Cardiomyopathies

Question 1

What are the three types of cariomyopathies?

Answer 1

Restrictive Dilated Hypertrophic

Question 2

Cardiomyopathies can develop to compensate for other underlying diseases such as…

(secondary cardiomyopathy)

Answer 2

Hypertension / Valve diseases

Question 3

Most common type of cadriomyopathy?

Answer 3

Dilated

Question 4

Dilated cardiomyopathy

Answer 4

• All 4 chambers of the heart dilate.
• Heart walls become thin and lose contractility.

Question 5

Hypertrophic cardiomyopathy

Answer 5

• Walls become thick, heavy and hypercontractile.

Question 6

Restrictive cardiomyopathy

Answer 6

• Heart muscle is restricted becoming stiff and less compliant.
• This prevents the heart from filling properly.
• Muscles and size of the ventricles are only slightly enlarged.

Question 7

Restrictive cardiomyopathy can be idopathic or secondary to what?

• think about what would cause restriction…

Answer 7

A disorder that results in the deposition of iron or amyloid in the tissue, or by fibrosis caused by immune cells / radiation.

Amyloidosis

Sarcoidosis

Hemochromatosis

Question 8

What symptoms can cardiomyopathy present with and why?

Answer 8

Cardiomyopathies prevent the heart from doing it’s job properly so signs and symptoms are similar to that of heart failure:

Fatigue

Dyspnea

Swelling of the feet

Acute symptoms: Presyncope / syncope (represents increased risk of MI).

Arrhythmias as cardiomypothies affect the heart muscle and also pace maker cells leads to arrhythmias.

Question 9

What is the main cause of mitral stenosis?

Answer 9

RHEUMATIC FEVER! ​RHEUMATIC FEVER! RHEUMATIC FEVER!

Question 10

What arrhythmias can result from cardiomyopathies?

Answer 10

Atrial fibrillation, ventricular ectopic beats, ventricular tachycardia or fibrillation, and atrioventricular block.

Question 11

Hypertrophic cardiomyopathy presents with what murmur?

Answer 11

Cresecendo-decrescendo murmur between S1 and S2.

Question 12

ECG changes in cardiomyopathies:

Answer 12

Question 13

How is cardiomyopathy type identified?

Answer 13

Question 14

How does hypertrophic cardiomyopathy present on echocardiogram?

Answer 14

Question 15

How does restrictive cm present on an echo?

Answer 15

Shape tends to be normal, as restrictive cardiomyopathy is characterised by non-dilated ventricles with impaired ventricular filling.

Question 16

MRI is particuarly useful for diagnosising what?

X-ray in diagnosis:

Answer 16

Cardiac MRI is useful for diagnosing restrictive cardiomyopathy.

Question 17

What is the relevance of the genetic background to these condition?

Answer 17

All the cardiomyopathies run in families - important to screen the immediate family with ECG and echocardiography.

This is important because many cases of sudden death are due to undiagnosed cardiomyopathies.

Question 18

Hypertrophic cardiomyopathy is the most common cause of death in whom?

Answer 18

Young athletes

Question 19

Cardiomyopathy Treatment:

Answer 19

No treatment except heart transplant.

Goal of treatment is symptom relief and ensuring the heart continues to function - avoiding MI and development of congestive HF.

Pacemakers may also be needed to treat arrhythmias.

Question 20

Summary of Inherited cardiac condtions:

Answer 20

Question 21

Cardiomyopathy MRI

Answer 21

Question 22

What are the functions of the pericardium?

Answer 22

• Protection
• Prevents over expansion with increasing preload
• Minor shock absorber
• Immunological / hormonal

Question 23

Dilated cardiomyopathy ejection fraction is…

Answer 23

<45%

Question 24

Arrhythmogenic (right) cardiomyopathy inheritence?

Answer 24

Autosomal dominant inheritance with mutations in desmosome proteins found in most patients.

Question 25

What is the main cause of amyloidosis?

Answer 25

Amyloidosis is caused by changes in proteins that make them insoluble, leading them to deposit in organs and tissues. These amyloid proteins accumulate mainly in the tissue space between cells. Changes in proteins that make them amyloid proteins occur because of gene mutations in DNA within cells.

Question 26

Long QT syndrome

Answer 26

Long QT syndrome (LQTS) is a condition in which repolarization of the heart after a heartbeat is affected. It results in an increased risk of an irregular heartbeat which can result in fainting, drowning, seizures, or sudden death.

Question 27

Brugada syndrome

Answer 27

Brugada syndrome is a condition that causes a disruption of the heart's normal rhythm. Specifically, this disorder can lead to irregular heartbeats in the heart's lower chambers (ventricles), which is an abnormality called ventricular arrhythmia.

Question 28

Acute pericarditis

Answer 28

Acute inflammation of the pericardium of the heart.

Question 29

What is the most common cause of acute pericarditis?

Answer 29

Usually viral cause.

Question 30

How does acute pericarditis present?

Answer 30

Pleuritic chest pain Other symptoms of viral illness. If in tamponade - breathlessness / pre-syncope/syncope. Pericardial rub on auscultation.

Question 31

Pericardial effusion is

Answer 31

Substance in the pericadial space - usually fluid, pus or blood.

Question 32

Pericardial effusion imaging:

Answer 32

Question 33

Cardiac tamponade presents with what triad?

Answer 33

**Beck's Triad:** Hypotension Jugular distension Diminished heart sounds

Question 34

Cardiac tamponade

Answer 34

Cardiac tamponade is when blood or fluids fill the pericardial space. This places extreme pressure on your heart. The pressure prevents the heart's ventricles from expanding fully and keeps your heart from functioning properly.

Question 35

Arrhythmogenic cm affects where?

Answer 35

Right ventricle

Question 36

What puts a patient with cardiomyopathy at risk of sudden death?

Answer 36

Syncope

Question 37

Cardiomyopathies summary: inheritance is 99% what?

Answer 37

Autosomal dominant