cardiomyopathy and specific heart disease Flashcards

1
Q

what is cardiomyopathy?

A

disease of the heart`

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2
Q

what are the main types of cardiomyopathy?

A

hypertrophic cardiomyopathy
dlated cardiomyopathy
restrictive cardiomyopathy
myocarditis

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3
Q

what are the main pericardial diseases?

A

pericaditis
constrictive pericarditis
pericardial effusion-leads to pericardial tamponade

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4
Q

what is hypertrophic cardiomyopathy?

A

inappropriate ventricular hypertrophy (myocardium hypertrophied without any other cause)

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5
Q

is hypertrophy of ventricles from aortic stenosis or hyertension hypertrophic cardiomyopathy?

A

no

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6
Q

is hypertrophy in hypertrophic cardiomyopathy symmetrical?

A

no (hypertrophy is symmetrical in appropriate hypertropy eg. hypertension)

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7
Q

where does hypertrophy normally occur in HCM?

A

the IV septum
apices
generalised hyertrophy

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8
Q

what is the aetiology of HCM?

A

autosomal dominant inheritance of a single point mutation of sarcomere protein. Phenotypic variation within and between families

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9
Q

what are the three main pathological outcomes if HCM?

A
  • increased muscle mass
  • mitral regurgitation
  • increased risk if pulmonary oedema
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10
Q

what are the problems caused by increased muscle mass in HCM?

A
  • decreased size of ventricle
  • lower LV compliance
  • blockage of ventricular outflow tract
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11
Q

how can HCM cause mitral regurgitation?

A

blockage of LV outflow tract caises Venturi effect

Mitral leaflets are sucked towards the IV septum so they cannot close together

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12
Q

how does HCM increase the risk of pulmonary oedema?

A
  • reduced LV compliance means blood ejected less easily
  • so causing higher pressure in the LV
  • high pressure backs up to the pulmonary circulation
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13
Q

what are the symptoms of HCM?

A
asymptomatic for many
dyspnoea
chest pain
palpitations (cardiac arrhythmias)
syncope
dizziness
sudden death: life-threatening arrythmias
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14
Q

what are the signs of HCM?

A
  • pansystolic murmur
  • notched carotid pulse/steeprising irregular if in AF
  • ejection systolic murmur
  • fourth heart sound and double apical pulsation
  • JVP raised in very restrictive filling
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15
Q

why does HCM cause a pansystolic murmur?

A

mitral regurgitation

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16
Q

why does HCM cause jerky carotid /steep rising pulse?

A

rapid ejection then sudden obstruction of outflow tract

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17
Q

why does HCM cause ejection systolic murmur?

A

LV outflow tract obstruction in systole

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18
Q

why is there a 4th herat sound and double apical pulsation in HCM?

A

forceful atrial contraction

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19
Q

what are the investigations carried out for HCM?

A

ECG
echocardiogram
cardiac MRI
genetic analysis (confirm diagnosis)

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20
Q

what are the general aims if treatment of HCM?

A

relieving symptoms

preventing sudden death

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21
Q

what are the treatments relieving symptoms of HCM

A

Beta blockers

CCBs- verapamil

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22
Q

what are the trearments carried out to prevent sudden death from HCM?

A

alcohol ablation
surgical resection
implantable cardioverter-defibrillation (ICD)

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23
Q

what can put HCM patients at risk if sudden cardiac death?

A
  • family history of sudden cardiac death
  • gene specific
  • syncope
  • ventricular tachycardia
  • exercise hypotension
  • massive left ventricular hypertrophy
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24
Q

what is the definition of dilated cardiomyopathy?

A

heart muscle of LV becomes thin and stretched out so the heart is unable to pump effectively

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25
Q

what is the commonest cardiomyopathy and commonest cause of heart failure?

A

DCM

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26
Q

what is the aetiology of DCM?

A

Genetic and familial DCM: SCN5A gene, muscular dystrophy
Inflammatory, infectious, autoimmune, postpartum
Toxic; drugs, exogenous chemicals, endocrine
Injury, cell loss, scar replacement

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27
Q

what are trhe conditions that DCM commonly presents with?

A
heart failure
cardiac arrhythmias
conduction defects
thromboembolism
sudden death
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28
Q

what are the symptoms of DCM?

A
  • dyspnoea
  • fatigue
  • peripheral oedema
  • orthopnoea
  • paroxysmal nocturnal dsypnoea
  • weight gain
  • cough
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29
Q

what are the signs of DCM?

A

thready pulse
irregular pulse if in AF
narrow pule pressure

poor superficial perfusion
diplaced apex beat
S3 and S4
MR murmur

pulmonary oedema
pleural effusion
SOB at rest

ankle oedema
sacral oedema
ascites
hepatomegaly
JVP elevated
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30
Q

what are the investigations that must be carried out for DCM?

A
  • ECG- noting LBBB
  • echocardiogram
  • cardiac MRI
  • coronary angiogram
  • sometimes biopsy
  • CXR
  • basic bloods FBC and U & E
  • N terminal pro Brain Natiuretic peptide
31
Q

what will show up on an echocardiogram in DCM?

A

dilatation of the left and/or right ventricle

32
Q

what will show up on an CXR in DCM?

A

generalised cardiac enlargement

33
Q

what is a cardiac MRI useful for in DCM?

A

identifying other aetiologies of LV systolic dysfunction (e. MI) or abdominal myocardial fibrosis

34
Q

why is a coronary amgiogram carried out for DCM?

A

exclude coronary artery disease

35
Q

what are the treatments of DCM?

A
treat heart failure with;
spiranolactone
oral nitrates
ACE-i
Beta Blockers
Digoxin
warfarin

corrct anaemia
correct endocrine disturbance
reuce fluid and salt intake
cardiac transplant

36
Q

what is alcohol heart disease?

