Cardiomyopathy

Question 1

Definition

Answer 1

Primary disease of the myocardium.

Cardiomyopathy may be:
o Dilated
o Hypertrophic
o Restrictive

Question 2

Aetiology/risk factors

Answer 2

· The majority are IDIOPATHIC

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

· Hypertrophic Cardiomyopathy
o Up to 50% are genetic

· Restrictive Cardiomyopathy
o Amyloidosis
o Sarcoidosis
o Haemochromatosis

Question 3

Epidemiology

Answer 3

· Prevalence of dilated and hypertrophic cardiomyopathy is 0.05-0.20%

· Restrictive is even rarer

Question 4

Presenting symptoms (dilated)

Answer 4

o Symptoms of heart failure
o Arrhythmias
o Thromboembolism
o Family history of sudden death

Question 5

Presenting symptoms (hypertrophic)

Answer 5

o Usually NO SYMPTOMS
o Syncope
o Angina
o Arrhythmias
o Family history of sudden death

Question 6

Presenting symptoms (restrictive)

Answer 6

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

Question 7

Signs on physical examination (dilated)

Answer 7

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

Question 8

Signs on physical examination (hypertrophic)

Answer 8

o Jerky carotid pulse
o Double apex beat
o Ejection systolic murmur

Question 9

Signs on physical examination (restrictive)

Answer 9

o Raised JVP
· Kussmaul Sign - paradoxical rise in JVP on inspiration due to restricted filling of the ventricle
o Palpable apex beat
o Third heart sound
o Ascites
o Ankle oedema
o Hepatomegaly

Question 10

Investigations (ECG)

Answer 10

o All Types
· Non-specific ST changes
· Conduction defects
· Arrhythmias

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

o Restrictive
· Low voltage complexes

Question 11

Investigations (echocardiogram)

Answer 11

o Dilated
· Dilated ventricles with global hypokinesia

o Hypertrophic
· Ventricular hypertrophy (asymmetrical septal hypertrophy)

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

Question 12

Investigations (other

Answer 12

· CXR
o May show cardiomegaly
o May show signs of heart failure

· Cardiac Catheterisation

· Endomyocardial Biopsy

· Pedigree or Genetic Analysis