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RACP Written 2018 > Cardiology - Cardiomyopathy > Flashcards

Cardiology - Cardiomyopathy Flashcards

1
Q

Is Takotsubo Cardiomyopathy more common in females or males?

A

Females

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2
Q

Pathogenesis of Takotsubo Cardiomyopathy

A
  • Transient LV ballooning

- thought to be related to catecholamine excess

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3
Q

Hallmark feature of a Takotsubo Cardiomyopathy…which part of the heart is affected?

A

There is wall motion abnormalities BEYOND a single territory

Brought on by acute emotional stressors

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4
Q

ECG features of Takotsubo Cardiomyopathy?

A
  • ST elevation in precordial leads
  • QT prolongation
  • Widespread T wave inversion
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5
Q

Management of Takotsubo Cardiomyopathy?

A

Acute: ACE inhibitor and BB
Supportive treatment
Don’t give GTN

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6
Q

Natural progression of Takotsubo Cardiomyopathy

A

95% will have resolution of symptoms and LV recovery within 7 days

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7
Q

What is the leading cause of death in young athletes <35 years old?

A

HOCM

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8
Q

HOCM:

Inheritance pattern?

A

Autosomal dominant

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9
Q

HOCM:

Which genes are mutated usually?

A

Usually genes encoding BETA MYOSIN HEAVY CHAIN PROTEIN

Usually sarcomere genes:

  • MYBPC3: cardiac myosin binding protein C
  • MYH7: beta myosin heavy chain
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10
Q

What happens the FUNCTION of the heart in HOCM?

A

DIASTOLIC dysfunction and poor myocardial COMPLIANCE

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11
Q

What are the cardinal structural features of HOCM

A

Septal hypertrophy
and
Anterior displacement of the mitral valve

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12
Q

What kind of outflow problem happens in HOCM?

A

Dynamic LVOT in 66%

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13
Q

What WORSENS the outflow problem in HOCM?

A 
Reduced preload (ie dehydration)
Reduced afterload (ie vasodilators)
Drugs that enhance outflow: digoxin and inotropes
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14
Q

Pulse in HOCM?

A

Rapid and bifid carotid upstroke (=pulsus bisferiens) if there is LVOT obstruction

And

‘Jerky’ pulse

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15
Q

JVP in HOCM

A

high ‘a’ wave if significant infundibular hypertrophy

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16
Q

Apex in HOCM

A

Sustained, localised and bifid or trifid

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17
Q

Extra heart sounds in HOCM?

A

S4 (especially if young)

Paradoxical splitting of S2 if significant LVOT obstruction

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18
Q

Murmur in HOCM?

A
  • Mitral regurgitation
  • systolic with ejection quality at LLSB
  • INCREASES with valsalva (decreased preload)
  • INCREASES with nitrates (decreased afterload
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19
Q

Other diseases associated with HOCM?

A

Freidreich’s ataxia

WPW

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20
Q

Complications of HOCM?

A

AF in 20 - 25%

Sudden cardiac death

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21
Q

Risk factors for Sudden Cardiac Death in HOCM?

A
  • Family history of premature cardiac death
  • Recurrent syncope
  • Nonsustained VT (in adults)
  • Severe LVH (septum >2.5 - 3cm)
  • Severe obstruction
  • Abnormal exercise BP response
  • Level of myocardial fibrosis on MRI (contraversial)
  • Genotype: Arg719Trp
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22
Q

What is the MOST SENSITIVE test to diagnose HOCM?

A

ECG

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23
Q

What ECG findings in HOCM?

A

LVH in 80 - 90%
Progressive T wave inversion
Deep Q waves
+/- AF

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24
Q

What echo findings in HOCM?

A

Systolic anterior motion of anterior mitral valve leaflet
and
Asymmetric hypertrophy

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25
Q

How do you quantify LVOT obstruction in HOCM? What makes it severe?

