Cardiomyopathy

Question 1

What is the pathology of hypertrophic obstructive cardiomyopathy (HOCM). (2)

Answer 1

Left ventricular hypertrophy, especially involving the septum.
Causes LV outflow obstruction from asymmetric septal hypertrophy.
There is varying degrees of myocardial fibrosis.

Question 2

Is family history important for HOCM.

Answer 2

Yes.

Approximately 50% of patients have a positive family history.

Question 3

What percentage of patients with HOCM have a positive family history.

Answer 3

Approximately 50%.

Question 4

How many mutations have been associated with HOCM.

Answer 4

Multiple.

Question 5

What other condition is strongly associated with HOCM.

Answer 5

Fredrich’s ataxia.

Question 6

What are the symptoms of HOCM. (78)

Answer 6

Many are asymptomatic. 
Dyspnoea. 
Fatigue. 
Palpitations. 
Angina. 
Syncope.
CCF.
Sudden death (may be the first manifestation).

Question 7

What are the clinical signs of HOCM. (7)

Answer 7

a wave in JVP. 
Double apical impulse. 
Ejection systolic murmur (harsh). 
Systolic thrill at lower left sternal edge.  
Pansystolic murmur at the apex.
4th heart sound.
Jerky pulse.

Question 8

What ECG changes are seen in a patient with HOCM. (6)

Answer 8

LV hypertrophy. 
Deep Q waves (inferior and lateral leads). 
Progressive T wave inversion. 
Arrhythmias (AF, WPW). 
Ventricular ectopics. 
VT.

Question 9

What is the prevalence of HOCM.

Answer 9

0.2%.

Question 10

What is the mode of inheritance of HOCM.

Answer 10

Autosomal dominant condition.

Question 11

What percentage of HOCM cases are sporadic.

Answer 11

50%.

Question 12

What should you ask a patient you suspect of having HOCM.

Answer 12

If there is a family history of sudden death.

Question 13

What may be seen on the echo of a patient with HOCM. (4)

Answer 13

Asymmetrical septal hypertrophy.
Small LV capacity with hypercontractile posterior wall.
Midsystolic closure of aortic valve.
Systolic anterior momement of mitral valve.

Question 14

What is the mortality of HOCM. (2)

Answer 14

5.9% if 14.

Question 15

What are some poor prognostic factors for HOCM. (3)

Question 16

What are the forms of cardiomyopathy. (3)

Answer 16

Dilated cardiomyopathy.
Hypertrophic cardiomyopathy.
Restrictive cardiomyopathy.

Question 17

What are some causes of restrictive cardiomyopathy. (7)

Answer 17

Idiopathic. 
Amyloidosis. 
Haemochromatosis. 
Sarcoidosis. 
Scleroderma. 
Loffler's eosinophilic endocarditis. 
Endomycocardial fibrosis.

Question 18

What is the presentation of restrictive cardiomyopathy. (5)

Answer 18

Presents like constrictive pericarditis.
Features of RVF predominate:
Raised JVP with prominent x and y descents.
Hepatomegaly.
Oedema.
Ascites.

Question 19

What is dilated cardiomyopathy.

Answer 19

It is characterised by dilatation of the ventricular chambers and systolic dysfunction with preserved wall thickness.

Question 20

What is restrictive cardiomyopathy.

Answer 20

A rare condition in which there is normal or decreased volume of both ventricles with bi-atrial enlargement, normal wall thickness, normal cardiac valves and impaired ventricular filling with restrictive physiology by near normal systolic function.

Question 21

What is the prevalence of dilated cardiomyopathy.

Answer 21

1 in 2500.

0.2%.

Question 22

What are the associations of dilated cardiomyopathy. (8)

Answer 22

Alcohol. 
Raised BP. 
Haemochromatosis. 
Viral infection. 
Autoimmune. 
Peri- or post partum. 
Thyrotoxicosis. 
Congenital (X linked).

Question 23

What is the presentation of dilated cardiomyopathy. (7)

Answer 23

Fatigue. 
Dyspnoea. 
Pulmonary oedema. 
RVF. 
Emboli. 
AF. 
VT.

Question 24

What are the physical signs of dilated cardiomyopathy. (11)

Answer 24

Raised pulse. 
Low BP. 
Raised JVP. 
Displacced diffuse apex. 
S3 gallop. 
Mitral or tricuspid regurgitation. 
Pleural effusion. 
Oedema. 
Jaundice. 
Hepatomegaly. 
Ascites.

Question 25

What is the mortality of dilated cardiomyopathy.

Answer 25

Variable, 40% in 2 years.