Cardiomyopathies

Question 1

What is Hypertrophic Obstructive Cardiomyopathy?

Answer 1

Autosomal dominant inheritance
Most common cause of cardiac death in young athletes
Mutation leads to left ventricular hypertrophy and remodelling

Question 2

What are clinical features of HOCM?

Answer 2

Syncope
Angina
Jerky pulse
Ejection systolic murmur
SoB on exertion
Double apical pulse
S4 heart sound

Associated with WPW and frederichs ataxia

Question 3

What investigations are required for HOCM?

Answer 3

ECG
- Deep Q waves
- Progressive T wave inversion
- LAD

ECHO
- Mitral regurg
- Systolic anterior motion of anterior mitral valve and asymmetric septal hypertrophy

Question 4

What is the management of HOCM?

Answer 4

ICD
Dual chamber PM
Amiodarone
Beta blockers
Rate Limiting CCBs

Question 5

What are features of dilated cardiomyopathy?

Answer 5

Most common- all 4 chambers dilate

Causes
- alcohol
- chemotherapy esp doxorubicin
- Myocarditis
- Peripartum
- Duchennes

CXR- balloon heart
3rd HS

Question 6

What are features of restrictive cardiomyopathy?

Answer 6

Diastolic dysfunction with impaired ventricular filling

Causes
- amyloidosis
- Post radiotherapy
- haemochromatosis
- scleroderma

ECG- low voltage complexes, bundle branch block

Question 7

What are features of Takotsubo cardiomyopathy?

Answer 7

Ballooning of myocardial apex due to severe hypokinesia

Causes
- severe stress e.g. bereavement

Chest pain, ST elevation but normal coronary angios

Treat supportively

Question 8

What are features of Arrhythmogenic right ventricular cardiomyopathy (ARVC)?

Answer 8

Second most common cause of cardiac death
autosomal dominant
Right ventricular myocardium replaced by fibrous fatty tisue

Question 9

What are investigations of ARVC?

Answer 9

ECG changes
- T wave inversion in V1-V3
- Epsilon wave

Management
- Sotalol
- ICD
- Catheter ablation to reduce risk of VT

Question 10

What are features of Brugada Syndrome?

Answer 10

Autosomal dominant inheritance
More common in Asian ethnicities

Question 11

What investigations are needed for Brugada?

Answer 11

ECG
- convex ST elevation following by negative T wave
- PArtial RBB
- Flecainide provocation test- ECG changes become more obvious

Manage with ICD

Question 12

What are features of long QT syndrome?

Answer 12

Autosomal dominant

Subtypes
-LQT1- exertional syncope e.g. swimming
- LQT2- present with syncope following auditory stimuli or emotional stress

Manage
-ICD

Question 13

What are features of Catecholaminergic polymorphic ventricular tachycardia?

Answer 13

Autosomal Dominant

Emotional stimuli/exertion causes PVT presenting with syncope or sudden death

Management
- ICD/BB