A

excessive alcohol consumption can lead to thiamine deficiency, direct toxicity and the adulaterations of alcohol causes effects
it presents similarly to dilated cardiomyopathy

37
Q

what are the general meausures to control symptoms of DCM?

A

avoid heavy exercise

avoid dehydration

38
Q

what are the infiltrative causes of restrictive and infiltrative cardiomyopathy?

A

amyloid sarcoid

39
Q

what are the non-infiltrative causes of restrictive and infiltrative cardiomyopathy?

A
familial
forms of HCM
sclerodoma
diabetes
pseudoxanthoma elasticum
40
Q

what are the storage diseases that can cuase restrcictive and infiltrative cardiomyopathy?

A

haemachromatosis

Fabry disease

41
Q

what are the endo myocrdial caused of restrictive and infiltrative cardiomyopathy?

A

fibrosis
carcinoid
radiation
drug effects

42
Q

what is the pathology of restrictive and infiltrative cardiomyopathy?

A

inability to fill the ventricles as they have reduced compliance. This leads to atrial dilatation as they gave to work harder and atrial fibrillation.

43
Q

what are the investigations carried out for restrictive and infiltrative cardiomyopathy?

A
  • repeated ECG (noting LBBB and other conduction defects)
  • ECHO
  • cardiac MRI
  • biopsy sometimes useful for amyloid and sarcoid
  • CXR
  • N termial pro Brain Natriuretic Peptide
  • Basic bloods, U and E and look out for sarcoid and haemochromatosis
  • autoantibodies for sclerotic CT diseases
  • low plasma alpha galactoside A activity for Fabry
44
Q

what are the treatments for restrictive and infiltrative crdiomyopathy?

A
  • anticoagulants if AF
  • limited diuretic as low filling pressures cause problems
  • Beta blockers
  • limited ACEi
  • ICD or CRT-D/P implant rare as prognosis is so low
  • cardiac transplant
45
Q

what is myocarditis?

A

acute or chronic inflammation of the myocardium

46
Q

what other heart conditions can myocarditis cause?

A

myocardial function impairment
conduction impairment- heart block
generate arrhthmia

47
Q

what are the causes of myocarditis?

A
viral - common
bacterial fungal and parasitic - uncommon
toxins eg. cocoaine
hypersensitivity
autoimmune activation
48
Q

what are the symptoms of myocarditis?

A
assymptomatic
heart failure
fatigue
SOB
chets pain in a 1/4 patients
fever
49
Q

what are the signs of myocarditis?

A

tachycardia
soft heart sounds
prominent third heart sound
pericardial rub

50
Q

what can chronic myocarditis lead to?

A

enlarged and hypertrophied heart and dilated cardiomyopathy

51
Q

what arethe investigations crried out for myocarditis?

A

ECG
ECHO
cardiac MRI
biopsy

troponin raised but unlike in MI it doesnt fall
viral dna pcr
autoantibodies
strep antibodies
lyme B burgdorferi
HIV
52
Q

what are the general meaures for the treatment of mocarditis?

A
  • treatment of heart failure
  • treatment of brady/tachy arrhythmias
  • immunotherapy
  • stop possible drugs bringing about inflammation
53
Q

what is pericarditis?

A

inflammation of the pericardial layers (with or without the myocardium)

54
Q

what are the most common causes of pericarditis?

A
viral
bacterial
post MI
perforation
dissection of proximal aorta
neoplasia
autoimmune response
55
Q

what are the symptoms of pericarditis?

A

chest pain:
relieved by sitting forward
execerbated by movement, repsiration and lying down
fever

56
Q

what are the signs of pericarditis?

A
pericardial rub
fever
raised JVP (if effusion causinf haemodynamic effects)
low BP
muffled heart sounds
57
Q

what are the investigations carried out for pericarditis?

A

ECG- ST segment raised and concave and PR dependent
echo
troponin (raised if myocardium involved)
CXR

58
Q

what is the treatment of viral pericarditis?

A

conservative

59
Q

what is the treatment of idiopathic pericarditis?

A

colchicine

limited use of NSAIDS

60
Q

what is the treatment of bacterial pericarditis?

A

drain pericardial effusion

antimicrobials after

61
Q

what is tamponade?

A

haemodynamically significanr pericardial effusion

62
Q

what are the causes of pericardial effucion?

A

often same as pericrditis

63
Q

what are the symptoms of pericardial effusion?

A

fatigue
SOB
dizzy with low BP
occasionally chest pain

64
Q

what are the signs of pericardil effusion?

A
pulsus paradoxus
JVP raised
low BP
rub
muffled HS
65
Q

what is pulsus paradoxus?

A

an abnormally large decrease in stroke volume, systolic blood pressure and pulse wave amplitude during inspiration

66
Q

what are the tests carried out for pericardial effusion?

A

echo

CXR

67
Q

what is the treatment of pericardial effusion?

A

drainage

underlying cause

68
Q

what are the causes of constrictive pericarditis?

A
idopathic
radiation
post surgery
autoimmune
renal failure
sarcoid.
69
Q

what is the pathology of constrictive pericarditis?

A

impaired filling although myocardium is normal

70
Q

what are the symptoms of contrictive pericarditis

A

fatigue
SOB
cough

71
Q

what are the signs of constrictive pericarditis?

A
RHF with oedema
ascites
high JVP
jaundice
hepatomegaly
AF
TR
pleural effusion
pericardial knock
72
Q

what are the investigations for constrictive pericarditis?

A

echo
right heart catheter to differentiate from RCM
ECG
CXR

73
Q

what is the treatment of constrictive pericarditis?

A

limited diuretic

pericardectomy