Cardiomyopathy

Question 1

Define the term cardiomyopathy

Answer 1

Refers to a disease of the heart muscle that cannot otherwise be explained by common cardiovascular diseases or congenital heart disease e.g. coronary artery disease, hypertension, valvular disease and congenital heart disease

Question 2

How many people are affected by cardiomyopathies in the UK

Answer 2

About 1 in 500 of the UK population

Question 3

What are the three main types of cardiomyopathies

Answer 3

1. Hypertrophic cardiomyopathy (HCM)
2. Dilated cardiomyopathy (DCM)
3. Arrhythmogenic cardiomyopathy (AMC)

Question 4

Define hypertrophic cardiomyopathy (HCM)

Answer 4

Genetic condition characterised by the disorganised arrangement of the myocardium causing thickening of the myocardium

Question 5

What are the two changes characterised by hypertrophic cardiomyopathy (HCM)

Answer 5

​Myocardial cells lies in disorganised, jumbled layers

Scarring of myocardium making the heart muscle stiff

Question 6

When does hypertrophic cardiomyopathy usually develop

Answer 6

Usually develops after puberty

Question 7

What are the 4 types of thickening pattern seen in hypertrophic cardiomyopathy (HCM)

Answer 7

1. Asymmetrical septal hypertrophy without obstruction
2. Asymmetrical septal hypertrophy with obstruction
3. Symmetrical hypertrophy (also known as concentric hypertrophy)
4. Apical hypertrophic cardiomyopathy

Question 8

Asymmetrical septal hypertrophy without obstruction is a pattern of hypertrophic cardiomyopathy.

What is it characterised by

Answer 8

Characterised by myocardial thickening of the septum

Does not restrict the normal flow of blood out of your heart through the left ventricular outflow tract

Question 9

Asymmetrical septal hypertrophy with obstruction is a pattern of hypertrophic cardiomyopathy.

What is it characterised by

Answer 9

Characterised by myocardial thickening of the septum and this thickening obstructs the flow of blood through the left ventricular outflow tract when the heart contracts

When the heart contracts, the mitral valve touches the septum causing mitral regurgitation

Question 10

Symmetrical hypertrophy is a pattern type of hypertrophic cardiomyopathy.

What is it characterised by

Answer 10

Hypertrophy which is evenly distributed through the left ventricle

Question 11

Apical hypertrophy is a pattern type of hypertrophic cardiomyopathy.

What is it characterised by

Answer 11

Hypertrophy is predominantly at the apex

Question 12

Which valve is between the right atrium and the right ventricle

Answer 12

Tricuspid

Question 13

Which valve is between the right ventricle and the lungs

Answer 13

Pulmonary valve

Question 14

Which valve is between the left artium and the left ventricle

Answer 14

Mitral valve

Question 15

Which valve is between the left ventricle and the peripheries

Answer 15

Aortic valve

Question 16

How is hypertrophic cardiomyopathy diagnosed

Answer 16

Electrocardiogram (ECG) and echocardiogram (ECHO)

Question 17

Name some of the clinical features for hypertrophic cardiomyopathy

Answer 17

Asymptomatic (major of cases)

Arrhythmia

Dyspnoea i.e. SoB – typically on exertion

Chest pain or angina – the coronary arteries are usually normal but the microscopic coronary arteries within the myocardium becomes narrow

Palpitations

Presyncope

Question 18

What is the definitive treatment for hypertrophic cardiomyopathies

Answer 18

There is none

Treatment aims to improve symptoms and prevent complications

Question 19

Name two complications associated with hypertrophic cardiomyopathy

Answer 19

Arrhythmias – most common AF and ventricular tachycardia

Heart block

Question 20

Define dilated cardiomyopathies (DCM)

Answer 20

Dilated cardiomyopathy refers to ventricular chamber enlargement and impaired contraction of one or both ventricles

Dilatation leads to progressive systolic dysfunction that manifests as poor myocardial contraction and features of heart failure

Question 21

What condition does dilated cardiomyopathy most commonly cause

Answer 21

Commonly cause heart failure

Question 22

What age group is dilated cardiomyopathy most common seen in

Answer 22

Most common between 20-60 years old

Question 23

Name 4 potential causes of dilated cardiomyopathy

Answer 23

• Idiopathic
• Infection e.g. parvovirus B19, coxsackievirus
• Drugs
• Pregnancy

Question 24

Name some of the clinical features of dilated cardiomyopathy

Answer 24

Characterised by the development of heart failure

Dyspnoea e.g. SoB

Paroxsymal nocturnal dyspnoea: severe, sudden shortness of breath, usually at nighttime

Palpitations: due to the development of arrhythmias

Question 25

How is dilated cardiomyopathy diagnosed

Answer 25

Visualisation of the heart by echocardiography (ECHO) causing evidence of dilation

Question 26

How is dilated cardiomyopathy managed

Answer 26

Use of both: Prognostic medications e.g. angiotensin receptor antagonists, beta-blockers And Medications to improve symptoms e.g. diuretics Other option is cardiac transplantation - more likely to be considered due to the young age of onset

Question 27

Define arrhythmogenic cardiomyopathy (AMC)

Answer 27

Defined as a group of disorders characterised by arrhythmia(s) alongside myocardial structural abnormalities

Question 28

Arrhythmogenic cardiomyopathy used to be called arrhythmogenic right ventricular cardiomyopathy (ARVC) Why did it change?

Answer 28

The condition can affect either the left and/or right ventricles

Question 29

Arrhythmogenic cardiomyopathy is an inherited condition caused by a mutation of what gene

Answer 29

desmosomal gene

Question 30

What happens to the heart in arrhythmogenic cardiomyopathy

Answer 30

The myocardium is replaced with fibrous and/or fatty tissue making the heart walls weak causing abnormal function and predisposition to arrhythmias

Question 31

Name some of the clinical features of arrhythogenic cardiomyopathy

Answer 31

* Asymptomatic * Palpitations * Presyncope * Chest pain * Breathlessness * Features of heart failure

Question 32

How is arrhythmogenic cardiomyopathy diagnosed

Answer 32

* May be challenging * Combination of typical ECG findings, echocardiography with/without cardiac MRI

Question 33

How is the aim of arrhythmogenic cardiomyopathy management

Answer 33

The aim is to prevent sudden cardiac death Usually requires anti-arrhythmias (e.g. beta-blockers) with or without the use of an implantable cardiac defibrillator

Question 34

What is a pacemaker

Answer 34

Device usually fitted clavicle to only help your heart’s rhythm when it doesn’t beat like it should.

Question 35

What is an Implantable Cardioverter Defibrillator (ICD)

Answer 35

Device fitted under chest’s skin which continuously monitor your heart’s rhythm and treat dangerous, abnormal rhythms

Question 36

What is cardioversion

Answer 36

Procedure that sets the heart rhythm back to a normal pattern by sending a strong, electrical impulse to your heart muscle

Question 37

What is catheter ablation

Answer 37

Procedure that sets the heart rhythm back to a normal pattern by blocking your heart’s electrical paths that aren’t working properly

Question 38

Name three potential complications of arrhythogenic cardiomyopathy

Answer 38

Heart block – block in the electrical activity of the heart Heart failure Sudden cardiac death

Question 39

The heart is made up of three layers. Name these layers

Answer 39

Endocardium Myocardium Pericardium

Question 40

The pericardium is a thin, double layer that forms a protective sac around the outside of the heart. It contains a small amount of fluid What is that fluid called and why is it important

Answer 40

Pericardial fluid Acts as a lubricant when the heart is contracting.