Cardiomyopathy and anti-arrythmatic Flashcards

1
Q

What are the features of the heart in dilated cardiomyopathy?

A

progressive cardiac dilation
systolic dysfunction
hypertrophy
enlarged, heavy flabby heart

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2
Q

What are common causes of dilated cardiomyopathy?

A
ischemia
valvular
HTN
idiopathic
familial
inflammmatory-infectious
toxic
metabolic
nutritional
neuromusc
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3
Q

What percentage of dilated myopaties are indicated by genetic casues?

A

20-50% majority autosomal dominant mutations encoding cytoskeleton proteins

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4
Q

What viruses have been linked to dilated cardiomyopathy?

A

coxasckie B or echovirus, self limited in young ppl

unclear mecchanism

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5
Q

What two ways can alcohol cause dilated cardiomyopathy?

A

alcohol: direct toxic effect myocardium
alcohol: indirect toxic effect associated with thiamine disease (beriberi disease0

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6
Q

What chemotherapy drug can lead to dilated cardiomyopathy

A

doxorubicin as well as other anthracyclines, which is dose dependent

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7
Q

What is the dilated cardiomyopathy due to pregnancy?

A
peripartum cardiomyopathy; late in pregnancy or several weeks to months posptartum;
probable cause
HTN
volume overload
nutritional def
prolactin
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8
Q

What is the dilated cardiomyopathy gross morphology?

A
four chamber dilation
variable thickness
mural thrombi
valves are normal, but may have tricuspid or mtiral regurg
cardiomegaly
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9
Q

What is the histology associated with dialted cardiomyopathy?

A

myocyte hypertrophy
interstital & endocardial fibrosis; no necrsois
not specific for DCM unless iron overload is cause adn iron present

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10
Q

What puts you at increased risk for idiopathic dilated cardiomyopathy

A

black
male
HTN
chronic Beta-agonist; relatively rare

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11
Q

How does idiopathic dilated cardiomyopathy usually present?

A

heart failure symtpoms
or anginal chest pain
occasionally sudden death

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12
Q

What are the treatmments for dilated cardiomyopahty?

A
ACE inhibitor, other diuretics
potassium/magnesium
beta blocker
digoxin in face of persistnent syndromes
wafarin-with thrombus/afib
ICD- MI oruncontrolled vT
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13
Q

What is the definition of arrhythmogenic right ventricular cardiomyopathy?

A

inherited disease of cardiac muscle
right ventricle failure and various rythm disturbance
sudden death possible

–right ventricle is severely thinned bc of loss of myocytes with extensive fatty infiltration and fibrosis—

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14
Q

What is the inheritance of arrhythmogenic right ventricular cardiomyopathy?

A

autosomal dominant

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15
Q

Where is the defect in arrhythmogenic right ventricular cardiomyopahty?

A

defective cell adhesion proteins in desmosomes that link adjacent cardiac myocytes

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16
Q

What is the clinical syndrome associated with arrhythmogenic right ventricular cardiomyopathy?

A

young adults
arrhythmia (VTach)
sudden death
right ventricular failure

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17
Q

What is the morphology of arrhythmogenic right ventricular cardio?

A

thin & dilated right ventricular wall with fatty infiltration (essential feature) and intersitial fibrosis

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18
Q

What is hypertrophic cardiomyopathy?

A

myocardial hypertrophy, with abnormal diastolic filling, poorly complian
heart is thick-walled, heavy and hypercontracting
priimarily diastolic dysfunction, systolic functio usually perserved
ventricular outflow obstruction

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19
Q

What is the gross morphology of HCM

A

massive myocardial hypertrophy, frequent disporportionate thickening of septum
endocardial thickening and mural plaques in outflow tract

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20
Q

What is the histology of HCM?

A

marked hypertrophy
intersitial fibrosis
myofiber disarray
intersitial fibrosis

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21
Q

What are the mutations associated with HCM?

A

increase myofilament activation resutling in myocyte cotnractility

missesnse in sarcomeric proteins
myosin heavy chain
myosin binding protein
cardiac TnT

DEFECTS IN DIRECT SARCOMERE FUNCTION
DEFECT IN TRANSFER OF ENERGY TO SARCOMERE

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22
Q

What is the genetic inheritance of HCM?

A

prognosis correlated with mutation
familiail, AD, Variable expression
one of the most common causes of sudden unexplained death in athletes

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23
Q

What are the arrythmias associated with HCM?

A

paroxysmal supraventricular arrthmias
Afib
Non-sustained ventricular tachy
sustained ventricular tachy/ventricular fibrillation

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24
Q

What are the clinical features of HCM?

A

presentation after puberty
asymptomatic frequently
sudden death in young athletes
syncope due to left ventricular outflow obstruction

Tx: surgical excision, ventricular relaxing drugs

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25
Q

What are the risk facctors for cardiac death in HCM?

A
marked ventricular hypertrophy
young age
hypotension on excercise
syncope
hx of aborted cardiac rest
FHx of sudden cardiac death
certain geneti mutations
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26
Q

What is used to treat HCM

A

Beta blockers

27
Q

What is a dual chamber pacemaker used for?

