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cardiovascular > cardiomyopathy > Flashcards

cardiomyopathy Flashcards

1
Q

types

A
  • hypertrophic
  • arrhythmogenic
  • dilated
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2
Q

what do they affect of the heart

A

mechanical or electrical function of the heart

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3
Q

what are the secondary causes

A
  • infiltrative
  • storage related (haemochromatosis)
  • drugs (alcohol)
  • neuromuscular
  • nutritional
  • autoimmune (systemic lupus)
  • cancer therapy
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4
Q

what are some causes of hypertrophic cardiomyopathy

A
  • aortic stenosis

- hypertension

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5
Q

what is the most common cause of sudden death in young people

A

hypertrophic cardiomyopathy

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6
Q

what kind of inheritance is hypertrophic cardiomyopathy

A

familial autosomal dominant

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7
Q

what are some mutations that cause hypertrophic cardiomyopathy

A
  • genes encoding sarcomeric proteins
  • ß-myosin heavy chain MYH7
  • myosin bingin protein C
  • troponin T and I
  • alpha myosin heavy chain
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8
Q

where does hypertrophic cardiomyopathy involve

A

interventricular septum

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9
Q

what can ventricular ejection cause

A

mitral regurgitation

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10
Q

what are some characteristics of a mutation in troponin T

A
  • mild hypertrophy

- high risk of sudden death

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11
Q

when does hypertrophy due to myosin binding protein manifest

A

not until sixth decade or later

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12
Q

symptoms of hypertrophic cardiomyopathy

A
  • asymptotic
  • chest pain
  • SOB
  • syncope
  • arrhythmias
  • sudden death
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13
Q

signs of hypertrophic cardiomyopathy

A
  • double apical pulsation
  • jerky carotid pulse
  • ejection systolic murmur
  • pan systolic murmur
  • fourth heart sound
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14
Q

why is there a jerky carotid pulse in hypertrophic cardiomyopathy

A

rapid ejection and sudden obstruction to left ventricular outflow

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15
Q

why is there ejection systolic murmur in hypertrophic cardiomyopathy

A

due to left ventricular outflow obstruction late in systole

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16
Q

why is there pan systolic murmur in hypertrophic cardiomyopathy

A

due to mitral regurgitation

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17
Q

what investigations are done for hypertrophic cardiomyopathy

A
  • ECG
  • echo
  • CMR
  • genetic analysis
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18
Q

what is seen in ECG of hypertrophic cardiomyopathy

A
  • left ventricular hypertrophy
  • ST and T wave changes
  • abnormal Q waves
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19
Q

what is seen in echo of hypertrophic cardiomyopathy

A
  • asymmetric left ventricular hypertrophy
  • vigorously contracting ventricle
  • patterns of hypertrophy
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20
Q

what is diagnostic for hypertrophic cardiomyopathy

A

echo

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21
Q

what can CMR detect in hypertrophic cardiomyopathy

A
  • hypertrophy

- abnormal myocardial fibrosis

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22
Q

treatment of hypertrophic cardiomyopathy

A

treatment of symptoms and prevent sudden death

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23
Q

what are the risk factors of sudden death in hypertrophic cardiomyopathy

A
  • massive left ventricular hypertrophy
  • family history of sudden death
  • non-sustained ventricular tachycardia
  • prior unexplained syncope
  • abnormal blood pressure on exercise
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24
Q

what is given if low risk of sudden death in hypertrophic cardiomyopathy

A

amiodarone

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25
Q

what is given if high risk of sudden death in hypertrophic cardiomyopathy

A

ICD insertion

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26
Q

what is given for chest pain and dyspnoea in hypertrophic cardiomyopathy

A

beta blockers and verapamil

alone or in combo

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27
Q

what is given for left ventricular outflow tract obstruction in hypertrophic cardiomyopathy

A

disopyramide

28
Q

what drugs should be avoided in hypertrophic cardiomyopathy

A

vasodilators as they may aggravate left ventricular outflow or cause refractory hypotension

