Cardiomyopathy

Question 1

define:

Answer 1

Primary disease of the myocardium

There are three types:

• Restrictive
• Dilated
• Hypertrophic

Question 2

Define dilated cardiomyopathy:

Answer 2

Dilated, flabby heart of unknown cause.

Thickening of the inner layer of the heart chamber causes the heart muscles to stretch and weaken.

THERE IS IMPAIRED CONTRACTION (SYSTOLIC FUNCTION)

Question 3

Define hypertrophic cardiomyopathy:

Answer 3

Thickening of the cardiac muscle

IMPAIRED COMPLIANCE (diastolic function)

Question 4

Define restrictive cardiomyopathy:

Answer 4

Normal cardiac cells are replaced by abnormal cells such as scar tissue. This leads the walls of the ventricles to become stiff

IMPAIRED COMPLIANCE (DIASTOLIC FUNCTION)

Question 5

What is the main aetiology of cardiomyopathy?

Answer 5

Idiopathic

Question 6

What is the aetiology/risk factors behind dilates cardiomyopathy?

Answer 6

Hypertension 
Peri/Post partum 
Thyrotoxicosis
Haemochromatosis
Post Viral myocarditis 
Alcohol
Drugs 
Familial
Autoimmune
Congenital (often x linked)

Question 7

What is the aetiology/risk factors behind hypertrophic cardiomyopathy?

Answer 7

50% are due to a genetic cause (autosomal dominant)

Question 8

What is the aetiology/risk factors behind restrictive cardiomyopathy?

Answer 8

Scleroderma
Sarcoidosis
Haemochromatosis 
Amyloidosis
Loffler's eosoniphilic endocarditis 
Endomyocardial fibrosis

Question 9

Epidemiology:

Answer 9

Hypertrophic and dilated prevalence 0.05-0.2%

Restrictive is even rarer

Question 10

Symptoms of dilated cardiomyopathy:

Answer 10

Symptoms of heart failure:

Fatigue 
Thromboembolism 
Dyspnoea 
Arrhythmia
Family history of sudden death

Question 11

Symptoms of hypertrophic cardiomyopathy:

Answer 11

Family history of sudden death 
Angina 
Arrhythmias 
Syncope
Dyspnoea
Palpitations 

USUALLY ASYMP

Question 12

Symptoms of restrictive cardiomyopathy:

Answer 12

Family history of sudden death 
Ankle and abdominal swelling 
Arrhythmias 
Dyspnoea
Fatigue

Question 13

Signs of dilated cardiomyopathy:

Answer 13

Raised JVP 
Third heart sound
Functional mitral and tricuspid regurgitation 
Ascites 
Hepatomegaly 
Peripheral oedema
Displaced apex beat
Tachycardia 
Hypotension 
Pleural effusion 
Jaundice 
AF

Question 14

Signs of hypertrophic cardiomyopathy:

Answer 14

Jerky carotid pulse

Double apex beat

Ejection systolic murmur

Systolic thrill on the left lower sternal edge

Question 15

Signs of restrictive cardiomyopathy:

Answer 15

Raised JVP

Kussmaul’s sign - paradoxical rise in the JVP on inspiration

Palpable apex beat

third heart sound

Ascites

ankle oedema

Hepatomeglay

Question 16

Investigations:

Answer 16

ECG - all will show non-specific ST changes, conduction defects and arrhythmias.

• -> Hypertrophic = LVH, Left axis deviation and Q waves in the lateral and inferior leads
• ->Restrictive = low voltage QRS complex

CXR - may show cardiomegaly or signs of right heart failure such as pulmonary oedema

Echo:
–>Hypertrophic = thickening of the ventricle walls
–>Dilated = dilated ventricles, hypokinesia, reduced ejection fraction, MR, TR and LV thrombus)
–> Restrictive :
•Non-dilated non-hypertrophied ventricles
• Atrial enlargement
• Preserved systolic function
• Diastolic dysfunction
• Granular or sparkling appearance of myocardium in amyloidosis

Cardiac Catheterisation
Endomyocardial Biopsy
Pedigree or Genetic Analysis