Cardiomyopathy Flashcards

1
Q

What is cardiomyopathy?

A

Primary disease of the myocardium.

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2
Q

What are the 4 different types of cardiomyopathy?

A

Dilated
Hypertrophic
Restrictive
Arrhythmogenic Right Ventricular (ARVC)

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3
Q

Describe the aetiology of cardiomyopathy

A

Majority are IDIOPATHIC

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4
Q

List 4 features of dilated cardiomyopathy

A

Sx of HF: fatigue, dyspnoea
Arrhythmias
Thromboembolism
FH of sudden death

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5
Q

List 10 causes of dilated cardiomyopathy

A
Post-viral myocarditis  
Alcohol 
Drugs (e.g. doxorubicin, cocaine) 
Familial 
Thyrotoxicosis  
Haemochromatosis 
Peripartum or postpartum  
HTN  
AI
Congenital (x linked)
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6
Q

List 6 causes of restrictive cardiomyopathy

A
Amyloidosis  
Sarcoidosis  
Haemochromatosis  
Scleroderma  
Loffler’s eosinophilic endocarditis 
Endomyocardial fibrosis
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7
Q

List 5 features of restrictive cardiomyopathy

A
Dyspnoea 
Fatigue  
Arrhythmias  
Ankle or abdominal swelling  
FH of sudden death
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8
Q

What are the signs and symptoms of restrictive cardiomyopathy similar to?

A

Constrictive pericarditis

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9
Q

List 12 signs of dilated cardiomyopathy

A
Raised JVP  
Displaced apex beat  
Functional mitral + tricuspid regurgitations  
3rd heart sound  
Tachycardia 
AF
Hypotension  
Pleural effusion 
Oedema 
Jaundice 
Hepatomegaly 
Ascites
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10
Q

List 7 signs of restrictive cardiomyopathy

A
Raised JVP  
Kussmaul Sign: paradoxical rise in JVP on inspiration due to restricted filling of the ventricles  
Palpable apex beat  
3rd heart sound  
Ascites  
Ankle oedema  
Hepatomegaly
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11
Q

What 3 common ECG features are seen in all types of cardiomyopathy?

A

Non-specific ST changes
Conduction defects
Arrhythmias

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12
Q

What may be seen on CXR in cardiomyopathy?

A

Cardiomegaly

Signs of heart failure: pulmonary oedema

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13
Q

What is seen in the ECG of restrictive cardiomyopathy?

A

Low voltage complexes

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14
Q

Which investigation is diagnostic and can differentiate between types of cardiomyopathy?

A

Echocardiography

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15
Q

What is seen on echocardiography in dilated cardiomyopathy?

A

Dilated ventricles with global hypokinesia + low ejection fraction
MR, TR, LV thrombus

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16
Q

What is seen on echocardiography in hypertrophic cardiomyopathy?

A

Ventricular hypertrophy (asymmetrical septal hypertrophy)

17
Q

What is seen on echocardiography in restrictive cardiomyopathy?

A

Non-dilated non-hypertrophied ventricles
Atrial enlargement
Preserved systolic function
Diastolic dysfunction
Granular/ sparkling appearance of myocardium in amyloidosis

18
Q

List 3 other investigations that may be performed in patients with cardiomyopathy

A

Cardiac Catheterisation: help exclude coronary disease
Endomyocardial Biopsy
Pedigree or Genetic Analysis

19
Q

What is dilated cardiomyopathy?

A

Dilated, flabby heart of unknown cause.
Thickening of inner layer of heart chambers, heart muscles stretch + weaken
Impairment of contractility (systolic function)

20
Q

What is restrictive cardiomyopathy?

A

Cardiac cells replaced with abnormal tissue e.g. scar tissue, causing muscle walls of ventricles to become stiff so relaxation (filling) phase is abnormal.
Impairment of compliance (diastolic function)

21
Q

Describe the pathophysiology of arrhythmogenic cardiomyopathy

A

Autosomal dominant with variable expression
RV myocardium replaced with fatty + fibrofatty tissue

22
Q

Give 3 presentations of arrhythmogenic cardiomyopathy

A

Palpitations
Syncope
Sudden cardiac death

23
Q

What ECG abnormalities can be seen in arrhythmogenic RV cardiomyopathy ?

A

T wave inversion in V1-3
Epsilon wave in ~50%= terminal notch in QRS complex

24
Q

What is seen on echo in arrythmogenic RV cardiomyopathy?

A

Often subtle in early stages
Enlarged, hypokinetic right ventricle with a thin free wall

25
Q

What can be seen on MRI in arrhthmogenic RV cardiomyopathy?

A

Fibrofatty tissue

26
Q

What is the management of arrhythmogenic RV cardiomyopathy?

A

Sotalol
Catheter ablation to prevent VT
Implantable cardioverter-defibrillator

27
Q

What is Naxos disease? What triad of features characterises this?

A

Autosomal recessive variant of Arrhythmogenic RV cardiomyopathy
ARVC
Palmoplantar keratosis
Woolly hair

28
Q

What is hypertrophic cardiomyopathy?

A

Autosomal dominant disorder of muscle tissue caused by defects in genes encoding contractile proteins

29
Q

How may HOCM present?

A

Asymptomatic
Exertional dyspnoea
Angina
Syncope
Sudden death

30
Q

Describe the syncope experienced in HOCM

A

Typically following exercise
Due to sub aortic hypertrophy of the ventricular septum, resulting in functional aortic stenosis

31
Q

What is sudden death in HOCM usually due to?

A

Ventricular arrhythmias

32
Q

List 5 signs of hypertrophic cardiomyopathy on examination

A

Jerky carotid pulse
Double apex beat
Systolic murmurs
Systolic thrill at LLSE
Large ‘a’ waves

33
Q

Which murmurs may be heard in HOCM?

A

ESM: due to LV outlflow tract obstruction. Increases with Valsalva manoeuvre, decreases on squatting

Pansystolic: due to systolic anterior motion of the mitral valve - mitral regurgitation

34
Q

Give 3 features of ECG in hypertrophic cardiomyopathy

A

Ventricular hypertrophy
Non-specific ST segment + T wave abnormalities, progressive T inversions
Deep Q waves in leads II + III

35
Q

HOCM is associated with which 2 conditions?

A

Friedreich’s ataxia
Wolff-Parkinson White

36
Q

What are the findings on echo in HOCM?

A

MR SAM ASH
Mitral regurgitation (MR)
Systolic anterior motion (SAM) of the anterior mitral valve leaflet
Asymmetric hypertrophy (ASH)

37
Q

What is used in the management of HOCM?

A

ABCDE
Amiodarone
B-blockers or Verapamil for Sx
Cardioverter defibrillator
Dual chamber pacemaker
(Endocarditis prophylaxis)

38
Q

List 3 drugs that must be avoided in HOCM

A

Nitrates
ACEi: reduce afterload which may worsen LV outflow tract gradient
Inotropes