Cardiomyopathy Flashcards

1
Q

what is the commonest cause of sudden cardiac death in young adults?

A

hypertrophic cardiomyopathy

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2
Q

what is cardiomyopathy?

A

structural and functional abnormality of the myocardium without coronary artery disease, hypertension, valvular or congenital heart diseases

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3
Q

what are the 4 main types of cardiomyopathy?

A
  1. dilated
  2. hypertrophic
  3. restricted
  4. arrhythmogenic right ventricular cardiomyopathy
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4
Q

what is the most common type of cardiomyopathy?

A

dilated cardiomyopathy

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5
Q

what are the cause of dilated cardiomyopathy?

A
  • ischaemic changes
  • hypertensive
  • genetic and congenital
  • toxin-related (e.g. alcohol abuse, cocaine, chloroquine, clozapine)
  • infiltrative (e.g. haemochromatosis, amyloidosis, sarcoidosis)
  • peripartum
  • thyotoxicosis
  • infectious
  • stress
  • idiopathic
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6
Q

what medications can cause dilated cardiomyopathy?

A
  • anthracyclin chemotherapy (doxorubicin)
  • cyclophosphamide
  • anti-retroviral drugs (zidovudine)
  • chloroquine
  • clozapine
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7
Q

what is takotsubo cardiomyopathy?

A

transient left ventricular ballooning precipitated by intense psychologic stress

stress-induced cardiomyopathy

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8
Q

what are the typical symptoms in dilated cardiomyopathy?

A
  • exertional dyspnoea
  • orthopnoea
  • paroxysmal nocturnal dyspnoea
  • peripheral oedema
  • arrhythmia - AF or ventricular tachycardia
  • conduction disturbances
  • sudden cardiac death
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9
Q

what are the examination findings in dilated cardiomyopathy?

A
  • displaced apex beat
  • S3 gallop rhythm (rapid ventricular filling)
  • murmur of mitral regurgitation - due to displacement of the valve leaflets
  • signs of heart failure - e.g. oedema, hepatomegaly, ascites, raised JVP
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10
Q

what is the diagnostic test for dilated cardiomyopathy?

A

echocardiogram - may show poor R-wave progression

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11
Q

what is hypertrophic cardiomyopathy (HCM)?

A

genetic condition characterised by left ventricular hypertrophy of varying degrees

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12
Q

what is the pathophysiology of hypertrophic cardiomyopathy?

A

mutation in one of several myocyte sarcomere genes such as myosin and troponin, caysing myocyte hypertrophy and disarray

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13
Q

what is the inheritance of hypertrophic cardiomyopathy?

A

autosomal dominant - though 50% of cases are sporadic mutations

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14
Q

what are the consequences from hypertrophic cardiomyopathy?

A
  • left ventricular outflow tract obstruction (LVOTO)
  • disastolic dysfunction
  • ischaemia
  • mitral regurgitation
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15
Q

what are the typical symptoms of hypertrophic cardiomyopathy?

A
  • presyncope
  • syncope
  • sudden death
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16
Q

what are the clinical signs of hypertrophic cardiomyopathy?

A
  • ‘jerky’ pulse
  • double apex beat
  • harsh ejection systolic murmur
  • apical thrill
17
Q

what ECG findings are seen in hypertrophic cardiomyopathy?

A
  • abnormal Q waves
  • deeply inverted T waves
  • left ventricular hypertrophy
18
Q

how do you diagnose hypertrophic cardiomyopathy?

A

echocardiography = left ventricular wall thickness in the absence of any other causes

19
Q

what is restrictive cardiomyopathy?

A

involves non-dilated non-hypertrophied ventricles with impaired ventricular filling

20
Q

what are the broad causes of restrictive cardiomyopathy?

A
  • familial non-infiltrative cardiomyopathy (inherited genetic disorders)
  • infiltrative disorders
  • storage disorders
  • others
21
Q

how do you diagnose restrictive cardiomyopathy?

A
  • echocardiogram (thickened ventricular walls and valves)
  • cardiac MRI - used to distinguish between restrictive cardiomyopathy and constrictive pericarditis
22
Q

what is the presenting features of hereditary haemochromatosis?

A
  • dilated cardiomyopathy
  • diabetes mellitus
  • joint pain
  • hepatomegaly
  • ascites
23
Q

what is hereditary haemochromatosis?

A

iron deposition affects the liver, pancreas, joints and heart. it can also affect the skin, pituitary and adrenal glands

24
Q

what is amyloidosis?

A

amyloid protein deposition in various tissues in the body, such as kidneys and the heart

25
Q

what kind of cardiomyopathy can amyloidosis cause?

A

restrictive

26
Q

describe infiltrative causes of restrictive cardiomyopathy

A
  • amyloidosis
  • sarcoidosis
  • gaucher disease
  • hurler syndrome
  • fatty infiltration
27
Q

describe the storage causes of restrictive cardiomyopathy

A
  • haemochromatosis
  • fabry disease
  • glycogen storage disorders
28
Q

describe the non-familial/infiltrative/storage causes of restrictive cardiomyopathy

A
  • diabetic cardiomyopathy
  • scleroderma
  • hypereosinophilic syndrome (lofflers)
  • radiation
  • chemotherapy familial non-infiltrative cardiomyopathy (inherited genetic disorders)