
sharp, pleuritic and position dependent chest pain
pericardial friction rub
pericardial effusion
ECG changes (ST elevation with PR depression)
fever
what is the most common type of pericarditis?
fibrinous/serofibrinous
what is fibrinous/serofibrinous pericarditis?
fibrinous inflammatory exudate with variable amount of serous fluid accumulates within pericardial sac
what causes fibrinous/serofibrinous pericarditis?
Acute MI
Dressler’s syndrome (post-infarction)
uremia (CKD with elevated BUN)
what is seen morphologically in fibrinous/serofibrinous pericarditis?
bread and butter appearance on pericardium
what causes serous pericarditis?
virus
inflammatory disease
what causes purulent/suppurative pericarditis?
active infection by bacterial invasion
what is seen histologically in purulent/suppurative pericarditis?
bacteria
neutrophils
fibrinopurulent debris
what causes caseous pericarditis?
tuberculosis
what causes hemorrhagic pericarditis?
malignant neoplasm
trauma
what is constrictive pericarditis?
heart becomes encased in dense, fibrous or fibrocalcific scar
limits diastolic expansion and cardiac output
mimics restrictive cardiomyopathy
what is the most common cardiac tumor in adults?
myxoma (benign)
what is the most common cardiac tumor in children?
rhabdomyoma (benign)
what is the most common malignant cardiac tumur?
angiosarcoma
what is a cardiac myxoma?
stromal tumor of mesenchymal origin
ranges from globular/hard to gelatinous
where does a myxoma typically occur?
left atrium beginning in the septal region of the fossa ovalis
what is the clinical presentation of myxoma?
"ball-valve" obstruction mechanical valve damage tumor embolization fever and malaise (IL-6 released from tumor) tumor "plop" on ausculation
what familial syndromes are associated with myxomas?
McCune-Albright syndrome (GNAS1) Carney complex (PRKAR1A)
what is a papillary fibroelastoma?
incidental “sea-anemone-like” lesion
usually located on valves
what genes are associated with rhabdomyomas?
TSC1
TSC2
which is more likely: a primary cardiac tumor or metastasis?
metastasis from lung, breast, melanoma or lymphoma
what are potential complications associated with cardiac tumors?
mass effect (limiting cardiac filling) decreased myocardial contractility symptomatic pericardial effusion superior vena cava syndrome production of circulating mediators
what is allograft vasculopathy?
late, progressive, diffusely stenosing intimal proliferation
silent MI – denervation of transplanted heart causes MI without angina
what complications are associated with heart transplant?
infection
malignancy
skin cancers
EBV positive lymphoproliferative disorder
