Cardiomyopathy

Question 1

Hypertrophic obstructive cardiomyopathy : Pathophysiology

Answer 1

Autosomal dominant inheritance : defect in sarcomere protein

1. Left ventricular hypertrophy - disorganised muscle growth in LV and AV septum
2. Hypertrophied septum
3. left ventricular outflow tract* (LVOT) obstruction
4. Displaced mitral valve leaflets - Mitral regurgitation

Question 2

Hypertrophic obstructive cardiomyopathy : Clinical features

Answer 2

Leading cause of sudden cardiac death in young athletes
May be asymptomatic
1. SOB, Syncope
2. Chest pain, palpitation
3. Most are asymptomatic
4. Assoc FH of sudden death

Question 3

Hypertrophic obstructive cardiomyopathy : Clinical signs

Answer 3

1. Ejection systolic murmur - 2nd to blockage
2. Mitral regurgitation - pan systolic murmur
3. Increased risk of arrhythmia - AF

Question 4

Hypertrophic obstructive cardiomyopathy : Investigations

Answer 4

Diagnosis : ECHO/Cardiac MRI
ECG - LV hypertrophy

Question 5

Hypertrophic obstructive cardiomyopathy : Management

Answer 5

Avoid ACEi + nitrates - as can worsen lLVOT obstruction
* Amiodarone
* Beta-blockers or verapamil for symptoms
* Cardioverter defibrillator
* Dual chamber pacemaker

Question 6

Arrhythmogenic cardiomyopathy- Definition

Answer 6

a genetic condition where the heart muscle is progressively replaced with fibrofatty tissue.

It becomes prone to ventricular arrhythmias.

It is a notable cause of sudden cardiac death in young people, including high-performing athletes.

Question 7

Dilated cardiomyopathy : Definition

Answer 7

condition where the heart muscle becomes thin and dilated
Classic causes include
* Alcohol } most common
* Coxsackie B virus

Question 8

Restrictive cardiomyopathy- Definition

Answer 8

heart becomes rigid and stiff, causing impaired ventricular filling during diastole.

Question 9

Restrictive Cardiomyopathy : Causes

Answer 9

Amyloidosis