Cardiomyopathy Flashcards

1
Q

Define the term cardiomyopathy

A

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

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2
Q

How many people are affected by cardiomyopathies in the UK

A

About 1 in 500 of the UK population

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3
Q

What are the three main types of cardiomyopathies

A
  1. Hypertrophic cardiomyopathy (HCM)
  2. Dilated cardiomyopathy (DCM)
  3. Arrhythmogenic cardiomyopathy (AMC)
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4
Q

Define hypertrophic cardiomyopathy (HCM)

A

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

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5
Q

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

A

​Myocardial cells lies in disorganised, jumbled layers

Scarring of myocardium making the heart muscle stiff

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6
Q

When does hypertrophic cardiomyopathy usually develop

A

Usually develops after puberty

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7
Q

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

A
  1. Asymmetrical septal hypertrophy without obstruction
  2. Asymmetrical septal hypertrophy with obstruction
  3. Symmetrical hypertrophy (also known as concentric hypertrophy)
  4. Apical hypertrophic cardiomyopathy
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8
Q

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

What is it characterised by

A

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

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9
Q

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

What is it characterised by

A

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

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10
Q

Symmetrical hypertrophy is a pattern type of hypertrophic cardiomyopathy.

What is it characterised by

A

Hypertrophy which is evenly distributed through the left ventricle

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11
Q

Apical hypertrophy is a pattern type of hypertrophic cardiomyopathy.

What is it characterised by

A

Hypertrophy is predominantly at the apex

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12
Q

Which valve is between the right atrium and the right ventricle

A

Tricuspid

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13
Q

Which valve is between the right ventricle and the lungs

A

Pulmonary valve

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14
Q

Which valve is between the left artium and the left ventricle

A

Mitral valve

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15
Q

Which valve is between the left ventricle and the peripheries

A

Aortic valve

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16
Q

How is hypertrophic cardiomyopathy diagnosed

A

Electrocardiogram (ECG) and echocardiogram (ECHO)

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17
Q

Name some of the clinical features for hypertrophic cardiomyopathy

A

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

18
Q

What is the definitive treatment for hypertrophic cardiomyopathies

A

There is none

Treatment aims to improve symptoms and prevent complications

19
Q

Name two complications associated with hypertrophic cardiomyopathy

A

Arrhythmias – most common AF and ventricular tachycardia

Heart block

20
Q

Define dilated cardiomyopathies (DCM)

A

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

21
Q

What condition does dilated cardiomyopathy most commonly cause

A

Commonly cause heart failure

22
Q

What age group is dilated cardiomyopathy most common seen in

A

Most common between 20-60 years old

23
Q

Name 4 potential causes of dilated cardiomyopathy

A
  • Idiopathic
  • Infection e.g. parvovirus B19, coxsackievirus
  • Drugs
  • Pregnancy
24
Q

Name some of the clinical features of dilated cardiomyopathy

A

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

25
Q

How is dilated cardiomyopathy diagnosed

A

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

26
Q

How is dilated cardiomyopathy managed

A

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

27
Q

Define arrhythmogenic cardiomyopathy (AMC)

A

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

28
Q

Arrhythmogenic cardiomyopathy used to be called arrhythmogenic right ventricular cardiomyopathy (ARVC)

Why did it change?

A

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

29
Q

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

A

desmosomal gene

30
Q

What happens to the heart in arrhythmogenic cardiomyopathy

A

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

31
Q

Name some of the clinical features of arrhythogenic cardiomyopathy

A
  • Asymptomatic
  • Palpitations
  • Presyncope
  • Chest pain
  • Breathlessness
  • Features of heart failure
32
Q

How is arrhythmogenic cardiomyopathy diagnosed

A
  • May be challenging
  • Combination of typical ECG findings, echocardiography with/without cardiac MRI
33
Q

How is the aim of arrhythmogenic cardiomyopathy management

A

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

34
Q

What is a pacemaker

A

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

35
Q

What is an Implantable Cardioverter Defibrillator (ICD)

A

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

36
Q

What is cardioversion

A

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

37
Q

What is catheter ablation

A

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

38
Q

Name three potential complications of arrhythogenic cardiomyopathy

A

Heart block – block in the electrical activity of the heart

Heart failure

Sudden cardiac death

39
Q

The heart is made up of three layers.

Name these layers

A

Endocardium

Myocardium

Pericardium

40
Q

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

A

Pericardial fluid

Acts as a lubricant when the heart is contracting.