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Med Gen Cardio > Cardiomyopathies > Flashcards

Cardiomyopathies Flashcards

1
Q

What is secondary cardiomyopathy?

A

cardiomyopathy that is caused by another disease such as hypertension or valve disease

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2
Q

What is hypertrophic cardiomyopathy?

A

when the walls of hte heart get thick, heavy, and hypercontractile
Thick because new sarcomeres are added in parallel

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3
Q

What type of heart condition do paeople with Friedreich’s Ataxia often develop?

AR neurodegenerative disease

A

hypertrophic cardiomyopathy

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4
Q

Which part of the heart is affected in hypertrophic cardiomyopathy?

A

left ventricle

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5
Q

Beta blockers, calcium channel blockers, or ? are all possible managements of hypertrophic cardiomyopathy

A

Beta blockers, calcium channel blockers, or cessation of high intensity athletics are all possible managements of hypertrophic cardiomyopathy

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6
Q

Hypertrophic cardiomyopathy is a cardiomyopathy that is often the cause of sudden cardiac death in young athletes due to ?

A

Hypertrophic cardiomyopathy is a cardiomyopathy that is often the cause of sudden cardiac death in young athletes due to ventricular arrhythmia

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7
Q

Hypertrophic cardiomyopathy is classified as a (systolic/diastolic) dysfunction.

A

Hypertrophic cardiomyopathy is classified as a (systolic/diastolic) diastolic dysfunction.

muscle around LV takes up more room, so ventricle is smaller –> less filling –> less pumped out –> decreased stroke volume. Considered a dysfunction of filling so is a diastolic heart failure

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8
Q

Hypertrophic cardiomyopathy can cause ? during exercise and may lead to sudden death in young athletes due to ventricular arrhythmia

A

Hypertrophic cardiomyopathy can cause syncope during exercise and may lead to sudden death in young athletes due to ventricular arrhythmia

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9
Q

What is often a presenting feature for cardiomyopathy?

A

exercise intolerance

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10
Q

Common clinical manifestations of heart failure

A
  • exercise intolerance
  • dyspnea (sensation of running out of air, not beinga ble to breathe fast or deep enough)
  • exertional chest pain
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11
Q

Heart failure is when the threshold needed to gernerate sufficient cardiac output to maintian basal body function is not met. This is often caused by ?

A

cardiomyopathy

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12
Q

Peripheral edema is a sign of (right/left) sided heart failure.

A

Peripheral edema is a sign of right sided heart failure.

heart doesn’t pump out enough blood –> ventricle backs up –> venous system backed up –> capillary pressure increaes –> edema (blood has nowhere to go)

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13
Q

What are some signs of pulmonary edema?

A
  • shortness of breath
  • poor O2 exchange
  • wet lung
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14
Q

Pulmonary edema is a sign of (left/right) sided heart failure.

A

Pulmonary edema is a sign of left sided heart failure.

L ventricular failure –> L atrium pressure –> pulmonary capillaries backed up –> fluid forced in to lungs

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15
Q

What percentage of hypertrophic cardiomyopathy is genetic?

A

100%
sarcomeric proteins

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16
Q

What percentage of dilated cardiomyopathy is genetic?

A

20-50%
primarily cytoskeleton or mitochondria

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17
Q

What are clinical findings you would expect to see in a family suspicious of dilated or hypertrophic cardiomyopathy?

A
  • heart failure
  • sudden death
  • atrial fibrilation
  • stroke
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18
Q

In hypertrophic cardiomyopathy the sarcomeres are not organized leading to ?

A

uncoordinated contraction of the heart

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19
Q

Most genes associated with HCM encode proteins in the ?

A

sarcomere, mainly: actins, myosins (light and heavy chains), troponins, tropomyosins and binding proteins

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20
Q

30-40% of HCM is caused by… (2 genes)

A
  • β-myosin heavy chain (MYH7)
  • cardiac myosin binding protein C (cMYBPC3)
  • cardiac troponins
21
Q

Inheritance pattern for nearly all genetic causes of HCM?

A

AD

22
Q

What is the predominant mechanism of genetic causation of HCM?

A

dominant negative missense variants

23
Q

What is the exception to the predominant dominant negative missense variants for genetic causes of HCM?

A

haploinsufficiency
MYBPC3

24
Q

In HCM, myosin tend to be more (relaxed/bound) compared to a normal heart where they tend to be more (relaxed/bound).

A

In HCM, myosin tend to be more bound compared to a normal heart where they tend to be more relaxed.

25
Q

What is dilated cardiomyopathy?

A
  • all 4 chambers of the heart dilate (get bigger)
  • sarcomeres are added in series and chanbers grow larger
  • leaves the walls thin and with less muscle to use for contractions
  • leads to weak contractions -> less blood pumped out (lower stroke volume) -> biventricular congestive heart failure
  • leads to systolic heart failure b/c of weak contractions
26
Q

Dilated cardiomyopathy leads to (diastolic/systolic) heart failure.

