Year 3 > Cardiomyopathy > Flashcards

Cardiomyopathy Flashcards

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1
Q

What is hypertrophic obstructive cardiomyopathy (HOCM)?

A

An autosomal dominant disorder of muscle tissue caused by defects in the genes encoding contractile proteins.

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2
Q

What is the estimated prevalence of HOCM?

A

1 in 500.

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3
Q

What is the most common cause of sudden cardiac death in the young?

A

Hypertrophic obstructive cardiomyopathy (HOCM).

A key risk factor for sudden cardiac death in the unexplained syncope

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4
Q

What are the most common genetic defects associated with HOCM?

A

Mutations in the gene encoding β-myosin heavy chain protein or myosin-binding protein C.

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5
Q

What type of dysfunction is primarily seen in HOCM?

A

Predominantly diastolic dysfunction.

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6
Q

What are the characteristic findings on biopsy for HOCM?

A

Myofibrillar hypertrophy with chaotic myocyte disarray and fibrosis.

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7
Q

List common symptoms of HOCM.

A
  • Exertional dyspnoea
  • Angina
  • Syncope
  • Sudden death
  • Arrhythmias
  • Heart failure.
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8
Q

What causes syncope in HOCM?

A

Subaortic hypertrophy of the ventricular septum, resulting in functional aortic stenosis.

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9
Q

What are the common pulse and heart sounds associated with HOCM?

A
  • Jerky pulse
  • Large ‘a’ waves
  • Double apex beat.
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10
Q

What type of murmurs are associated with HOCM?

A
  • Ejection systolic murmur
  • Pansystolic murmur.
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11
Q

What is the mnemonic for echo findings in HOCM?

A

MR SAM ASH.

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12
Q

What does MR stand for in the echo findings mnemonic for HOCM?

A

Mitral regurgitation.

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13
Q

What does SAM stand for in the echo findings mnemonic for HOCM?

A

Systolic anterior motion of the anterior mitral valve leaflet.

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14
Q

What does ASH stand for in the echo findings mnemonic for HOCM?

A

Asymmetric hypertrophy.

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15
Q

What ECG findings are commonly seen in HOCM?

A
  • Left ventricular hypertrophy
  • Non-specific ST segment and T-wave abnormalities
  • Deep Q waves.
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16
Q

What are the management options for HOCM?

A
  • Amiodarone
  • Beta-blockers or verapamil
  • Cardioverter defibrillator
  • Dual chamber pacemaker
  • Endocarditis prophylaxis.
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17
Q

List drugs to avoid in HOCM.

A
  • Nitrates
  • ACE-inhibitors
  • Inotropes.
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18
Q

What is arrhythmogenic right ventricular dysplasia (ARVD)?

A

A condition where right ventricular myocardium is replaced by fatty and fibrofatty tissue.

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19
Q

What percentage of ARVD patients have a mutation in desmosome components?

A

Around 50%.

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20
Q

What is a common ECG finding in ARVD?

A

T wave inversion in leads V1-3.

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21
Q

What is the epsilon wave in ARVD?

A

A terminal notch in the QRS complex found in about 50% of patients.

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22
Q

What are the common presentations of ARVC?

A
  • Palpitations
  • Syncope
  • Sudden cardiac death.
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23
Q

What is the classic triad of Naxos disease?

A
  • ARVC
  • Palmoplantar keratosis
  • Woolly hair.
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24
Q

What are the classic causes of dilated cardiomyopathy?

