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
What is adrenal pheochromocytoma?
Tumor arising from catecholamine producing chromaffin cells in the adrenal gland medulla
26
Who classically presents with Takotsubo ("Broken Heart")?
Postmenopausaul women
27
What is thought to be the cause of Takotsubo?
Associated with a huge catecholamine surge, microvascular dysfunction and spasm of the coronaries
28
Chronic ETOH use can cause what kind of cardiomyopathy?
Dilated cardiomyopathy
29
Hypertrophic cardiomyopathy is most commonly caused by what type of genetic inheritance?
Autosomal Dominant
30
A mutation in what protein can cause hypertrophic cardiomyopathy?
Missense mutation in myosin, leads to increased systolic function initially but diminished relaxation overtime
31
Why does Hypertrophic Obstructive Cardiomegaly (HOCM) lead to decreased cardiac output?
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
What heart sound is associated with hypertrophic cardiomyopathy?
S4, due to atrial hypertrophy and atrial kick into the limited LV
33
What heart sound is associated with dilated cardiomyopathy?
S3
34
What myopathy results in fibrosis of the mycardium?
Restrictive cardiomyopathy
35
What is the pathophysiology of restrictive cardiomyopathy?
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
What are the causes of restrictive cardiomyopathy?
Endomyocardial fibrosis Radiation fibrosis Amyloidosis Sarcoidosis Hemochromatosis
37
What leads to myocardial fibrosis in amyloidosis?
Misfolded antibodies (genetic cause) that deposit within the tissues causing chronic inflammation that is then deposited into the myocardium
38
Hemochromatosis can lead to what type of cardiomyopathy due to iron deposits within the myocardium?
Restrictive Cardiomyopathy
39
What type of cardiomyopathy can cause sudden cardiac death?
HOCM
40
What is the most common type of cardiomyopathy?
Dilated cardiomyopathy
41
What is the epidemiology of dilated cardiomyopathy?
- up to 60% genetic - M > F (3:1) - Black patients at higher risk
42
Per Collins, what are the top causes of dilated cardiomyopathy?
Infection (enterovirus and adenovirus) Toxic and overload (alcohol, cirrhosis) Endocrine (DM)
43
How is dilated cardiomyopathy characterized?
Ventricular enlargement (LV>RV>atria) No associated hypertrophy Impaired systolic function Big and Floppy One
44
What is the presentation of dilated cardiomyopathy?
'Asymptomatic' until CO falls Signs and symptoms consistent with HF (decreased perfusion, fluid overload, compensatory to increase pressure/oxygenation)
45
What are the signs of left-sided dilated cardiomyopathy?
Dyspnea on exertion Orthopnea Paroxysmal nocturnal dyspnea Cough Fatigue Altered mental status S3 or S4 heart sounds Valvular regurgitation
46
What are the symptoms of right-sided dilated cardiomyopathy?
Peripheral edema RUQ pain Anorexia or reduced appetite Bloating/weight gain Fatigue
47
What are the signs of right-sided dilated cardiomyopathy?
Peripheral edema Elevated JVP Kussmauls sign Hepatomegaly Anasarca Ascites Weight gain
48
What is the best test in working up dilated cardiomyopathy?
Echocardiogram
49
What findings on CXR are going to be indicative of dilated cardiomyopathy (HF)?
Hilar Fullness Cephalization Kerley B lines Pleural effusions
50
What are Kerley B lines
Edema of the interlobular septa
51
What is cephalization
Increased prominence of upper lobe vasculature
52
What is the treatment for dilated cardiomyopathy?
Treat like heart failure All should get beta blocker and ACEi (ARBs second line)
53
What is the treatment for an acute exacerbation of cardiomyopathy/HF?
Diuretics Furosemide (Lasix) Bumetanide (Bumex) Torsemide (PO)
54
What is the number one cause of sudden cardiac death in young children/young adults?
Hypertrophic Obstructive Cardiomyopathy (HOCM)
55
How is HOCM characterized?
Myocyte disarray Left ventricular hypertrophy Impaired diastolic function LV outflow obstruction
56
What is the presentation of HOCM?
Dyspnea most common Angina (without CAD) Syncope Orthostatic hypertension Arrhythmias (V.Fib)
57
Will the physical exam be normal in HOCM?
Yes, early on
58
What associated physical exam abnormalities maybe seen with HOCM?
S4 heart sound (atrial kick) Exaggerated PMI Systolic crescendo-decrescendo murmur left sternal border Mitral regurgitation murmur Bifid carotid pulse
59
What findings on EKG will be seen in HOCM?
LVH, Left atrial enlargement, left axis deviation Q-waves ST depression or inversion A Fib
60
What are three important tests to order in working-up HOCM?
Cardiac MRI Stress ECHO Genetic testing
61
What is the preferred treatment in HOCM?
Calcium Channel Blockers (Verapamil, Diltiazem)
62
What medications should be avoided in HOCM?
Diuretics - worsens LVOO Nitrates - decreased preload
63
What cardiomyopathy has the worst prognosis?
Restrictive cardiomyopathy
64
What is Collin's term for restrictive cardiomyopathy?
Big and stiff
65
What are the causes of restrictive cardiomyopathy?
Amyloidosis Scleroderma Idiopathic Infiltrative causes (sarcoidosis, hemochromatosis) Tumors Radiation
66
What is the best diagnostic test for restrictive cardiomyopathy?
Biopsy (congo red stain, looking for amyloidosis deposits or fibrosis)
67
What is the treatment for restrictive cardiomyopathy?
Treat underlying cause (amyloidosis) and reduce salt Diuretics in acute HF