Cardiomyopathy

Question 1

What is cardiomyopathy?

Answer 1

A group of diseases of the heart that effect the mechanical or electrical function of the heart making it harder for it to pump blood

Question 2

What are the different types of cardiomyopathy?

Answer 2

• restrictive
• arrhythmogenic
• dilated
• hypertrophic
• unclassified

Question 3

what is restrictive cardiomyopathy?

Answer 3

• a stiff heart with a lack of compliance (diastolic dysfunction) which can appear grossly normal

Question 4

cause of restrictive cardiomyopathy?

Answer 4

• idiopathic
• amyloidosis
• sarcoidosis
• oesinophilic endocarditis
• fibrosis following radiation

Question 5

signs and symptoms of restrictive cardiomyopathy?

Answer 5

the same as constrictive pericarditis

Signs

• right heart failure
• soft apex beat
• S3
• diastolic pericardial knock
• ascites
• oedema
• splenomegaly

Question 6

how to tell the difference between restrictive cardiomyopathy and constrictive pericarditis?

Answer 6

-MRI/CT

Question 7

treatment for restrictive myopathy?

Answer 7

-treat the underlying cause

Question 8

what is the most common cause of sudden cardiac death in young people?

Answer 8

-hypertrophic cardiomyopathy

Question 9

what type of myopathy that causes apple green bifringence in polarised light?

Answer 9

-restrictive cardiomyopathy

Question 10

what is the change in histology for hypertrophic cardiomyopathy?

Answer 10

-the myofibres become disorganised and swirly

Question 11

cause of hypertrophic cardiomyopathy?

Answer 11

• autosomal dominant gene

- most commonly affecting tropomyosin/beta myosin/ alpha myosin/ myosin binding protein C

Question 12

symptoms of hypertrophic cardiomyopathy?

Answer 12

• sudden death
• chest pain
• syncope
• dysponoea
• cardiac arrhythmias
• palpitations
• angina

Question 13

investigations for hypertrophic cardiomyopathy?

Answer 13

• echo

- ECG (LVH)

Question 14

what would be seen on an echo of someone with hypertrophic cardiomyopathy?

Answer 14

• asymmetrical septal hypertrophy

- small LV cavity

Question 15

management for hypertrophic cardiomyopathy?

Answer 15

SYMPTOMS

• BB
• CCB (verapamil)

ARRHYTHMIAS
-amiodarone

anticoagulation and ICD pacemaker

Question 16

what is dilated cardiomyopathy?

Answer 16

-muscle in the heart become flabby and dilated, causing systolic dysfunction

Question 17

causes of dilated cardiomyopathy?

Answer 17

• 50% of time genetic (autosomal dominant gene)
• toxins
• alcohol
• chemotherapy
• cardiac infection
• pregnancy
• autoimmune

Question 18

symptoms of dilated cardiomyopathy?

Answer 18

• HF
• shortness of breath
• fatigue
• low ejection fraction
• arrhythmias
• sudden death

Question 19

investigations of dilated cardiomyopathy?

Answer 19

• CXR (cardiomegaly and pulmonary oedema)
• echo (increased size of ventricles)
• ECG (tachycardia)

Question 20

complications of dilated cardiomyopathy?

Answer 20

-HF

Question 21

management of dilated cardiomyopathy?

Answer 21

• diuretics
• BB
• ACEI
• anticoagulation
• ICD pacemaker
• LVADs
• transplantation

Question 22

what is arrhythmogenic right ventricular dysplasia?

Answer 22

-rarest subtype if cardiomyopathy causing RV almost entirely replaced by fat

Question 23

cause of arrhythmogenic right ventricular dysplasia?

Answer 23

-autosomal dominant condition

Question 24

presentation of arrhythmogenic right ventricular dysplasia?

Answer 24

• funny turns
• syncope
• arrhythmia
• sudden death

Question 25

complications of arrhythmogenic right ventricular dysplasia?

Answer 25

-HF

Question 26

management of arrhythmogenic right ventricular dysplasia?

Answer 26

• BB for asymptomatic arrhythmias
• amiodarone for symptomatic arrhythmias
• ICD for life threatening arrhythmias