inherited Flashcards

1
Q

what are the 3 basic type of inherited cardiac conditions

A

cardiomyopathy
channelopathy
arthropathies

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2
Q

examples of cardiomyopathies

A

hypertrophic cardiomyopathies
dilated cardiomyopathies
arrhythmogenic right ventricular cardiomyopathy

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3
Q

examples of channelopathies

A

long QT syndrome
brugada syndrome

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4
Q

example of arthropathies

A

Marfan syndrome

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5
Q

examples of arrhythmogenic inherited cardiac conditions

A

cardiomyopathies
1) hypertrophic cm
2) dilated cm
3) arrhythmogenic right ventricular cm

channelopathies
1) long QT syndrome
2) brugada syndrome
3) familial AF

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6
Q

what are the different presentations of inherited conditions ?

A

asymptomatic
symptoms related to underlying causes: arrhythmia or HF
hear rhythm related: breathlessness, chest pain, palpitations, presyncope, syncope , sudden death

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7
Q

what are channelopathies?

A

mutation in genes responsible for the coding for channels

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8
Q

what are the characteristics of channelopathies?

A

abnormalities on ecg eg: long qt on ecg
mainly affecting repolarization
watch for young patients with AF

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9
Q

12-year-old presents with lone AF what is most likely diagnosis

A

channelopathy

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10
Q

what are the length of QT in cLQTs

A

males more than 440
in females more than 460

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11
Q

what is the hallmark arrhythmia of cLQTs ?

A

Polymorphic VT

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12
Q

what are signs of LQTS

A

polymorphic VT
lone AF
Heart block
syncope, SCD in children and young adults

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13
Q

what are the triggers of cLQTS and what can cause long qt

A

holding breath
sudden auditory stimuli
sleep

medications like k channel blockers
hypokalemia

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14
Q

management of long QT syndrome

A

beta-blockers
avoid triggers
correct electrolyte balance

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15
Q

what are the signs of brugada syndrome

A

polymorphic vt
vf
af
st elevation and rbbb in V1 to v3

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16
Q

brugada is autosomal recessive t/f

A

false

17
Q

what are the triggers of VF in brugada syndrome

A

rest or sleep
fever
excessive alcohol or large meals

18
Q

management of Brugada syndrome

A

avoidance of excessive alcohol or large meal

treatment of fever

ICD if ventricular arrhythmias

19
Q

what does ICD stands for

A

implantable-cardio-defibrillator

20
Q

patients with brugada are treated with anti-arrhymaic drugs t/f

A

false
as beta-blockers may cause more harm

21
Q

what are two types of cardiomyopathies on the basis of underlying causes

A

primary: no underlying cause
secondary: the known underlying cause

22
Q

what type of murmur can be heard in the case of hypertrophic cardiomyopathy?

A

crescendo-decrescendo-murmur

23
Q

clinical presentation of HOCM

A

asymptomatic
sudden death
heart failure
angina
atrial fibrillation

24
Q

treatment for HOCM

A

beta-blockers
calcium channel blockers : verapamil and diltiazem

25
Q

contraindication for HOCM

A

digoxin : increases foc : inc. obstruction

26
Q

what is dilated cardiac myopathy

A

thin walls + less foc + less blood out

27
Q

murmur heard in dilated cm

A

pan systolic murmur
mitral regurgitation

28
Q

management of dilated CM

A

same as heart failure
AMAN BDS
acei
minralocorticoid
arbs
nisperlyin
beta blockers
diuretics
digoxin
sglt2 inhibitor

29
Q

what happens to cardiomyocytes in case of the ARVCM

A

fibro-fatty replacement of cardiomyocytes in RV

30
Q

treatment of ARVCM

A

ICD

31
Q

what medications to avoid in cLQTS

A

anti-depressants
antibiotics
antihistamines

32
Q
A