Cardiomyopathy Flashcards

1
Q

Cardiomyopathy definition

A

group of diseases of the myocardium associated with mechanical &/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation

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2
Q

Cardiomyopathy often lead to

A

to cardiovascular death or progressive heart failure-related disability

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3
Q

Dilated cardiomyopathy

A

Dilatation and impaired contraction of the left or both ventricles.

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4
Q

Hypertrophic cardiomyopathy

A

Left and/or right ventricular hypertrophy, typically involving the IVS

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5
Q

Restrictive cardiomyopathy

A

Restricted filling and reduced diastolic size of either or both ventricles with normal or near-normal systolic function

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6
Q

Arrhythmogenic right ventricular cardiomyopathy (ARD)

A

Progressive fibro-fatty replacement of the right ventricle

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7
Q

Congestive presentation is due to what type of cardiomyopathy?

A

Dilated cardiomyopathy

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8
Q

Describe the ventricle/s in Dilated cardiomyopathy.

A

large chambers + thin/weak muscular wall

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9
Q

Causes of Dilated cardiomyopathy

A

Idiopathic, EtOH, Radiation, Infectious/Acute viral myocarditis, MI, Peripartum, Genetics, Sarcoidosis, Hemochromatosis, Thiamine deficiency (Beriberi), thyrotoxicosis, Toxin Induced: anthryacycline, cobalt, catecholamine,

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10
Q

Toxin-induced causes of Dilated cardiomyopathy

A

EtOH, anthryacycline, cobalt, catecholamine

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11
Q

Dilated Cardiomyopathy is characterized by

A

large weak R/L/Both ventricles w/ impaired systolic function

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12
Q

Patient population of Dilated Cardiomyopathy

A

middle-aged, Male > Females

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13
Q

Annual mortality rate of Dilated Cardiomyopathy

A

12%; complete recovery from DCM is rare

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14
Q

Beriberi

A

Thiamine deficiency + Systolic HF (Dilated Cardiomyopathy)

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15
Q

Primary Dilated Cardiomyopathy

A

DCM of unknown etiology leading to LV dilation and systolic dysfunction

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16
Q

Secondary causes of Dilated Cardiomyopathy most commonly include

A

ischemia, alcoholic, peripartum, post-infectious, viral

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17
Q

Sx of Dilated Cardiomyopathy

A

Pulmonary congestion, dyspnea, orthopnea
Systemic congestion, edema, nausea, abdominal pain, nocturia
Hypotension, tachycardia, tachypnea
Fatigue and weakness

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18
Q

Cardiac S/S of Dilated Cardiomyopathy

A

Cardiac exam reveals -> S3, S4 and murmur of TR and/or MR; Low cardiac output;
Atrial fibrillation, conduction delays, complex PVC’s, sudden death

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19
Q

For Dilated Cardiomyopathy , what would be seen on CXR?

A

enlarged heart, CHF

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20
Q

For Dilated Cardiomyopathy , what would be seen on Electrocardiogram?

A

tachycardia, A-V block, LBBB, NSSTT changes, PVC’s

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21
Q

For Dilated Cardiomyopathy , what would be seen on EKG?

A

left ventricular dilation, global hypo kinesis, low EF

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22
Q

For Dilated Cardiomyopathy, when would cardiac catheterization be done?

A

if age >40, ischemic history, high risk profile, abnormal ECG

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23
Q

Pharmaceutical Treatments for Dilated Cardiomyopathy

A

Diuretics, Beta Blockers, ACE/ARB

Hydralazine/nitrate combination if cannot tolerate ACE/ARB

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24
Q

Pharmaceutical Treatments for Dilated Cardiomyopathy for EF < 35% with Class III/VI HF

A

Spironolactone

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25
Q

Non-Pharmaceutical Treatments for Dilated Cardiomyopathy

A

Limited activity, Na+ restriction, Fluid restriction, transplantation

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26
Q

Pharmaceutical Treatments for Dilated Cardiomyopathy for EF <30%, history of LV thrombus or embolic events

A

Anticoagulation

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27
Q

Non-Pharmaceutical Treatments for Dilated Cardiomyopathy for EF <35% on optimal medical therapy

A

Implantable defibrillators

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28
Q

Most common indication for heart transplant (non-ischemic causes)?

A

Dilated Cardiomyopathy

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29
Q

Procedures to treat Dilated Cardiomyopathy once clotting is apparent

A

LV reshaping

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30
Q

Hypertrophic Cardiomyopathy

A

hypertrophy of the left ventricle with marked variable clinical manifestations morphologic and hemodynamic abnormalities

31
Q

Obstructive Hypertrophic Cardiomyopathy

A

hypertrophied septum interferes w/ the anterior mitral leaflet displacement, both obstruct LV outflow

32
Q

Causes of Hypertrophic Cardiomyopathy

A

Genetic (AD, beta-myosin heavy-chain mutation)

33
Q

The severity of Sx increases in Hypertrophic Cardiomyopathy w/

A

any maneuver that increases the force of contraction or decreases filling of the ventricle

34
Q

What type of Cardiomyopathy is associated w/ sudden cardiac death in young athletes?