A

Dilated cardiomyopathy leads to systolic heart failure.

This is becuase the issue is with weak contractions due to thin muscle layer of heart. Contractions/ejections = systole

27
Q

If a patient states that they had an LVAD, what type of heart condition would they likely have?

A

dilated cardiomyopathy
LVAD = left ventricular assist device (mechanical pump)

28
Q

? cardiomyopathy is the most common cause of death in Ducheme muscular dystrophy

A

Dilated

29
Q

What is restrictive cardiomyopathy?

A
  • muscle is stiffened and less compliant
  • no expansion/stretching when ventricles are filled with blood (as would normally happen)
  • leads to less filling of ventricles -> heart fails to pump enough blood to body
  • ledas to diastolic heart failure
30
Q

Restrictive cardiomyopathy leads to (diastolic/systolic) heart failure.

A

Restrictive cardiomyopathy leads to diastolic heart failure.

diastolic b/c of the lack of ability to fill ventricles well b/c of stiffness of the heart muscle

31
Q

What are amyloids?

A

misfolded proteins that are insoluble and can be deposited in various tissues and organs making those less compliant

32
Q

What type of heart disease can be caused by amyloidosis?

A

restrictive cardiomyopathy

b/c the amyloid (misfolded protein) deposits make the heart muscle less compliant and unbable to stretch to fill properly leading to insufficent diastole and heart failure

33
Q

Familial Amyloid Cardiomyopathy gene

A

TTR (transthyretin)

type of amyloidosis

34
Q

What type of heart condition are people with hemochromatosis at risk for?

A

restrictive cardiomyopathy
* iron overload condition
* iron can deposit in heart tissue and make the muscle less compliant

35
Q

The heart is unable to (dilate/contract) properly in restrictive cardiomyopathy.

A

The heart is unable to dilate properly in restrictive cardiomyopathy.

36
Q

(Dilated/Restrive) cardiomyopathy is the most common type of cardiomyopathy seen in amyloidosis.

A

Restrictive cardiomyopathy is the most common type of cardiomyopathy seen in amyloidosis.

37
Q

What percentage of dilated cardiomyopathies are genetic?

A

~50%

38
Q

For DCM, in children, (younger/older) age of presentation is associated with a better prognosis.

A

For DCM, in children, younger age of presentation is associated with a better prognosis.

39
Q

What is the inheritance pattern for dilated cardiomyopathy?

A
  • most = AD
  • some = XLr, mito
40
Q

Genetics causes for DCM include a wide variety of gene functions.
T or F?

A

T

41
Q

Mechanism of development of DCM follows a pattern similar to cancer known as ?

A

two hit hypothesis

42
Q

List cardiomyopathies from most to least common.

A
  1. dilated cardiomyopathy
  2. hypertrophic cardiomyopathy
  3. restrictive cardiomyopathy
43
Q

Restrictive cardiomyopathy is associated with infiltrative disorders of the myocardium such as:

A
  • amyloidosis
  • sarcoidosis
  • lysosomal stroage diseases
44
Q

Transthyretin (TTN)-associated cardiomyopathy is associated with what type of cardiomyopathy and why?

A
  • restrictive cardiomyopathy
  • cardiac amyloidosis (deposits of amyloids in myocardial tissue)

monogenic cause of RCM

45
Q

What is the inheritance and penetrance of TTR-associated amyloidosis?

A
  • Autosomal dominant (however, carrier rates are high so homoxygotes and compound heterozygotes are not uncommon)
  • penetrance is believed to be rather low in areas with high incidence of carrier frequency since disease rates aren’t very high
46
Q

Mehcnism of disease for TTR-associated amyloidosis

A
  • Gain of function
  • mutant TTR proteins (amyloids) deposit in ECM of the myocardium and are insoluble leading to RCM
47
Q

TTR-associated amyloidosis can cause lots of other symptoms

A

Often, the cardiac manifestation is part of a more
systemic disease that involve the central nervous system, eyes and kidneys.
Neurologic involvement includes autonomic and peripheral neuropathy, dementia,
psychosis visual impairment, seizure, motor paresis, ataxia, hydrocephalus or
intracranial hemorrhage. Ocular involvement includes vitreous opacity, glaucoma
and angiopathy. Kidney dysfunction is usually mild but can be severe advanced
stages

48
Q

Lysosomal stroage diseases are also a cause of genetic RCM. These conditions include:
They are assocaited with what other cardiomyopathy as well?

A
  • Fabry disease, Pompe disease (MPS2), Hurler disease (MPS1)
  • HCM
49
Q

Danon disease:
* inheritance
* gene
* disease category

A
  • X-linked
  • LAMP2
  • cardiomyopathy

Med Gen Cardio (7 decks)

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