Inherited cardiac conditions Flashcards
What is the reason for sudden death if the heart has a normal structure
Sudden arrhythmic death
What are the categories of inherited cardiac conditions
Cardiomyopathy
Channelopathy
Aortopathy
What is cardiomyopathy and what are examples
Heart muscle abnormality
Hypertrophic cardiomyopathy
ARVC
What is channelopathy and what are examples of it
Heart rhythm abnormality
Long QT syndrome
Bugada syndromeme
What is Aortopathy and what are examples of it
Arterial blood vessel abnormality
Marfan’s syndrome
Ehlos Danlos
How does a heart rhythm condition may present
Palpitations, presyncope, syncope, breathlessness, chest pain, sudden death
How does cardiomyopathy may present
Symptoms of congestive heart failure
Breathlessness, Peripheral oedema, orthopnoea weakness
What is the physiology behind channelopathies
Mutation encodes cardiac ion channels
Affecting repolerization
Abnormality on ECG
Normal cardiac structure and function
Propensity to develop arrhythmia both atrial and ventricular
What are the abnormalities in an ECG of someone that has Congenital Long QT syndrome cLQTS
QT c interval prolongation
>440 ms in males
>460 ms in females
What arrhythmia are associated with Congenital QT syndrome
Hallmark arrhythmia is polymorphic VT(tornadoes de Pointes VT)
Lone AF heart block
What is the presentation of Congenital QT syndrome
Syncope and SCD
What is the management of cLQTS
Beta-blockers - very effective at reducing SCD
Avoidance of triggers
Correction of elecrtolyte abnormalities, maintenance of serum K at upper limit of normal range
What are the risks with Brugada syndrome and what is common ton accure
Risk of polymorphic VT, VF
Atrial fibrillation very common
How does the ECG present of patients with Brugada syndrome
ST elevation
RBBB in V1-V3
Intermittent change over time and may need provocative test with flecainide or ajmaline
Management of burgada syndrome
Avoidance of drugs that may induce brigade changes on ECG
Avoidance of excessive alcohol and large meals
Promps treatment of fever with anti pyretic medications
ICD if ventricular arrhythmia
What drugs should be avoided in Brugada syndrome
Anti-arrhythmic drugs
Psychotropics
Analgesics
Anaesthetics
What causes Hypertrophic cardiomyopathy
Mutation in sarcomeric genes
What is the clinical presentation of HOCM
Sudden death
HF
Angina
Atrial fibrillation
Asymptomatic
What is the management of Cardiomyopathy
as for HF
What is arrhythmogenic right ventricular cardiomyopathy and when is it treated
Fibrous-fatty replacement of cardiomyocytes in RV
Treat if high SCD risk with ICD
What are lifestyle and triggers for SAD in inherited cardiac conditions
Sports and physical activity
Diet, alcohol
Medications
Specific triggers in different types of ICC
What drugs to avoid with cLQTS
Anti depressants
Antibiotics
Antibistamines
What are the ICC issues in young people
Psycho-social impact
Lifestyle
Multidisciplinary approach
