Cardiomyopathy

Question 1

What is cardiomyopathy classified into

Answer 1

Hypertrophic
Dilated
Restrictive
A lot of overlap so new way to classify

Question 2

What are new classification

Answer 2

Genetic
Secondary cause
Acquired
Mixed

Question 3

What are genetic causes

Answer 3

Autosomal dominant

Mutation in B-myosin / protein C

Question 4

What do genetic mutation cause

Answer 4

HCM

Arrythmogenic R ventricular cardiomyopathy - replaced fatty tissue

Question 5

What does HCM cause

Answer 5

Leading cause of cardiac death in the young

Question 6

How do you Dx

Answer 6

Genetic test
ECG - arrythmia
ECHO - MR / hypertrophy
Cardiac MRI

Question 7

What is the treatment

Answer 7

Defibrillator

Question 8

What are secondary causes

Answer 8

Drugs

• Alcohol / thiamine deficiency
• Doxorubin (chemo)

Endocrine

• DM
• Thyrotoxicosis
• Acromegaly

Infection
- Coxsackie

Infiltrative

• Amyloidosis
• Haemochromatosis
• Sarcoidosis
• SLE

Other

• Myotonic dystrophy
• Post RT
• Endocarditis

Question 9

What are acquired causes

Answer 9

Post partum

Tokotsubo

Question 10

Who is at greater risk of post partum

Answer 10

Older women
Greater parity
Multiple

Question 11

What is tokotsubo

Answer 11

Stress induced transient ballooning of myocardium

Follows severe stress such as family death / break up

Question 12

How does tokotsubo present

Answer 12

ACS 
Chest pain
HF features
ST elevation
Normal angiogram
Rx = supportive

Question 13

What is HCM

Answer 13

Inappropriate ventricular hypertrophy
Causes impaired relaxation
Systolic usually preserved
Eventually reduced LVOT and reduced CO

Question 14

What is associated with HCM

Answer 14

WPW

Freidreich’s ataxia

Question 15

What are the symptoms of HCM

Answer 15

Asymptomatic
Sudden cardiac death
Chest pain
Angina
SOB
Palpitations
Syncope
Fatigue
Cardiac failure

Question 16

What causes syncope

Answer 16

Arrhythmia or LVOT / AS

Question 17

What are signs of HCM

Answer 17

Jerky pulse
AF
Double impulse over apex
Ejection systolic murmur increases valsalva 
Thrill 
Raised JVP

Question 18

What are RF for HCM

Answer 18

FH sudden cardiac death

Maternal DM

Question 19

How do you Dx HCM

Answer 19

ECG
ECHO
MRI
Angiogram
Genetic test

Question 20

What are ECHO findings

Answer 20

MR SAM ASH
Mitral regurgitation as mitral valve doesn’t close properly
Systolic anterior motion of MV
Asymmetric hypertrophy

Question 21

What are ECG findings

Answer 21

LVH
ST and T wave abnormality
Q waves
AF
WPW
VT
Ectopics

VT = cause of death

Question 22

How do you treat HCM

Answer 22

Avoid heavy exercise
Anti-coagulation if AF
Surgical resection / ablation
ABCDE

Question 23

What is ABCDE in HCM

Answer 23

Anti-arrhythmia - amiadarone + anti-coagulant if AF
BB / verapamil for Sx
Cardiac defibrillator - ICD
Dual chamber pacemaker
Endocarditis - watch

Question 24

What should you avoid

Answer 24

Digoxin
INotrope
Nitrates
As increase contractility of heart

Question 25

What are complications of HCM

Answer 25

``` Sudden death LVOT Arrhythmia HF AF MR ```

Question 26

What is most common cardiomyopathy

Answer 26

Dilated

Question 27

What is dilated

Answer 27

Enlarged heart Often all chambers - LV>RV Ventricular function impaired, systolic dysfunction resulting in reduced CO and increased EDV Leads to HF

Question 28

What causes dilated

Answer 28

``` Mixed Genetics Idiopathic Infection - Coxsackie / HIV / viral Toxins - alcohol / Doxcirubin (chemo) / cocaine High BP Inflammation - SLE Infiltration - amyloid / sarcoid / hamochromatosis Post partum Endocrine - Hyperthyroid ```

Question 29

What gene

Answer 29

DCN SCN5A | DMD

Question 30

How does dilated cardiomyopathy present

Answer 30

``` Classic HF signs Progressive SOB Fatigue Pulmonary oedema Cough Palpitations Arrhythmia - AF / VT RVF Emboli Mitral and tricuspid regurgitation - pan systolic ```

Question 31

What are the signs of dilated cardiomyopathy

Answer 31

``` Systolic murmur Tachycardia Hypotension Poor perfusion Thread pulse Elevated JVP Displaced apex S3+S4 Pleural effusion Ascites Hepatomegaly as RHF develops ```

Question 32

What is important in the history

Answer 32

``` Travel Systemic illness High BP / vascular Thyroid Neuromuscular ```

Question 33

What investigations do you do

Answer 33

``` Bloods ECG CXR ECHO Angiogram BNP Biopsy ```

Question 34

What does ECG show

Answer 34

LBBB | Tachy

Question 35

What does ECHO show

Answer 35

Low ejection | Global dilatation

Question 36

What does CXR show

Answer 36

Cardiomegaly | Oedema

Question 37

How do you treat

Answer 37

``` Treat cause if known Remove exacerbating drugs e.g. NSAIDs Reduce fluid and salt intake ACEI, diuretic, BB, spironolactone Anti-coagulate if indicated Treat HF + arrhythia VTE prophylaxis SCD assessment ```

Question 38

What are the complications of dilated

Answer 38

VTE Heart failure Renal failure Sudden cardiac death

Question 39

What is restrictive cardiomyopathy

Answer 39

Relaxation affected Unable to fill ventricle Systolic may or not be impaired

Question 40

What causes restrictive

Answer 40

``` Form of HCM Haemochrmatosis Amyloid - biopsy Sarcoid - ACE Scleroderma - Ab Fibrosis Radiation Drugs Malignancy DM Storage disease - low plasma galactodise ```

Question 41

What are the symptoms of restrictive

Answer 41

``` Present like constrictive pericarditis Back pressure leads to RVF Oedema Increased JVP with X and Y descent Hepatomegaly Ascites ```

Question 42

How do you treat restrictive

Answer 42

``` Treat cause BB Anti-coagulant Transplant Specific treatment for iron / storage SCD assessment ```

Question 43

What has limited use in restrictive

Answer 43

ACEI/ diuretic | Low filling pressure so worsen