Hypertrophic Obstructive Cardiomyopathy (HOCM)

Question 1

PATHOLOGY: HOCM

Answer 1

• Left ventricular hypertrophy (esp. involving septum)
• Varying degrees of myocardial fibrosis

Question 2

AETIOLOGY: HOCM

Answer 2

• Autosomal dominant disorder
  
  • Mutated genes involved in contractile proteins
  • Associated w/ Freidrich’s Ataxia
• Men>Women
• 1:500

Question 3

SYMPTOMS: HOCM

Answer 3

• Asymptomatic*
• SOB
• Fatigue
• Palpitations
• Angina
• Syncope
• Sudden death (most common cause in young patients)

Question 4

CLINICAL SIGNS: HOCM

Answer 4

• Peripheral*
• JVP a wave
• Central*
• Double apical impulse
• Ejection systolic mumur at lower left sternal border
• Pansystolic murmur at apex
• S4

Question 5

INVESTIGATIONS: HOCM

Answer 5

Imaging

1. ECG

• LV hypertrophy
• Q waves
• Arrhythmias

2. CXR

• Increased cardiothoracic ratio

3. Echo* (GOLD)

• Assymetric septal hypertrophy
• Increased myocardium thickness
• Left-ventricle is non-dilated
• Absence of underlying cardiac disease

Question 6

MANAGEMENT: HOCM

Answer 6

1. Asymptomatic patients –

• can generally be left alone. They should be
• assessed for risk of sudden cardiac death (consider ICD)

2. Control arrhythmias

• amiodarone often used

3. Control Left Ventricle function

• beta blockers and verapamil
• Surgical myectomy

4. High risk patients

• Implanatable carioverter defibrillator
• Heart transplant