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Cardiovascular > Inherited Cardiac Conditions > Flashcards

Inherited Cardiac Conditions Flashcards

1
Q

what are 3 types of inherited cardiac conditions and what are they

A

cardiomyopathy (disease of muscle)
channelopathy (heart rhythm abnormality)
Aortopathy (arterial blood vessel abnormality)

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2
Q

what is tricky about cardiomyopathies

A

no ECG changes, asymptomatic

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3
Q

what do cardiomyopathies usually entail

A

changes in heart chamber size and thickness of heart

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4
Q

what are 4 types of cardiomyopathies

A

hypertrophic cardiomyopathy
arrhythmic RV cardiomyopathy
idiopathic dilated cardiomyopathy
restrictive

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5
Q

what is hypertrophic cardiomyopathy

A

big SOLID heart, diastolic dysfunction

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6
Q

what is cause of hypertrophic cardiomyopathy

A

mutation in sarcomere genes

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7
Q

symptoms of hypertrophic cardiomyopathy

A

SCD*, HF, angina, AFib, dyspnoea

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8
Q

signs of hypertrophic cardiomyopathy

A

jerky pulse, alpha wave in JVP, ejection systolic murmur

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9
Q

ix for hypertrophic cardiomyopathy

A

ECG: large QRS, T inversion
echo: bulging IV septum
clinical and genetic testing

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10
Q

tx for hypertrophic cardiomyopathy

A

BBs, verapamil

surgical (septal myomectomy)

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11
Q

what is arrhythmic RV cardiomyopathy

A

fibro-fatty replacement of cardiomyocytes

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12
Q

causes of arrhythmic RV cardiomyopathy

A

genetic, fatty diet/ atherosclerosis

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13
Q

symptoms of arrhythmic RV cardiomyopathy

A

syncope, SCD, arrhythmia

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14
Q

signs of arrhythmic RV cardiomyopathy

A

RV failure

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15
Q

ix for arrhythmic RV cardiomyopathy

A

clinical and genetic testing

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16
Q

tx for arrhythmic RV cardiomyopathy

A

BBs

surgical- cardiac transplant

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17
Q

what is idiopathic dilated cardiomyopathy?

A

big FLOPPY heart

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18
Q

causes of idiopathic dilated cardiomyopathy?

A

genetics, toxins e.g. alcohol, infection, pregnancy, fhx, chemo

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19
Q

symptoms of idiopathic dilated cardiomyopathy?

A

SOB, fatigue, PO, RV failure, tachycardia

20
Q

ix for idiopathic dilated cardiomyopathy?

A

CXR- cardiomegaly & PO
ECG- sinus tachycardia
Echo- globally dilated

21
Q

tx for idiopathic dilated cardiomyopathy?

A

ACEIs etc

transplant

22
Q

is idiopathic dilated cardiomyopathy more common in males or females?

A

males

23
Q

what is restrictive cardiomyopathy?

A

stiff, non-compliant heart = diastolic dysfunction

24
Q

causes of restrictive cardiomyopathy?

A

deposition of stuff in myocardium (metabolic by-products), sarcoid, tumours, fibrosis, amyloid

25
Q

what is amyloid

A

deposition of proteins that body can’t get rid of

26
Q

symptoms of restrictive cardiomyopathy?

A

RV failure

27
Q

signs of restrictive cardiomyopathy?

A

elevated JVP, hepatomegaly, ascites, oedema

28
Q

ix for restrictive cardiomyopathy

A

echo- bi-atrial dilation due to back pressure

ECG, MRI

29
Q

tx for restrictive cardiomyopathy

A

treat underlying cause

30
Q

True/False…

ECG abnormalities seen and arrhythmias never develop in channelopathy disease?

A

False…

ECG abnormalities are seen and arrhythmias OFTEN develop

31
Q

what are 2 main channelopathy diseases

A

Long QT Syndrome

Brugada Syndrome

32
Q

what is long QT syndrome?

A

abnormal cardiac electrophysiology which causes prolonged QT interval

33
Q

cause of LQTS?

A

genetic

34
Q

what may trigger LQTS?

A

exercise, loud noise, swimming underwater, QT prolonging states e.g. hypokalaemia

35
Q

what are presenting symptoms of LQTS?

A

syncope & SCD

36
Q

what arrhythmia is common in LQTS

A

torsades de pointes VT, AF, heart block

37
Q

what is seen on ECG for LQTS

A

QT is long (>440/450ms)

38
Q

what is mx for LQTS?

A

BBs

lifestyle: avoid triggers, maintain high K+ diet

39
Q

True/ False…

young men are more susceptible to Brugada syndrome?

A

True

40
Q

what is Brugada syndrome?

A

abnormal cardiac electrophysiology which leads to fatal arrhythmias

41
Q

cause of Brugada?

A

genetic

42
Q

what may trigger Brugada?

A

sleeping, fever, excess alcohol, large meals

43
Q

presenting symptoms of Brugada?

A

syncope, palpitations, pre-syncope, chest pain, breathless, SCD

44
Q

arrhythmias associated with Brugada?

A

polymorphic VT, VFib, AFib

45
Q

ix of Brugada?

A

provocative testing

ECG- RBBB, ST elevation in V1-V3

46
Q

Mx of Brugada?

A
  • Avoid drugs that induce Brugada e.g. anti-histamines
  • Treat pyrexia
  • ICD device (implanted cardioverter defibrillator) if ventricular arrhythmia present
  • Lifestyle: alcohol avoidance
47
Q

which ICD is better; Subcutaneous- ICD or Transvenous-ICD?

A

depends but usually S-ICD

Cardiovascular (12 decks)

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