Cardiomyopathies Flashcards

1
Q

Endocardium layer is made up of both these tissue types?

A

Endothelium and Connective Tissue

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2
Q

The endocardium is contiguous with

A

vasculature

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3
Q

Myocardium is made up of what kind of cells

A

striated cardiac muscle cells

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4
Q

Myocardium is excited by electrical depolarization and calcium causes what to bind?

A

Actin and myosin

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5
Q

Coronaries are tucked under what heart layer

A

Epicardium

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6
Q

Positive chronotropes help with ________ heart rate and negative chronotropes _________ heart rate

A

Increase, decrease

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7
Q

Hypothyroidism has what effect on heart rate

A

Increased metabolism —> Increased body temp —-> Increased HR

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8
Q

Hypoxemia and acidosis has what effect on heart rate

A

Increased sympathetic tone –> increased HR

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9
Q

What are the effects that will decrease HR?

A

Parasympathetic activity
Hormones
Medications
Exercise (athletes)

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10
Q

What is the equation for ejection fraction

A

Stroke Volume/ End-Diastolic Volume

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11
Q

How much blood volume in the left ventricle should be pumped out during each heartbeat?

A

Half the volume

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12
Q

What is the concept of the Frank-Starling Curve?

A

Plotting stroke volume vs. ventricular end-diastolic volume to estimate optimal sarcomere length and optimal cardiac output

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13
Q

What is the disease process of cardiomyopathy?

A

Change in the cardiac structure and affects the myocardium itself

m/c referencing disease process of the LV

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14
Q

What are the different types of cardiomyopathy?

A

Hypertrophic
Dilated (most common)
Arrhythmogenic
Restrictive (least common)
Unclassified

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15
Q

What is the Classification MOGE(s) system?

A

Classification system gives information about the disease but also may be able to help risk stratify patients for potential future cardiovascular events

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16
Q

What does MOGE(s) stand for?

A

M= morphofunctional
O= Organ involvement
G= genetic predisposition
E= etiological definition
S= functional status

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17
Q

Where is the problem with systolic dysfunction?

A

Decreased contractility leading to reduced ejection fraction, progression will eventually exceed Frank-Sterling Curve

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18
Q

What is usually the cause for systolic dysfunction?

A

Dilated Cardiomyopathy

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19
Q

What are the usual causes for diastolic dysfunction?

A

Hypertrophic Cardiomyopathy
Restrictive Cardiomyopathy

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20
Q

In dilated cardiomyopathy, the ventricle will be dilated which will lead to?

A

Reduced contractility- decreased cardiac output

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21
Q

Dilation of the LV will lead to:

A

Increased preload (EDV)
Increased atrial pressure
Atrial dilation

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22
Q

What is the most common cause of right sided heart failure is what?

A

Left sided heart failure

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23
Q

In the setting of decreased CO and decreasing BP, the body is going to compensate by activating what system?

A

Renin-angiotensin aldosterone system (activated by the decreased cardiac output)

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24
Q

Dilated cardiomyopathy is most commonly associated with what etiology?

A

Genetic (TTN)

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25
Q

What is adrenal pheochromocytoma?

A

Tumor arising from catecholamine producing chromaffin cells in the adrenal gland medulla

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26
Q

Who classically presents with Takotsubo (“Broken Heart”)?

A

Postmenopausaul women

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27
Q

What is thought to be the cause of Takotsubo?

A

Associated with a huge catecholamine surge, microvascular dysfunction and spasm of the coronaries

28
Q

Chronic ETOH use can cause what kind of cardiomyopathy?

A

Dilated cardiomyopathy

29
Q

Hypertrophic cardiomyopathy is most commonly caused by what type of genetic inheritance?

A

Autosomal Dominant

30
Q

A mutation in what protein can cause hypertrophic cardiomyopathy?

A

Missense mutation in myosin, leads to increased systolic function initially but diminished relaxation overtime

31
Q

Why does Hypertrophic Obstructive Cardiomegaly (HOCM) lead to decreased cardiac output?

A

LV hypertrophy along the septum can begin to occlude the outflow tract (aortic valve), increased work for the LV, pushing against narrowed aortic outflow tract

As this narrows, vacuum can form d/t blood flow turbulence which will lead to decreased CO –> hypotension and syncope (DEATH)

32
Q

What heart sound is associated with hypertrophic cardiomyopathy?

A

S4, due to atrial hypertrophy and atrial kick into the limited LV

33
Q

What heart sound is associated with dilated cardiomyopathy?

A

S3

34
Q

What myopathy results in fibrosis of the mycardium?

A

Restrictive cardiomyopathy

35
Q

What is the pathophysiology of restrictive cardiomyopathy?

A

Fibrotic changes within the myocardium which causes decreased compliance of the ventricles and decreased ventricular filling capacity

Decreased preload, CO, and supply/demand mismatch

36
Q

What are the causes of restrictive cardiomyopathy?

