Cardiomyopathy

Question 1

Define cardiomyopathy

Answer 1

Primary disease of the myocardium. Cardiomyopathy may be:
Dilated
Hypertrophic
Restrictive

Question 2

Explain the aetiology/risk factors of cardiomyopathy

Answer 2

The majority are IDIOPATHIC

Dilated Cardiomyopathy:
Post-viral myocarditis
Alcohol
Drugs (e.g. doxorubicin,cocaine)
Familial
Thyrotoxicosis
Haemochromatosis
Peripartum

Hypertrophic Cardiomyopathy:
Up to 50% are genetic

Restrictive Cardiomyopathy:
Amyloidosis
Sarcoidosis
Haemochromatosis

Question 3

Summarise the epidemiology of cardiomyopathy

Answer 3

Prevalence of dilated and hypertrophic cardiomyopathy is 0.05-0.20%

Restrictive is even rarer

Question 4

Recognise the presenting symptoms of cardiomyopathy

Answer 4

Dilated:
Symptoms of heart failure
Arrhythmias
Thromboembolism
Family history of sudden death

Hypertrophic:
Usually NO SYMPTOMS
Syncope
Angina
Arrhythmias
Family history of sudden death

Restrictive:
Dyspnoea
Fatigue
Arrhythmias
Ankle or abdominal swelling
Family history of sudden death

Question 5

Recognise the signs of cardiomyopathy on physical examination

Answer 5

Dilated:
Raised JVP
Displaced apex beat
Functional mitral and tricuspid regurgitations
Third heart sound

Hypertrophic:
Jerky carotid pulse
Double apex beat
Ejection systolic murmur

Restrictive
Raised JVP (Kussmaul Sign -paradoxical rise in JVP on inspiration due to restricted filling of the ventricles)
Palpable apex beat
Third heart sound
Ascites
Ankle oedema
Hepatomegaly

Question 6

Identify appropriate investigations for cardiomyopathy

Answer 6

CXR:
May show cardiomegaly
May show signs of heart failure

ECG
All Types
Non-specific ST changes
Conduction defects
Arrhythmias

Hypertrophic
Left-axis deviation
Signs of left ventricular hypertrophy
Q waves in inferior and lateral leads

Restrictive
Low voltage complexes
Echocardiography
Cardiac Catheterisation
Endomyocardial Biopsy
Pedigree or Genetic Analysis

Question 7

What indicates cardiomyopathy on Echocardiograph?

Answer 7

Dilated:
Dilated ventricles with global hypokinesia

Hypertrophic:
Ventricular hypertrophy (asymmetrical septal hypertrophy)

Restrictive:
Non-dilated non-hypertrophied ventricles
Atrial enlargement
Preserved systolic function
Diastolic dysfunction
Granular or sparkling appearance of myocardium in amyloidosis