Cardiomyopathies

Question 1

Four main classifications of cardiomyopathies?

Answer 1

Dilated (commonest)
Hypertrophic
Restrictive
Arrythmogenic right ventricular cardiomyopathy (ARVC)

Question 2

How does dilated cardiomyopathy usually present?

Answer 2

With heart failure; may also be arrythmia/syncope/peripheral emboli

Question 3

Viral cause of dilated cardiomyopathy?

Answer 3

Coxsackie virus

Question 4

Commonest causes of dilated cardiomyopathy?

Answer 4

Idiopathic (50%)

Familial (20%)

Question 5

Management of dilated cardiomyopathy? (4)

Answer 5

Advise abstinence from alcohol
Most need long-term anticoagulation
Management of heart failure/arrythmias (e.g. CRT, ICD)
Surgery- cardiomyoplasty/transplant

Question 6

Asymmetrical septal hypertrophy +/- outflow obstruction

Answer 6

hypertrophic (+/- obstructive) cardiomyopathy

Question 7

Inheritance of H(O)CM?

Answer 7

Autosomal dominant

50% sporadic

Question 8

How are most cases of HOCM diagnosed?

Answer 8

Screening of asymptomatic patients with a family history

Question 9

Why is digoxin contraindicated for control of AF in HOCM?

Answer 9

Positively inotropic; can exacerbate symptoms of left ventricular outflow tract obstruction (LVOTO)

Question 10

Causes of restrictive cardiomyopathy? (3)

Answer 10

Amyloid
Sarcoid
Haemochromatosis

Question 11

Autosomal dominant condition causing fatty infiltration of the right ventricular wall, predisposing to arrythmia and sudden death

Answer 11

Arrythmogenic right ventricular cardiomyopathy

Question 12

How should family history of cardiac sudden death be managed? (3)

Answer 12

Refer 1st degree relatives of people who died <45 years of age
Antenatal screening for HOCM + LQTS is possible if mutation known
Regular Echo + ECG