A

Endomyocardial fibrosis
Radiation fibrosis
Amyloidosis
Sarcoidosis
Hemochromatosis

37
Q

What leads to myocardial fibrosis in amyloidosis?

A

Misfolded antibodies (genetic cause) that deposit within the tissues causing chronic inflammation that is then deposited into the myocardium

38
Q

Hemochromatosis can lead to what type of cardiomyopathy due to iron deposits within the myocardium?

A

Restrictive Cardiomyopathy

39
Q

What type of cardiomyopathy can cause sudden cardiac death?

A

HOCM

40
Q

What is the most common type of cardiomyopathy?

A

Dilated cardiomyopathy

41
Q

What is the epidemiology of dilated cardiomyopathy?

A
  • up to 60% genetic
  • M > F (3:1)
  • Black patients at higher risk
42
Q

Per Collins, what are the top causes of dilated cardiomyopathy?

A

Infection (enterovirus and adenovirus)
Toxic and overload (alcohol, cirrhosis)
Endocrine (DM)

43
Q

How is dilated cardiomyopathy characterized?

A

Ventricular enlargement (LV>RV>atria)
No associated hypertrophy
Impaired systolic function

Big and Floppy One

44
Q

What is the presentation of dilated cardiomyopathy?

A

‘Asymptomatic’ until CO falls

Signs and symptoms consistent with HF (decreased perfusion, fluid overload, compensatory to increase pressure/oxygenation)

45
Q

What are the signs of left-sided dilated cardiomyopathy?

A

Dyspnea on exertion
Orthopnea
Paroxysmal nocturnal dyspnea
Cough
Fatigue
Altered mental status
S3 or S4 heart sounds
Valvular regurgitation

46
Q

What are the symptoms of right-sided dilated cardiomyopathy?

A

Peripheral edema
RUQ pain
Anorexia or reduced appetite
Bloating/weight gain
Fatigue

47
Q

What are the signs of right-sided dilated cardiomyopathy?

A

Peripheral edema
Elevated JVP
Kussmauls sign
Hepatomegaly
Anasarca
Ascites
Weight gain

48
Q

What is the best test in working up dilated cardiomyopathy?

A

Echocardiogram

49
Q

What findings on CXR are going to be indicative of dilated cardiomyopathy (HF)?

A

Hilar Fullness
Cephalization
Kerley B lines
Pleural effusions

50
Q

What are Kerley B lines

A

Edema of the interlobular septa

51
Q

What is cephalization

A

Increased prominence of upper lobe vasculature

52
Q

What is the treatment for dilated cardiomyopathy?

A

Treat like heart failure

All should get beta blocker and ACEi (ARBs second line)

53
Q

What is the treatment for an acute exacerbation of cardiomyopathy/HF?

A

Diuretics
Furosemide (Lasix)
Bumetanide (Bumex)
Torsemide (PO)

54
Q

What is the number one cause of sudden cardiac death in young children/young adults?

A

Hypertrophic Obstructive Cardiomyopathy (HOCM)

55
Q

How is HOCM characterized?

A

Myocyte disarray
Left ventricular hypertrophy
Impaired diastolic function
LV outflow obstruction

56
Q

What is the presentation of HOCM?

A

Dyspnea most common
Angina (without CAD)
Syncope
Orthostatic hypertension
Arrhythmias (V.Fib)

57
Q

Will the physical exam be normal in HOCM?

A

Yes, early on

58
Q

What associated physical exam abnormalities maybe seen with HOCM?

A

S4 heart sound (atrial kick)
Exaggerated PMI
Systolic crescendo-decrescendo murmur left sternal border
Mitral regurgitation murmur
Bifid carotid pulse

59
Q

What findings on EKG will be seen in HOCM?

A

LVH, Left atrial enlargement, left axis deviation
Q-waves
ST depression or inversion
A Fib

60
Q

What are three important tests to order in working-up HOCM?

A

Cardiac MRI
Stress ECHO
Genetic testing

61
Q

What is the preferred treatment in HOCM?

A

Calcium Channel Blockers (Verapamil, Diltiazem)

62
Q

What medications should be avoided in HOCM?

A

Diuretics - worsens LVOO
Nitrates - decreased preload

63
Q

What cardiomyopathy has the worst prognosis?

A

Restrictive cardiomyopathy

64
Q

What is Collin’s term for restrictive cardiomyopathy?

A

Big and stiff

65
Q

What are the causes of restrictive cardiomyopathy?

A

Amyloidosis
Scleroderma
Idiopathic
Infiltrative causes (sarcoidosis, hemochromatosis)
Tumors
Radiation

66
Q

What is the best diagnostic test for restrictive cardiomyopathy?

A

Biopsy (congo red stain, looking for amyloidosis deposits or fibrosis)

67
Q

What is the treatment for restrictive cardiomyopathy?

A

Treat underlying cause (amyloidosis) and reduce salt

Diuretics in acute HF