Cardiomyopathies Flashcards

1
Q

What are 2 genetic cardiomyopathies?

A
  1. Hypertrophic obstructive cardiomyopathy
  2. Arrhythmogenic right ventricular dysplasia
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2
Q

What is hypertrophic obstructive cardiomyopathy?

A
  • Leading cause of sudden cardiac death in young athletes
  • Usually due to a mutation in the gene encoding β-myosin heavy chain protein
  • Common cause of sudden death
  • Echo findings include:
    • MR
    • systolic anterior motion (SAM) of the anterior mitral valve
    • asymmetric septal hypertrophy
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3
Q

What is arrhythmogenic right ventricular dysplasia?

A
  • Right ventricular myocardium is replaced by fatty and fibrofatty tissue
  • Around 50% of patients have a mutation of one of the several genes which encode components of desmosome
  • ECG:
    • abnormalities in V1-3, typically T wave inversion
    • An epsilon wave is found in about 50% of those with ARV - this is best described as a terminal notch in the QRS complex
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4
Q

Name mixed cardiomyopathies?

A
  • Dilated cardiomyopathy:
    • A dilated, flabby heart of unknown cause
  • Restrictive cardiomyopathy
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5
Q

What is the cause of dilated cardiomyopathy?

A

Classic causes include:

  • Alcohol
  • Coxsackie B virus
  • wet beri beri (thiamine deficiency)
  • doxorubicin
  • ↑bp
  • haemochromatosis
  • autoimmune
  • peri- or postpartum
  • thyrotoxicosis
  • congenital (x-linked)
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6
Q

What are the presentation and signs of dilated cardiomyopathy?

A

Presentation:

  1. Overall: fatigue, dyspnoea, pulmonary oedema
  2. Cardiac: RVF, Emboli, AF, VT

Signs:

  1. Overloaded: ↑Pulse, ↓bp, ↑jvp, oedema, pleural effusion
  2. Rhythm + cardiac: Displaced diffuse apex, S3 gallop, Mitral or tricuspid regurgitation (mr/tr)
  3. Abdominal: Jaundice, hepatomegaly, ascites
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7
Q

What tests do you order for dilated cardiomyopathy?

A

Blood:

  • plasma bnp
  • ↓Na+ indicates a poor prognosis

CXR:

  • cardiomegaly (baloon appearance)
  • pulmonary oedema

ECG:

  • tachycardia
  • non-specific t wave changes
  • poor r wave progression

ECHO:

  • globally dilated hypokinetic heart
  • low ejection fraction
  • Look for mr, tr, lv mural thrombus
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8
Q

What is the treatment for dilated cardiomyopathy?

A
  • Bed rest
  • Diuretics
  • Digoxin
  • ACE-i
  • Anticoagulation
  • Biventricular pacing
  • ICDS
  • Cardiac transplantation.
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9
Q

What are the causes of restrictive myopathy?

A
  1. Idiopathic
  2. Amyloidosis
  3. Haemochromatosis
  4. Sarcoidosis
  5. Scleroderma
  6. Löffler’s eosinophilic endocarditis
  7. Endomyocardial fibrosis.
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10
Q

What is the presentation of restrictive cardiomyopathy?

A
  1. Features of rvf predominate:
  • Overload: ↑jvp with prominent x and y descents, oedema
  • Abdomen: hepatomegaly; ascites
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11
Q

How do you diagnose and treat restrictive cardiomyopathy?

A

Cardiac catheterization + treat the cause

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12
Q

What are examples of acquired cardiomyopathy?

A
  • Peripartum cardiomyopathy
  • Takotsubo cardiomyopathy
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13
Q

What is peripartum cardiomyopathy?

A
  • Typical develops between last month of pregnancy and 5 months post-partum
  • More common in older women, greater parity and multiple gestations
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14
Q

What is takotsubo cardiomyopathy?

A
  • ‘Stress’-induced cardiomyopathy e.g. patient just found out family member dies then develops chest pain and features of heart failure
  • Transient, apical ballooning of the myocardium
  • Treatment is supportive
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15
Q

What are secondary cardiomyopathies?

A
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16
Q

What is the pathophysiology of dilated cardiomyopathy?

A
  1. Dilated heart leading to predominately systolic dysfunction
  2. All 4 chambers are dilated, but the left ventricle more so than right ventricle
  3. Eccentric hypertrophy (sarcomeres added in series) is seen