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

Hypertrophic cardiomyoptahy;
50% are genetic

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

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%

Dilated is most common

Restrictive is even rarer

Question 4

Recognise the presenting symptoms of cardiomyopathy

Answer 4

Dilated;
Symptoms of heart failure.. Dyspneoa
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
ECG
Echo

Cardiac catheter
Pedigree/ Genetic analysis
Endomyocardial biopsy

Question 7

What would the investigations for cardiomyopathy show?

Answer 7

1. CXR; May show cardiomegaly
   May show signs of heart failure

2. ECG 
A. All Types 
  - Non-specific ST changes  
  - Conduction defects  
  - Arrhythmias  

B. Hypertrophic

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

C. Restrictive
Low voltage complexes

2. Echocardiography
   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

Question 8

Complications?

Answer 8

progressive heart failure

Question 9

rx?

Answer 9

Treat as per Heart failure

+- Heart transplant