A

Severe LVOT obstruction if pressure gradient is:
>=30mmHg at rest
>=50mmHg at exertion/provocation

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26
Q

Why is MRI useful in HOCM?

A

Better for eccentric and apical atrophy

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27
Q

Primary prevention measures in HOCM?

A

Betablockers
ICD if indicated
Anticoagulation if AF

28
Q

What indications for ICD insertion in HOCM?

A
  • FHx of sudden death
  • interventricular septum >30mm
  • Unexplained syncope
  • NSVT >3 beats on holter
  • Blunted increase (<20mmHg) in BP on exercise
29
Q

Drugs to avoid in HOCM?

A

Nitrates
ACE inhibitors
Inotropes
Digoxin

30
Q

How to reduce the LVOT obstruction in HOCM?

A

Septal surgical myectomy:

  • GOLD STANDARD with symptom relief in >90%
  • low peri-op mortality

Alcohol ablation (better if high risk BUT then needs PPM)

31
Q

Who needs genetic screening if their relative has HOCM?

A 
In ALL first degree relatives (examination, ECG and echo):
Age 0 - 10yrs: every 2-3 years
Age 11-20yrs: every 1 year
Age 21-30yrs: every 2-3 years
Age >31yrs: every 3-5 years
32
Q

Poor PROGNOSTIC factors for HOCM?

A
  • Syncope
  • FHx of sudden death
  • YOUNG age at presentation
  • NSVT
  • Abnormal BP response to exercise
  • increased septal wall thickness
33
Q

Causes of RESTRICTIVE cardiomyopathy?

A 
Amyloidosis
Post-Radiotherapy
Scleroderma
Fabry's
Loeffler's endocarditis
Haemochromatosis
Familial restrictive cardiomyopathy
34
Q

In RESTRICTIVE cardiomyopathy, how does the apex compare on examination to DILATED?

A

Apex is LESS DISPLACED than in dilated CM

35
Q

In RESTRICTIVE cardiomyopathy, how does the apex compare on examination to DILATED?

A

Apex is LESS DYNAMIC than in dilated CM

36
Q

First line management in RESTRICTIVE cardiomyopathy?

A

Negative chronotropic agents (CCBs and BBs) to lengthen diastolic filling time and improve myocardial relaxation

ACE inhibitors MAY improve diastolic dysfunction

37
Q

In RESTRICTIVE cardiomyopathy when might digoxin need to be avoided?

A

In AMYLOID cause of restrictive cardiomyopathy don’t use digoxin as it binds to amyloid fibrils and patient then becomes digoxin-toxic

38
Q

Differentiating Constrictive versus Restrictive:

Apex beat?

A

Diminished in constrictive

39
Q

Differentiating Constrictive versus Restrictive:

Diastolic elevated BP?

A

Seen in BOTH

40
Q

Differentiating Constrictive versus Restrictive:

Kaussmaul’s sign?

A

Seen in BOTH

41
Q

Differentiating Constrictive versus Restrictive:

High BNP levels?

A

Seen in RESTRICTIVE

42
Q

Differentiating Constrictive versus Restrictive:

CT with pericardial thickening and calcification?

A

Seen in CONSTRICTIVE

43
Q

Differentiating Constrictive versus Restrictive:

Echo with equalisation of R and L ventricular diastolic pressures?

A

Seen in CONSTRICTIVE

44
Q

Differentiating Constrictive versus Restrictive:

Peripheral oedema?

A

Seen in BOTH

45
Q

Differentiating Constrictive versus Restrictive:

Symptoms of HF OUT OF PROPORTION to systolic dysfunction?

A

Seen in BOTH

46
Q

Differentiating Constrictive versus Restrictive:

Elevated JVP? Which JVP abnormality might you see?

A

Seen in BOTH

** prominant ‘y’ descent due to accentuation of early filling

47
Q

Differentiating Constrictive versus Restrictive:

Presence of pulmonary hypertension?

