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Y3 CLASP > Sudden Death Conditions > Flashcards

Sudden Death Conditions Flashcards

1
Q

Congenital Long QT Syndrome ECG

A

Polymorphic VT (torsades de pointes)

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2
Q

What is Congenital Long QT Syndrome triggered by?

A

Adrenergic stimulation so sudden awakening by alarm clock

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3
Q

Congenital Long QT Syndrome’s Autosomal Dominant form with isolated LQT is called?

A

Romano Ward Syndrome

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4
Q

Congenital Long QT Syndrome’s Autosomal Dominant form with extra cardiac features are called?

A

Ander-Tawil Syndrome, Timothy Syndrome

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5
Q

Congenital Long QT Syndrome’s Autosomal Recessive form is called?

A

Jervell and Lange-Nielsen Syndrome

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6
Q

Congenital Long QT Syndrome’s Autosomal Recessive form is assoc. with?

A

Deafness

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7
Q

How to diagnose Congenital Long QT Syndrome

A
  • QT interval longer than 480ms
  • Confirmed pathogenic LQTS mutation
  • QT interval longer than 460ms + unexplained syncopal episode
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8
Q

What is the scoring system called for diagnosis of LQTs

A

Schwartz

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9
Q

What are the risks of Sudden Cardiac Death in Long QT syndrome

A

Pre adolescence male, adult female, Longer QT intervals and QT prolonging drugs

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10
Q

What are some QT prolonging drugs?

A

Erythromycin / Clarithromycin

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11
Q

Where is the mutation in Short QT Syndrome?

A

Cardiac K+ Channels

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12
Q

How to diagnose Short QT Syndrome ?

A

QT <300ms at heart rate <80bpm

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13
Q

What is Short QT Syndrome assoc. with

A

AF

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14
Q

Short QT Syndrome typical presentation

A

Young children, very malignant

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15
Q

Brugada syndrome ECG

A
  • Polymorphic VT, VF
  • AF is common
  • ST elevation and Right Bundle Branch Block RBBB in V1-V3
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16
Q

Brugada syndrome’s diagnostic ECG can only be done when? and why?

A

Patient undergoes provocative testingw tih flecanide or ajmaline to block the cardiac sodium channel

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17
Q

Some of the genes assoc. with Brugada syndrome?

A

SCN5A - Sodium channel

CACN1Ac - Calcium channel

18
Q

Autosomal dominant form of Brugada syndrome is assoc with male or females?

A

Male 8 fold

19
Q

VF triggers in Brugada syndrome

A

Rest or sleep, fever, excess alcohol, large meals, phenotype, FHx

20
Q

What are the drugs to avoid in Brugada syndrome

A

Antiarrythmics, ST elevating drugs, excessive alcohol, food consumption, ICD

21
Q

Catecholaminergic Polymorphic Ventricular Tachycardia CPVT is?

A

Adrenergic induced bidirectional and polymorphic VT, SVTs

22
Q

Triggers of Catecholaminergic Polymorphic Ventricular Tachycardia CPVT

A

Emotional stress, physical activity

23
Q

Catecholaminergic Polymorphic Ventricular Tachycardia CPVT Investigations will show?

A

Normal ECG and ECHO

24
Q

Catecholaminergic Polymorphic Ventricular Tachycardia CPVT autosomal dominant mutation is?

A

RyR2

25
Q

Catecholaminergic Polymorphic Ventricular Tachycardia CPVT autosomal recessive mutation is?

A

CASQ2

26
Q

What to avoid in Catecholaminergic Polymorphic Ventricular Tachycardia CPVT?

A

Competitive sports, strenuous exercise and stressful environments

27
Q

Treatment for Catecholaminergic Polymorphic Ventricular Tachycardia CPVT

A

Beta blockers, ICD implantation

28
Q

Wolff Parkinson White Syndrome ECG

A

Short PR interval, delta wave, ventricular preexcitation

Delta wave: the upstroke of QRS complex is curved instead of a straight right angle, caused by ventricular preexcitation

29
Q

Wolff Parkinson White Syndrome Presentation

A

Presents with SVT, ablated and then solved

30
Q

Hypertrophic Cardiomyopathy HOCM mutations are in what genes?

A

Sarcomeric genes MYBPC3

31
Q

What is the ECG for Hypertrophic Cardiomyopathy HOCM?

A

Left ventricular hypertrophy voltage criteria

32
Q

Clinical presentation of Hypertrophic Cardiomyopathy HOCM

A

Sudden death, HF, End stage HF, AF

33
Q

Treatment for Hypertrophic Cardiomyopathy HOCM

A

Implantable cardiac device ICD

34
Q

Dilated Cardiomyopathy is seen most commonly in?

A

Males

35
Q

What genes are affected in Dilated Cardiomyopathy?

A

Sarcomere and desmosomal genes

36
Q

What genes are affected in Dilated Cardiomyopathy if its x-linked?

A

Dystrophin

37
Q

What happens to the heart in Arrhythmogenic Right Ventricular Cardiomyopathy ARVC?

A

Fibrofatty replacement of cardiomyocytes with LV involvement in >50% of cases

38
Q

Arrhythmogenic Right Ventricular Cardiomyopathy ARVC Autosomal Dominant form affects what genes?

A

Desmosomal proteins genes

39
Q

Arrhythmogenic Right Ventricular Cardiomyopathy ARVC Autosomal recessive form affects what genes?

A

Nondesmosomal genes

40
Q

What increases the risk of Sudden Cardiac Death SCD in Arrhythmogenic Right Ventricular Cardiomyopathy ARVC?

A

FHx of SCD, severity of RV and LV function, frequent non sustained VT

41
Q

Treatment for Arrhythmogenic Right Ventricular Cardiomyopathy ARVC ?

A

Beta blockers, ICD, catheter ablation

Y3 CLASP (2 decks)

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