Hypertrophic Cardiomyopathy Flashcards
1
Q
ESSENCE
A
Cardiomyopathy characterised by ventricular hypertrophy and diastolic dysfunction
2
Q
AETIOLOGY
Risk factors
A
Family history sudden cardiac death
3
Q
AETIOLOGY
Most common
A
Familial
4
Q
GENETICS
Inheritance pattern
A
Autosomal dominant
5
Q
GENETICS
Mutations
A
- Chromosome 14
- Genes encoding sarcomere proteins
- Myosin binding protein C and B-myosin heavy chain
6
Q
CLINICAL FEATURES
Symptoms
A
- Syncope during exercise
- Dyspnoea on exertion
- Angina
- Palpitations
- Sudden death from ventricular arrhythmia
7
Q
CLINICAL FEATURES
Signs
A
- S4 gallop (sign of left ventricular hypertrophy)
- Systolic murmur without radiation to carotides
- Mitral regurgitation
8
Q
INVESTIGATIONS
First choice
A
- Echocardiography
- ECG
9
Q
INVESTIGATIONS
ECHO findings
A
- Maybe normal ejection fraction
- Hypertrophy of ventricular walls and interventricular septum
- Mitral regurgitation
10
Q
INVESTIGATIONS
ECG findings
A
Left ventricular hypertrophy
11
Q
How to distinguish from restrictive and dilated
A
- Restrictive has
- Kussmaul sign on exam
- ECG with low voltages
- Dilated has
- ECHO with reduced ejection fraction
- Progressive heart failure
12
Q
MANAGEMENT
Approach
A
- Typically managed with B blockers and CCB
- Diuretics may be used cautiously in patients without left ventricular outflow tract obstruction
- Digoxin and spironolactone no benefit
13
Q
MANAGEMENT
Conservative options
A
Avoidance of overexertion
14
Q
MANAGEMENT
Medical lines
A
- 1) Beta blockers
- 2) CCB if contraindicated for beta blockers
15
Q
MANAGEMENT
Surgical options
A
- Surgical septal myectomy
- Implntable cardioverter defibrillator (ICD)