A

Seen in RESTRICTIVE more commonly (as more severe diastolic abnormality)

48
Q

Differentiating Constrictive versus Restrictive:

Early diastolic filling sounds?

A

In CONSTRICTIVE: ‘knock’

In RESTRICTIVE: ‘S3’

49
Q

Dilated Cardiomyopathy:

What infective diseases can cause it?

A

VIRAL: coxsackie B, HIV, adenovirus and Hep C
BACTERIAL: diphtheria
PARASITE: Chagas and Toxoplasma
Q fever

50
Q

Dilated Cardiomyopathy:

Which rheum conditions are associated?

A

Poly and Dermatomyositis
Sarcoid
Collagen vascular disease
Giant cell myocarditis and eosinophilic myocarditis

51
Q

Dilated Cardiomyopathy:

Which toxins/drugs may cause it?

A 
Alcohol
Catecholamines
Chemo: anthracyclines and Trastuzumab
Interferon
Hydroxychloroquine
Lead 
Mercury
52
Q

Dilated Cardiomyopathy:

Which neuromuscular diseases are associated?

A

Duchennes
Beckers muscular dystrophy
Mitochondrial myopathy

53
Q

Dilated Cardiomyopathy:

Which endocrine conditions are associated?

A

HypERthyroidism
Diabetes
Pheochromocytoma

54
Q

Dilated Cardiomyopathy:

Which nutritional deficiencies are causes?

A 
Thiamine deficiency
Selenium deficiency
Niacin deficiency
Carnitine deficiency (= cofactor in long chain fatty acid metabolism)
55
Q

Dilated Cardiomyopathy:

Which electrolyte abnormalities can cause it?

A

Hypocalcaemia
Hypomagnesaemia
Hypophosphataemia

56
Q

Dilated Cardiomyopathy:

Which genetic conditions can cause it?

A
  • Gene coding TTN (titin) is most common
  • LMNA
  • SCN5A
  • PRKAG2 (also assoc WPW and AV block)
  • LAMP (Danon’s disease)
57
Q

What is Danon’s Disease?

A

X-linked mutationin LAMP (lysosome associated membrane protein)

  • skeletal myopathy
  • mental retardation
  • LFT derangement
  • EXTREME LVH!!
58
Q

Other than dilated cardiomyopathy what is the gene PRKAG2 associated with?

A

WPW

AV block

59
Q

When does Peri-Partum Cardiomyopathy present?

A

Develops in the last month of pregnancy up until 5 months post partum

60
Q

Pathological process implicated in Peri-Partum Cardiomyopathy?

A

Prolactin Processing with 16kDA prolactin fragment causing endothelial and myocardial damage

61
Q

Risk factors for Peri-Partum Cardiomyopathy

A
  • Age >30 years
  • African
  • Multi-foetus pregnancy
  • Greater parity
  • Multiple gestations
  • Maternal COCAINE abuse
  • Hx of pre-eclampsia / eclampsia / postpartum hypertension
  • Longterm (>4 weeks) tocolytics with beta-agonists (ie TERBUTALINE)
62
Q

What to avoid in peri-partum cardiomyopathy?

A

ACE inhibitors, ARBs

Aldosterone antagonists

63
Q

What is a woman at high risk of if she has peri-partum cardiomyopathy?

A

HIGH risk of thrombus

64
Q

When to avoid pregnancy with hx of peri-partum cardiomyopathy?

A

Avoid 2nd pregnancy if:

  • EF persistently <50%
  • EF <25% at diagnosis
65
Q

How does BROMOCRIPTINE work in peri-partum cardiomyopathy?

A

Bromocriptine causes prolactin blockade activating dopamine D2 receptors

and actives post-synaptic dopamine receptors in TUBEROFUNDIBULAR pathway (–> inhibits prolactin secretion)
and in NIGROSTRIATAL pathway (–> enhances motor control)

RACP Written 2018 (167 decks)

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