Inherited cardiac conditions

Question 1

What is the reason for sudden death if the heart has a normal structure

Answer 1

Sudden arrhythmic death

Question 2

What are the categories of inherited cardiac conditions

Answer 2

Cardiomyopathy
Channelopathy
Aortopathy

Question 3

What is cardiomyopathy and what are examples

Answer 3

Heart muscle abnormality
Hypertrophic cardiomyopathy
ARVC

Question 4

What is channelopathy and what are examples of it

Answer 4

Heart rhythm abnormality
Long QT syndrome
Bugada syndromeme

Question 5

What is Aortopathy and what are examples of it

Answer 5

Arterial blood vessel abnormality
Marfan’s syndrome
Ehlos Danlos

Question 6

How does a heart rhythm condition may present

Answer 6

Palpitations, presyncope, syncope, breathlessness, chest pain, sudden death

Question 7

How does cardiomyopathy may present

Answer 7

Symptoms of congestive heart failure
Breathlessness, Peripheral oedema, orthopnoea weakness

Question 8

What is the physiology behind channelopathies

Answer 8

Mutation encodes cardiac ion channels
Affecting repolerization
Abnormality on ECG
Normal cardiac structure and function
Propensity to develop arrhythmia both atrial and ventricular

Question 9

What are the abnormalities in an ECG of someone that has Congenital Long QT syndrome cLQTS

Answer 9

QT c interval prolongation
>440 ms in males
>460 ms in females

Question 10

What arrhythmia are associated with Congenital QT syndrome

Answer 10

Hallmark arrhythmia is polymorphic VT(tornadoes de Pointes VT)
Lone AF heart block

Question 11

What is the presentation of Congenital QT syndrome

Answer 11

Syncope and SCD

Question 12

What is the management of cLQTS

Answer 12

Beta-blockers - very effective at reducing SCD
Avoidance of triggers
Correction of elecrtolyte abnormalities, maintenance of serum K at upper limit of normal range

Question 13

What are the risks with Brugada syndrome and what is common ton accure

Answer 13

Risk of polymorphic VT, VF
Atrial fibrillation very common

Question 14

How does the ECG present of patients with Brugada syndrome

Answer 14

ST elevation
RBBB in V1-V3
Intermittent change over time and may need provocative test with flecainide or ajmaline

Question 15

Management of burgada syndrome

Answer 15

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

Question 16

What drugs should be avoided in Brugada syndrome

Answer 16

Anti-arrhythmic drugs
Psychotropics
Analgesics
Anaesthetics

Question 17

What causes Hypertrophic cardiomyopathy

Answer 17

Mutation in sarcomeric genes

Question 18

What is the clinical presentation of HOCM

Answer 18

Sudden death
HF
Angina
Atrial fibrillation
Asymptomatic

Question 19

What is the management of Cardiomyopathy

Answer 19

as for HF

Question 20

What is arrhythmogenic right ventricular cardiomyopathy and when is it treated

Answer 20

Fibrous-fatty replacement of cardiomyocytes in RV
Treat if high SCD risk with ICD

Question 21

What are lifestyle and triggers for SAD in inherited cardiac conditions

Answer 21

Sports and physical activity
Diet, alcohol
Medications
Specific triggers in different types of ICC

Question 22

What drugs to avoid with cLQTS

Answer 22

Anti depressants
Antibiotics
Antibistamines

Question 23

What are the ICC issues in young people

Answer 23

Psycho-social impact
Lifestyle
Multidisciplinary approach