inherited Flashcards
what are the 3 basic type of inherited cardiac conditions
cardiomyopathy
channelopathy
arthropathies
examples of cardiomyopathies
hypertrophic cardiomyopathies
dilated cardiomyopathies
arrhythmogenic right ventricular cardiomyopathy
examples of channelopathies
long QT syndrome
brugada syndrome
example of arthropathies
Marfan syndrome
examples of arrhythmogenic inherited cardiac conditions
cardiomyopathies
1) hypertrophic cm
2) dilated cm
3) arrhythmogenic right ventricular cm
channelopathies
1) long QT syndrome
2) brugada syndrome
3) familial AF
what are the different presentations of inherited conditions ?
asymptomatic
symptoms related to underlying causes: arrhythmia or HF
hear rhythm related: breathlessness, chest pain, palpitations, presyncope, syncope , sudden death
what are channelopathies?
mutation in genes responsible for the coding for channels
what are the characteristics of channelopathies?
abnormalities on ecg eg: long qt on ecg
mainly affecting repolarization
watch for young patients with AF
12-year-old presents with lone AF what is most likely diagnosis
channelopathy
what are the length of QT in cLQTs
males more than 440
in females more than 460
what is the hallmark arrhythmia of cLQTs ?
Polymorphic VT
what are signs of LQTS
polymorphic VT
lone AF
Heart block
syncope, SCD in children and young adults
what are the triggers of cLQTS and what can cause long qt
holding breath
sudden auditory stimuli
sleep
medications like k channel blockers
hypokalemia
management of long QT syndrome
beta-blockers
avoid triggers
correct electrolyte balance
what are the signs of brugada syndrome
polymorphic vt
vf
af
st elevation and rbbb in V1 to v3
brugada is autosomal recessive t/f
false
what are the triggers of VF in brugada syndrome
rest or sleep
fever
excessive alcohol or large meals
management of Brugada syndrome
avoidance of excessive alcohol or large meal
treatment of fever
ICD if ventricular arrhythmias
what does ICD stands for
implantable-cardio-defibrillator
patients with brugada are treated with anti-arrhymaic drugs t/f
false
as beta-blockers may cause more harm
what are two types of cardiomyopathies on the basis of underlying causes
primary: no underlying cause
secondary: the known underlying cause
what type of murmur can be heard in the case of hypertrophic cardiomyopathy?
crescendo-decrescendo-murmur
clinical presentation of HOCM
asymptomatic
sudden death
heart failure
angina
atrial fibrillation
treatment for HOCM
beta-blockers
calcium channel blockers : verapamil and diltiazem