A
  • Alcohol
  • Coxsackie B virus
  • Wet beri beri
  • Doxorubicin.
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25
What are the classic causes of restrictive cardiomyopathy?
* Amyloidosis * Post-radiotherapy * Loeffler's endocarditis.
26
What drug is most widely used as an antiarrhythmic in ARVC?
Sotalol.
27
What is the role of catheter ablation in ARVC management?
To prevent ventricular tachycardia.
28
What is hypertrophic obstructive cardiomyopathy (HOCM)?
An autosomal dominant disorder of muscle tissue caused by defects in the genes encoding contractile proteins.
29
What is the estimated prevalence of HOCM?
1 in 500.
30
What is the most common cause of sudden cardiac death in the young?
Hypertrophic obstructive cardiomyopathy (HOCM).
31
What are the most common genetic defects associated with HOCM?
Mutations in the gene encoding β-myosin heavy chain protein or myosin-binding protein C.
32
What type of dysfunction is primarily seen in HOCM?
Predominantly diastolic dysfunction.
33
What are the characteristic findings on biopsy for HOCM?
Myofibrillar hypertrophy with chaotic myocyte disarray and fibrosis.
34
List common symptoms of HOCM.
* Exertional dyspnoea * Angina * Syncope * Sudden death * Arrhythmias * Heart failure.
35
What causes syncope in HOCM?
Subaortic hypertrophy of the ventricular septum, resulting in functional aortic stenosis.
36
What are the common pulse and heart sounds associated with HOCM?
* Jerky pulse * Large 'a' waves * Double apex beat.
37
What type of murmurs are associated with HOCM?
* Ejection systolic murmur * Pansystolic murmur.
38
What is the mnemonic for echo findings in HOCM?
MR SAM ASH.
39
What does MR stand for in the echo findings mnemonic for HOCM?
Mitral regurgitation.
40
What does SAM stand for in the echo findings mnemonic for HOCM?
Systolic anterior motion of the anterior mitral valve leaflet.
41
What does ASH stand for in the echo findings mnemonic for HOCM?
Asymmetric hypertrophy.
42
What ECG findings are commonly seen in HOCM?
* Left ventricular hypertrophy * Non-specific ST segment and T-wave abnormalities * Deep Q waves.
43
What are the management options for HOCM?
* Amiodarone * Beta-blockers or verapamil * Cardioverter defibrillator * Dual chamber pacemaker * Endocarditis prophylaxis.
44
List drugs to avoid in HOCM.
* Nitrates * ACE-inhibitors * Inotropes.
45
What is arrhythmogenic right ventricular dysplasia (ARVD)?
A condition where right ventricular myocardium is replaced by fatty and fibrofatty tissue.
46
What percentage of ARVD patients have a mutation in desmosome components?
Around 50%.
47
What is a common ECG finding in ARVD?
T wave inversion in leads V1-3.
48
What is the epsilon wave in ARVD?
A terminal notch in the QRS complex found in about 50% of patients.
49
What are the common presentations of ARVC?
* Palpitations * Syncope * Sudden cardiac death.
50
What is the classic triad of Naxos disease?
* ARVC * Palmoplantar keratosis * Woolly hair.
51
What are the classic causes of dilated cardiomyopathy?
* Alcohol * Coxsackie B virus * Wet beri beri * Doxorubicin.
52
What are the classic causes of restrictive cardiomyopathy?
* Amyloidosis * Post-radiotherapy * Loeffler's endocarditis.
53
What drug is most widely used as an antiarrhythmic in ARVC?
Sotalol.
54
What is the role of catheter ablation in ARVC management?
To prevent ventricular tachycardia.
55
What is the second most common cause of sudden cardiac death in young people after hypertrophic obstructive cardiomyopathy?
arrhythmogenic right ventricular dysplasia (ARVD). This is an inherited cardiac disorder
56
What are the ECG findings for ARBD?
T-wave inversion in leads V1-3 and a terminal notch in the QRS complex (epsilon wave
57
When is the murmur with HOCM louder?
Performing valsalva
58
When is the murmur with HOCM quieter??
On squatting
59
What is the most common cause of cardiomyoapthy?
Idiopathic dilated cardiomyopathy is often caused by sporadic genetic mutations; They often present between the third and sixth decades of life with signs and symptoms of heart failure, in addition to arrhythmias such as atrial fibrillation.
60
What to suspect in young patients with new chest pain?
Cocain induced cardiomyopathy from induce coronary arterial vasospasm, leading to myocardial ischaemia
61
How does HOCM present?
typically manifest by the early third decade of life. Typical symptoms of HOCM are predominantly palpitations and chest pain
62
What are the causes of dilated cardiomyoapthy,
idiopathic alcohol Cocksackie B virus (via an acute myocarditis), wet beri beri
63
Which drugs are linked to dilated cardiomyoapthy.
anthracycline chemotherapy (Doxorubicin), Cyclophosphamide antiretroviral drugs (Zidovudine) Chloroquine Clozapine.
64
What PMHx is linked to dilated cardiomyoapthy?
Infiltrative - haemochromatosis, amyloidosis and sarcoidosis Thyrotoxicosis
65
What cardiomyoapthy is linked sparkling echocardiogram appearance?
Amyloidosis, which causes Restrictive cardiomyopathy and significant oedema
66
What dysfunction is caused by HOCM?
Beta myosin heavy chain mutation
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