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
what is the commonest cardiomyopathy and commonest cause of heart failure?
DCM
26
what is the aetiology of DCM?
Genetic and familial DCM: SCN5A gene, muscular dystrophy Inflammatory, infectious, autoimmune, postpartum Toxic; drugs, exogenous chemicals, endocrine Injury, cell loss, scar replacement
27
what are trhe conditions that DCM commonly presents with?
``` heart failure cardiac arrhythmias conduction defects thromboembolism sudden death ```
28
what are the symptoms of DCM?
- dyspnoea - fatigue - peripheral oedema - orthopnoea - paroxysmal nocturnal dsypnoea - weight gain - cough
29
what are the signs of DCM?
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 ```
30
what are the investigations that must be carried out for DCM?
- ECG- noting LBBB - echocardiogram - cardiac MRI - coronary angiogram - sometimes biopsy - CXR - basic bloods FBC and U & E - N terminal pro Brain Natiuretic peptide
31
what will show up on an echocardiogram in DCM?
dilatation of the left and/or right ventricle
32
what will show up on an CXR in DCM?
generalised cardiac enlargement
33
what is a cardiac MRI useful for in DCM?
identifying other aetiologies of LV systolic dysfunction (e. MI) or abdominal myocardial fibrosis
34
why is a coronary amgiogram carried out for DCM?
exclude coronary artery disease
35
what are the treatments of DCM?
``` 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
what is alcohol heart disease?
excessive alcohol consumption can lead to thiamine deficiency, direct toxicity and the adulaterations of alcohol causes effects it presents similarly to dilated cardiomyopathy
37
what are the general meausures to control symptoms of DCM?
avoid heavy exercise | avoid dehydration
38
what are the infiltrative causes of restrictive and infiltrative cardiomyopathy?
amyloid sarcoid
39
what are the non-infiltrative causes of restrictive and infiltrative cardiomyopathy?
``` familial forms of HCM sclerodoma diabetes pseudoxanthoma elasticum ```
40
what are the storage diseases that can cuase restrcictive and infiltrative cardiomyopathy?
haemachromatosis | Fabry disease
41
what are the endo myocrdial caused of restrictive and infiltrative cardiomyopathy?
fibrosis carcinoid radiation drug effects
42
what is the pathology of restrictive and infiltrative cardiomyopathy?
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
what are the investigations carried out for restrictive and infiltrative cardiomyopathy?
- 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
what are the treatments for restrictive and infiltrative crdiomyopathy?
- 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
what is myocarditis?
acute or chronic inflammation of the myocardium
46
what other heart conditions can myocarditis cause?
myocardial function impairment conduction impairment- heart block generate arrhthmia
47
what are the causes of myocarditis?
``` viral - common bacterial fungal and parasitic - uncommon toxins eg. cocoaine hypersensitivity autoimmune activation ```
48
what are the symptoms of myocarditis?
``` assymptomatic heart failure fatigue SOB chets pain in a 1/4 patients fever ```
49
what are the signs of myocarditis?
tachycardia soft heart sounds prominent third heart sound pericardial rub
50
what can chronic myocarditis lead to?
enlarged and hypertrophied heart and dilated cardiomyopathy
51
what arethe investigations crried out for myocarditis?
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
what are the general meaures for the treatment of mocarditis?
- treatment of heart failure - treatment of brady/tachy arrhythmias - immunotherapy - stop possible drugs bringing about inflammation
53
what is pericarditis?
inflammation of the pericardial layers (with or without the myocardium)
54
what are the most common causes of pericarditis?
``` viral bacterial post MI perforation dissection of proximal aorta neoplasia autoimmune response ```
55
what are the symptoms of pericarditis?
chest pain: relieved by sitting forward execerbated by movement, repsiration and lying down fever
56
what are the signs of pericarditis?
``` pericardial rub fever raised JVP (if effusion causinf haemodynamic effects) low BP muffled heart sounds ```
57
what are the investigations carried out for pericarditis?
ECG- ST segment raised and concave and PR dependent echo troponin (raised if myocardium involved) CXR
58
what is the treatment of viral pericarditis?
conservative
59
what is the treatment of idiopathic pericarditis?
colchicine | limited use of NSAIDS
60
what is the treatment of bacterial pericarditis?
drain pericardial effusion | antimicrobials after
61
what is tamponade?
haemodynamically significanr pericardial effusion
62
what are the causes of pericardial effucion?
often same as pericrditis
63
what are the symptoms of pericardial effusion?
fatigue SOB dizzy with low BP occasionally chest pain
64
what are the signs of pericardil effusion?
``` pulsus paradoxus JVP raised low BP rub muffled HS ```
65
what is pulsus paradoxus?
an abnormally large decrease in stroke volume, systolic blood pressure and pulse wave amplitude during inspiration
66
what are the tests carried out for pericardial effusion?
echo | CXR
67
what is the treatment of pericardial effusion?
drainage | underlying cause
68
what are the causes of constrictive pericarditis?
``` idopathic radiation post surgery autoimmune renal failure sarcoid. ```
69
what is the pathology of constrictive pericarditis?
impaired filling although myocardium is normal
70
what are the symptoms of contrictive pericarditis
fatigue SOB cough
71
what are the signs of constrictive pericarditis?
``` RHF with oedema ascites high JVP jaundice hepatomegaly AF TR pleural effusion pericardial knock ```
72
what are the investigations for constrictive pericarditis?
echo right heart catheter to differentiate from RCM ECG CXR
73
what is the treatment of constrictive pericarditis?
limited diuretic | pericardectomy