A

HCM, used in pts with sxs who cant tolerate surgical therapy

28
Q

How do you prevent sudden cardiac death in HCM pts?

A

pts at high risk receive implantable cardioverter-defibrillators

29
Q

What is restrictive cardiomopathy?

A

primary deccrease in ventricular compliance resulting in impaired ventricular during

-idiopathic
-secondary to
-post radiation fibrosis
-amyloidosis
sarcoidosis
metastases
inborn error of metabolism

30
Q

What is restrictive cardiomyopathy morphology gross?

A
  • normal ventricles
  • bi-atrial dilation
  • firm myocardium
  • interstital fibrosis
31
Q

What si restrictive cardiomyopathy histology?

A

patchy or diffuse intersitial fibrosis and disease specific changes

32
Q

What is the clinical features of RCM?

A
congestive heart failure
--severe pulmonary congestion
--hepatic congestion
similar S/S as constricitve pericarditis
Dx: endomyocardial biopsy
33
Q

What happens in senile cardiac amyloidosis?

A

transthyretin (prealbumin)-deposits in ventricles and atria
>60 yrs of age
AA majority

34
Q

What are the restrictive hemodynamics?

A

asymptomatic
pressure atrophy of fibers
deposition in regions of conduction system leads to arrhythmiass

35
Q

Loeffler endomyocarditis?

A

endomyocardial fibrosis, large mural thrombi, peripheral eosinophilia, eosinophilic infiltrates in organs

36
Q

Endocardial fibroelastosis is indicated by what?

A

common first two years of life, in young children in africa, focal or diffuse fibroelatic thickening

37
Q

What can hyperthyroidism cause with respect to the heart?

A

tachy, palpitations and cardiomegaly

38
Q

What can hypothyroidism cause with respect to the heart?

A

CO is decreased
flabby, enlarged and dilated
myofiber swelling

39
Q

What is the effect of catecholamines on the heart when induced either in intesnse stress or froma pheochromocytoma?

A

takotsubo cardiomyopathy

40
Q

What is afterdepolarizations?

A

spontaneous action potentials during or immediately after phase 3 repolarization. Produced by abnormal Ca2+ influx during or after phase 3 of ventricular action potential

leading to premature contractions and ventricular tachycardia

observeed with digoxin toxicity and conditions that prolong QT interval

41
Q

Quinidine therapy results in what?

A

action potential prolognation increased QT duration, early afterdepolarizations and torsade de pointes

42
Q

Class 1 are sodium channel blockers do what?

A

bind fast sodium channels in the open and inactivated states and dissociate from channels during resting state

43
Q

What is in ddrug class 1a?

A

quinidine, procainamide, disopyramide

44
Q

What is the drug dissociationrate of drug class 1a?

A

slow

45
Q

What is the drug dissociation of drug class 1B?

A

lidocaine, mexilitene; rapid

46
Q

What is drug dissociation rate of class 1C?

A

very slow

47
Q

What is drug class 1a mechanism of action?

A

slow phase 0, and conduction velocity and prolong action portential duration and refactory period

proarrhythmic

48
Q

Class 1b drugs does what?

A

greater affinity for inactivated vs open channel

49
Q

What is special about lidocaine?

A

extensive first pass hepatic inactivation; more active in ischemic tissue due to less negative resting membrane, minimal effect on normal cardiac tissue

50
Q

What is class 1c drugs?

A

block fast sodium chanels more potently than class 1 agents, slow conduction throughout heart, less effect on K+ current

51
Q

What is flecainide used to treat

A

SVT and VT,

may inhibit Ca2+ releases from cardiac SR

52
Q

What is propafenone side effects?

A

ventricular proarrhythmia, agranulocytosis, anemia, thrombocytopenia

53
Q

What are class II Beta-blockers do?

A

inhibit sympathetic activation of cardiac autmoaticity and conduction

effect mainly occurs at AV node refactory period;

54
Q

What are class III anti-arrhythmic drugs?

A

block potassium recitifier, demonstrate reverse-use dependence

with more potent Kv block occuring at slower heart rates.

55
Q

What is amiodarone?

A

iodinated compound, half life approx 40 days

decrease SA node automaticity, decrease AV conduction velocity

56
Q

What are the side effects of amiodarone?

A

hypothyroidism, hyperthyroidism, neulogic toxicity, and more

QT prolongation

57
Q

What does amiodarone block?

A

Ikr and Iks

58
Q

What does sotalol do?

A

selectively blocks beta-adrenoreceptors and delayed recitifier potassium channel

slow AV conduction velocity

blocks Ikr

59
Q

What does dofetilide do?

A

block rapidly activating delayed recitifier channel,may prolong QT and produce TdP; blocks Ikr

60
Q

What does class 4 drugs do?

A

block voltage gated L type Ca2+ channels

decrease AV node conduction

61
Q

What is adenosine?

A

elimination half life rapid, decrease action potential duration and hyperpolarizing membrane potential in atrium SA and AV node

62
Q

What does digoxin do?

A

Na-K-ATPase membrane pump,; increase itnracellular Na+ cocentration. Increase through automatic nervous system

63
Q

What does class I and III act through which cells?

A

ventricular action potential

64
Q

What does calss II and IV drugs act through cells?

A

SA node