29
Q

where does the arrhythmogenic cardiomyopathy affect

A

right ventricle

30
Q

what are the myocytes replaced with in arrhythmogenic cardiomyopathy

A

fatty or fibro-fatty replacements

31
Q

what does fibrosis-fatty replacement lead to in arrhythmogenic cardiomyopathy

A

ventricular arrhythmia

32
Q

what mutations are there in arrhythmogenic cardiomyopathy

A

in genes coding for desmosomal proteins

33
Q

what is Naxos disease associated with

A

palmoplantar keratoderma and wooly hair

34
Q

what is Naxos disease due to

A

mutation in junctional plakoglobin

35
Q

what is Carvajal syndrome due to

A

mutation in desmoplakin

36
Q

what are the 2 recessive forms of arrhythmogenic cardiomyopathy

A
  • Naxos disease

- Carvajal syndrome

37
Q

symptoms of arrhythmogenic cardiomyopathy

A
  • asymptomatic
  • syncope
  • sudden death
  • signs of right heart failure
38
Q

what investigations are done for arrhythmogenic cardiomyopathy

A
  • ECG
  • echo
  • CMR
  • genetic test
39
Q

what is seen on ECG of arrhythmogenic cardiomyopathy

A
  • T wave inversion in precordial leads related to right ventricle
  • right uncle branch block
  • delayed depolarisations
40
Q

what is seen on echo of arrhythmogenic cardiomyopathy

A

right ventricular dilatation and aneurysm formation

left ventricular dilatation

41
Q

what is seen on CMR of arrhythmogenic cardiomyopathy

A

can assess right ventricle more accurately

can demonstrate fibro-fattty infiltration

42
Q

what can be the gold standard for arrhythmogenic cardiomyopathy

A

genetic testing

43
Q

what is first line treatment for arrhythmogenic cardiomyopathy

A

beta blockers

44
Q

what is treatment for symptomatic arrhythmias 1

A

amiodarone or sotalol

45
Q

what is done for life-threatening arrhythmogenic cardiomyopathy

A

ICD

46
Q

what is dilated cardiomyopathy characterised by

A

dilatation of ventricular chambers
and
systolic dysfunction with preserved wall thickness

47
Q

what kind of inheritance is dilated cardiomyopathy

A

autosomal dominant

48
Q

what are causes of sporadic dilated cardiomyopathy

A
  • myocarditis
  • toxins
  • autoimmune
  • endocrine
  • neuromuscular
49
Q

what does dilated cardiomyopathy present with

A
  • heart failure
  • cardiac arrrhythmias
  • condition defects
  • thromboembolism
  • sudden death
50
Q

what investigation are done for dilated cardiomyopathy

A
  • CXR
  • ECG
  • echo
  • CMR
  • coronary angiography
  • biopsy
51
Q

what is seen on CXR for dilated cardiomyopathy

A

cardiac enlargement

52
Q

what is seen on ECG for dilated cardiomyopathy

A
  • non specific ST and T wave changes
  • sinus tachycardia
  • arrhythmias
53
Q

what is seen on echo of dilated cardiomyopathy

A
  • dilatation of left and/or right ventricle with poor contraction function
54
Q

what is seen on CMR of dilated cardiomyopathy

A

can show other causes of left ventricular dysfunction
or
abnormal myocardial fibrosis

55
Q

what are treatment options of dilated cardiomyopathy

A
  • cardiac resynchronisation
  • ICDs
  • cardiac transplantation
56
Q

what is the appearance of left ventricular non-compaction

A

sponge like appearance of left ventricle

57
Q

where does left ventricular non-compaction affect

A

apical portion of the left ventricle

58
Q

how is left ventricular non-compaction diagnosed

A
  • echo
  • CMR
  • left ventricular angiography
59
Q

what is Primary restrictive non-hypertrophic cardiomyopathy

A

rare condition in which there is normal or decreased volume of both ventricles with bi-atrial enlargement, normal wall thickness, normal cardiac valves and impaired ventricular function

60
Q

what condition are associated with primary restrictive non-hypertrophic cardiomyopathy

A
  • amyloidosis
  • sarcoidosis
  • Loefflers endocarditis
  • end-myocardial fibrosis
61
Q

what investigations are done for primary restrictive non-hypertrophic cardiomyopathy

A
  • CXR
  • ECG
  • echo
  • CMR
  • cardiac catheterisation
  • biopsy
62
Q

what is seen on echo of Primary restrictive non-hypertrophic cardiomyopathy

A
  • symmetrical myocardial thickening
  • notmal systolic ejection fraction
  • imparted ventricular filling
  • myocardium is speckled
  • tramlines on M mode echo
63
Q

treatment for Primary restrictive non-hypertrophic cardiomyopathy

A

no specific treatment

64
Q

what treatment is sometimes done for Primary restrictive non-hypertrophic cardiomyopathy

A

cardiac transplantaiton

65
Q

what is effective for familial amyloidosis

A

liver transplantation

cardiovascular (36 decks)

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