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Block A > WCS8 Cardiology inherited cardiac diseases > Flashcards

WCS8 Cardiology inherited cardiac diseases Flashcards

1
Q

types of inherited cardiac diseases

A
  • inherited cardiomyopathies
  • inherited disorders of rhythm and conduction
  • familial hypercholesterolemia
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2
Q

Are VSD, ASD, WPW inherited cardiac diseases?

A

no. they are congenital

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3
Q

types of inherited cardiomyopathies (morphological classification)

A
  • hypertrophic
  • dilated
  • restricted
  • arrhythmogenic right ventricular disease
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4
Q

types of inherited disorders of rhythm and conduction

A
  • Long QT syndrome
  • Brugada syndrome
  • CPVT
  • PCD
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5
Q

feature of cardiomyopathies

A

inappropriate ventricular hypertrophy or dilation

–> mechanical and/or electrical dysfunction

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6
Q

morphological abnormality in hypertrophic cardiomyopathy

A

inappropriate LVH +/- LVOT obstruction

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7
Q

morphological abnormality in dilated cardiomyopathy

A

LV dilation

Systolic dysfunction

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8
Q

morphological abnormality in arrhythmogenic RV cardiomyopathy

A

fibroadipose infiltrate

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9
Q

morphological abnormality in restrictive cardiomyopathy

A

abnormal LV filling

diastolic dysfunction

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10
Q

Pathophysiology of HOCM

A
  • LVOT obstruction
  • diastolic dysfunction
  • myocardial ischemia
  • MR
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11
Q

Risk factors for sudden death in HCM

A 
High risk
- cardiac arrest 
- sustained vtach
moderate to high risk
- FH of premature sudden death
- extreme LVH 
- abnormal exercise blood pressure
- unexplained syncope
- NSVT
low risk
- mild LVH 
others
- afib
- mi
- LVOT obstruction
- high risk mutation
- intense physical exertion
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12
Q

Hallmark of RCM

A

rigid ventricular walls with ventricular filling dysfunction
–> normal systolic but abnormal diastolic function

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13
Q

secondary causes of hcm

A

amyloidosis
pheochromocytoma
metabolic disease

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14
Q

secondary causes of rcm

A

amyloidosis
hemochromatosis
endomyocardial fibrosis/disease

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15
Q

condition with functional resemblance as rcm

A

constrictive pericarditis

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16
Q

causes for DCM

A
  • viral
  • valvular
  • toxin
  • ischemic
  • infiltrative
  • idiopathic
17
Q

complications for DCM

A
  • heart failure

- sudden death

18
Q

Pathogenesis of DCM

A
  • Genetic factors –> DNA mutation –> altered gene products –> altered myocardial function –> dcm
  • genetic factors –> MHC –> alteration of immune system –> t cell dysfunction autoantibodies –> altered myocardial function –> DCM
  • viral infection –> myocarditis –> interference with immune response –> tcell dysfunction autoantibodies –> altered myocardial function –> dcm
  • viral infection –> myocarditis –> viral persistence –> altered myocardial function –> dcm
19
Q

LQTS hallmarks

A

QT prolongation on ECG

propensity to Vtach

20
Q

causes of LQTS

A

congenital - ion channelopathies (sporadic, Jervell& Lange-Neilson, Romano-ward)

acquired

  • drug - abx, antiarrhythmic, psychotropics
  • myocardial (ischemia, MVP, myocarditis),
  • metabolic disturbance - DM, hyperparathyroid, hypothyroid, pheo,
  • electrolyte abnormalities,(hypoK,Ca, Mg)
  • malnutrition,
  • CNS - hemorrhage, trauma, cva, encephalitis
21
Q

Pathophysiology of LQTS

A

reduced repolarization current/ enhanced depolarization current –> prolonged ventricular repolarization (QT)

22
Q

How to differentiate LQT from LQTS

A
  • Schwartz score clinical syndrome recognition (fh, symptoms, ecg)
  • exercise testing
  • genetic identification
23
Q

Treatment of LQTS

A
  • B-blockers
  • lifestyle advices
  • left cervicothoracic stellectomy
  • icd implantation
  • pacemaker implantation
24
Q

Brugada syndrome hallmarks

A
  • ST elevation (coved/saddle-back type) in right precordial leads +/- apparent RV conduction block
  • heart grossly structurally normal
  • propensity for life-threatening Vtachs
25
Q

What drug can be used to unmask Brugada syndrome, what will be seen after admin?

A

Na channel blockers

polymorphic VT

26
Q

treatment for symptomatic brugada

A

ICD

27
Q

familial hypercholesterolemia inheritance

A

AD

28
Q

familial hypercholesterolemia biochemical finding

A

elevated LDL

29
Q

familial hypercholesterolemia associated risk

A

premature atherosclerotic CHD

30
Q

familial hypercholesterolemia treatment

A

statins +/- ezetimibe, bile acid sequestrants, niacin

Block A (11 decks)

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