Sudden Death Conditions Flashcards
Congenital Long QT Syndrome ECG
Polymorphic VT (torsades de pointes)
What is Congenital Long QT Syndrome triggered by?
Adrenergic stimulation so sudden awakening by alarm clock
Congenital Long QT Syndrome’s Autosomal Dominant form with isolated LQT is called?
Romano Ward Syndrome
Congenital Long QT Syndrome’s Autosomal Dominant form with extra cardiac features are called?
Ander-Tawil Syndrome, Timothy Syndrome
Congenital Long QT Syndrome’s Autosomal Recessive form is called?
Jervell and Lange-Nielsen Syndrome
Congenital Long QT Syndrome’s Autosomal Recessive form is assoc. with?
Deafness
How to diagnose Congenital Long QT Syndrome
- QT interval longer than 480ms
- Confirmed pathogenic LQTS mutation
- QT interval longer than 460ms + unexplained syncopal episode
What is the scoring system called for diagnosis of LQTs
Schwartz
What are the risks of Sudden Cardiac Death in Long QT syndrome
Pre adolescence male, adult female, Longer QT intervals and QT prolonging drugs
What are some QT prolonging drugs?
Erythromycin / Clarithromycin
Where is the mutation in Short QT Syndrome?
Cardiac K+ Channels
How to diagnose Short QT Syndrome ?
QT <300ms at heart rate <80bpm
What is Short QT Syndrome assoc. with
AF
Short QT Syndrome typical presentation
Young children, very malignant
Brugada syndrome ECG
- Polymorphic VT, VF
- AF is common
- ST elevation and Right Bundle Branch Block RBBB in V1-V3
Brugada syndrome’s diagnostic ECG can only be done when? and why?
Patient undergoes provocative testingw tih flecanide or ajmaline to block the cardiac sodium channel
Some of the genes assoc. with Brugada syndrome?
SCN5A - Sodium channel
CACN1Ac - Calcium channel
Autosomal dominant form of Brugada syndrome is assoc with male or females?
Male 8 fold
VF triggers in Brugada syndrome
Rest or sleep, fever, excess alcohol, large meals, phenotype, FHx
What are the drugs to avoid in Brugada syndrome
Antiarrythmics, ST elevating drugs, excessive alcohol, food consumption, ICD
Catecholaminergic Polymorphic Ventricular Tachycardia CPVT is?
Adrenergic induced bidirectional and polymorphic VT, SVTs
Triggers of Catecholaminergic Polymorphic Ventricular Tachycardia CPVT
Emotional stress, physical activity
Catecholaminergic Polymorphic Ventricular Tachycardia CPVT Investigations will show?
Normal ECG and ECHO
Catecholaminergic Polymorphic Ventricular Tachycardia CPVT autosomal dominant mutation is?
RyR2
Catecholaminergic Polymorphic Ventricular Tachycardia CPVT autosomal recessive mutation is?
CASQ2
What to avoid in Catecholaminergic Polymorphic Ventricular Tachycardia CPVT?
Competitive sports, strenuous exercise and stressful environments
Treatment for Catecholaminergic Polymorphic Ventricular Tachycardia CPVT
Beta blockers, ICD implantation
Wolff Parkinson White Syndrome ECG
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
Wolff Parkinson White Syndrome Presentation
Presents with SVT, ablated and then solved
Hypertrophic Cardiomyopathy HOCM mutations are in what genes?
Sarcomeric genes MYBPC3
What is the ECG for Hypertrophic Cardiomyopathy HOCM?
Left ventricular hypertrophy voltage criteria
Clinical presentation of Hypertrophic Cardiomyopathy HOCM
Sudden death, HF, End stage HF, AF
Treatment for Hypertrophic Cardiomyopathy HOCM
Implantable cardiac device ICD
Dilated Cardiomyopathy is seen most commonly in?
Males
What genes are affected in Dilated Cardiomyopathy?
Sarcomere and desmosomal genes
What genes are affected in Dilated Cardiomyopathy if its x-linked?
Dystrophin
What happens to the heart in Arrhythmogenic Right Ventricular Cardiomyopathy ARVC?
Fibrofatty replacement of cardiomyocytes with LV involvement in >50% of cases
Arrhythmogenic Right Ventricular Cardiomyopathy ARVC Autosomal Dominant form affects what genes?
Desmosomal proteins genes
Arrhythmogenic Right Ventricular Cardiomyopathy ARVC Autosomal recessive form affects what genes?
Nondesmosomal genes
What increases the risk of Sudden Cardiac Death SCD in Arrhythmogenic Right Ventricular Cardiomyopathy ARVC?
FHx of SCD, severity of RV and LV function, frequent non sustained VT
Treatment for Arrhythmogenic Right Ventricular Cardiomyopathy ARVC ?
Beta blockers, ICD, catheter ablation
