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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?

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
what is amyloid
deposition of proteins that body can't get rid of
26
symptoms of restrictive cardiomyopathy?
RV failure
27
signs of restrictive cardiomyopathy?
elevated JVP, hepatomegaly, ascites, oedema
28
ix for restrictive cardiomyopathy
echo- bi-atrial dilation due to back pressure | ECG, MRI
29
tx for restrictive cardiomyopathy
treat underlying cause
30
True/False... | ECG abnormalities seen and arrhythmias never develop in channelopathy disease?
False... | ECG abnormalities are seen and arrhythmias OFTEN develop
31
what are 2 main channelopathy diseases
Long QT Syndrome | Brugada Syndrome
32
what is long QT syndrome?
abnormal cardiac electrophysiology which causes prolonged QT interval
33
cause of LQTS?
genetic
34
what may trigger LQTS?
exercise, loud noise, swimming underwater, QT prolonging states e.g. hypokalaemia
35
what are presenting symptoms of LQTS?
syncope & SCD
36
what arrhythmia is common in LQTS
torsades de pointes VT, AF, heart block
37
what is seen on ECG for LQTS
QT is long (>440/450ms)
38
what is mx for LQTS?
BBs | lifestyle: avoid triggers, maintain high K+ diet
39
True/ False... | young men are more susceptible to Brugada syndrome?
True
40
what is Brugada syndrome?
abnormal cardiac electrophysiology which leads to fatal arrhythmias
41
cause of Brugada?
genetic
42
what may trigger Brugada?
sleeping, fever, excess alcohol, large meals
43
presenting symptoms of Brugada?
syncope, palpitations, pre-syncope, chest pain, breathless, SCD
44
arrhythmias associated with Brugada?
polymorphic VT, VFib, AFib
45
ix of Brugada?
provocative testing | ECG- RBBB, ST elevation in V1-V3
46
Mx of Brugada?
- Avoid drugs that induce Brugada e.g. anti-histamines - Treat pyrexia - ICD device (implanted cardioverter defibrillator) if ventricular arrhythmia present - Lifestyle: alcohol avoidance
47
which ICD is better; Subcutaneous- ICD or Transvenous-ICD?
depends but usually S-ICD

Cardiovascular (12 decks)

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