A

Hypertrophic Cardiomyopathy; Left ventricular outflow obstruction

35
Q

Myocyte characteristics of DCM?

A

Myocyte injury and fibrosis

36
Q

Atrial fibrillation is seen w/ DCM, why is this?

A

thinning of the atrial septum which contains the SA node -> SA node impairment

37
Q

Why is thromboembolism a problem in DCM?

A

LV dilation, pressure and reduced blood flow -> stagnant blood -> clotting in LV

38
Q

How can you measure LV outflow obstruction?

A

Outflow tract gradient: measure blood flow before and above the LV obstruction

39
Q

Hypertrophic Cardiomyopathy is considered a systolic or diastolic dysfunction?

A

Diastolic (inability to fill, increased filling pressure)

40
Q

Maneuvers/Things that cause worsening of Sx in HCM patients

A

exercise, positive inotropic agents, volume depletion, sudden upright position, tachycardia, valsalva

41
Q

Explain how valsalva causes a worsening of HCM.

A

valsalva -> increased intrathoracic Pressure -> decreases venous return to the heart -> decreases ventricular filling (low preload)

42
Q

Clinical presentation of HCM is typically:

A

asymptomatic

43
Q

Be suspicious of HCM when

A

family member w/ sudden cardiac death at younger age

44
Q

Symptoms of HCM?

A

Dyspnea, Angina, Fatigue, Syncope, Palpitations

45
Q

Cardiac Findings w/ HCM

A

Systolic ejection murmur at left sternal border, Afib, thromboembolism

46
Q

HCM w/ loss of consciousness indicates what treatment?

A

defibrillation

47
Q

Angina w/ HCM is due to

A

reduced blood flow, less blood entering coronaries

48
Q

What maneuvers increase the ejection murmur associated w/ HCM?

A

Valsalva, Standing (decreased venous return)

49
Q

What maneuvers decrease the ejection murmur associated w/ HCM?

A

squatting (increases venous return)

50
Q

Imaging of the heart in HCM pts shows:

A

EKG - LVH

51
Q

negative ECHO in child w/ FMHx of HCM

A

does NOT rule out HCM, serial ECHOs are required up to 20 y/o

52
Q

EKG Findings in HCM

A

Abnormal ST-T’s, giant T wave inversion, abnormal Q, Bundle Branch Block, Left atrial enlargement, Ventricular arrhthymias

53
Q

Management of HCM patients

A

FHx of SCM, exercise testing, avoidance of strenuous exercise, screen family members, genetic testings

54
Q

Pharmacologic Treatment of HCM patients

A

b-blockers, CCB, anti-arrhythmics

55
Q

Non-Pharmacologic Treatment of HCM patients

A

Myomectomy, EtOH septal ablation

56
Q

Beta-blockers are used for treatment of HCM, due to their…

A

Negative Inotropic Effects

57
Q

Restrictive Cardiomyopathy characteristics

A

small ventricular chamber, inability of ventricles to stretch and fill during diastole causing increased ventricular filling P

58
Q

Restrictive Cardiomyopathy is a systolic or diastolic dysfunction?

A

Diastolic

59
Q

Restrictive Cardiomyopathy is most common where

A

tropics, Africa, India, S/C America, Asia

60
Q

Common Causes of Infiltrative Restrictive Cardiomyopathy

A

Amyloidosis, Sarcoidosis, Hemochromatosis

61
Q

Common Causes of Non-Infiltrative Restrictive Cardiomyopathy

A

Idiopathic, Genetics, Radiation, Chemotherapy toxicity

62
Q

Genetic causes of Restrictive Cardiomyopathy

A

Loffler Syndrome (endomyocardial fibrosis + eosinophilic infiltrate), fibroelastosis, Fabry’s

63
Q

Sx of Restrictive Cardiomyopathy

A

JVD, Hepatomegaly, Ascites, weakness, fatigue

64
Q

Restrictive Cardiomyopathy physiology

A

increased diastolic ventricular pressure + decreased ventricular filling –> venous congestion, decreased CO

65
Q

ECHO of Restrictive Cardiomyopathy shows

A

prominent E wave, abnormal mitral inflow -> dilated Left atrium

66
Q

patient with predominantly R-sided heart failure without evidence of either cardiomegaly or systolic dysfunction indicates

A

Restrictive Cardiomyopathy

67
Q

Cardiac Findings in Restrictive Cardiomyopathy

A

S3, Dilated left atrium

68
Q

Treatment for Restrictive Cardiomyopathy

A

CHF therapy, no clear medical therapy –> transplant

69
Q

EF in HCM?

A

increased EF

70
Q

EF in DCM?

A

decreased EF

71
Q

EF in RCM

A

normal-decreased EF

72
Q

Diagnostic test of choice for HCM?

A

ECHO

73
Q

First line therapy for asymptomatic HCM pts?

A

beta-blockers (Negative inotropic effects)