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Hypertrophic Obstructive Cardiomyopathy

Title

Ñ    Hypertrophic Obstructive Cardiomyopathy

Definition

Ñ    Cardiomyopathy

Characterised by:

Ñ    Variable myocardial hypertrophy

Ñ    Most commonly involving the ventricular septum

Ñ    Disorganisation of cardiac myocytes and myofibrils

Pathogenesis

Causes

Ñ    Autosomal dominant inheritance

Ñ    Sporadic cases may occur

Ñ    Associations:

Friedrich’s ataxia

Mitochondrial myopathy

Glycogen storage disease

Noonan’s syndrome

Pathological Process

Ñ    Variable hypertrophy of the myocardium

Ñ    Disorganisation of cardiac myocytes and myofibrils

Clinical Features

History

CVS

Ñ    Chest pain

Ñ    Dyspnoea

Ñ    Syncope

Ñ    Palpitations:

Cardiac arrhythmias

Ñ    Sudden death

Examination

CVS

Ñ    Jerky carotid pulse

Ñ    Atrial fibrillation

Ñ    Double apical impulse:

Forceful atrial contraction

Ñ    Fourth heart sound

Ñ    Ejection systolic murmur at the base of the heart

Ñ    Pan systolic murmur at the apex:

Mitral regurgitation secondary to systolic anterior movement of the mitral valve

Investigations

Fluids

Genetic analysis

Ñ    Confirms diagnosis

Ñ    Prognostic value

Imaging

ECHO

Ñ    Asymmetric left ventricular hypertrophy

Ñ    Systolic anterior movement of the mitral valve

Cardiac Catheterisation

Ñ    Confirm normal coronary arteries

Ñ    Evaluate degree of mitral regurgitation

Ñ    Measure apex to base pressure gradient in left ventricle

Electrophysiology

ECG

Ñ    LVH

Ñ    ST, T wave changes

Ñ    Abnormal Q waves in inferolateral leads

Exercise testing, Ambulatory ECG monitoring

Ñ    Provides information of prognostic value

Management 

Control

Drugs

Ñ    Chest pain and dyspnoea:

Beta-blockers

Verapamil

Ñ    Anticoagulation:

Atrial fibrillation

Ñ    Risk factors for sudden death:

Amiodarone

Surgery

Ñ    Septal myomectomy (surgical or chemical with alcohol), mitral valve replacement:

Benefit not conclusively proved

Equipment

Ñ    Dual chamber pacing:

Symptomatic patients

Ñ    Implantable defibrillator:

Risk factors for sudden death           

Prognosis

Risk factors for sudden death

Ñ    Family history of sudden cardiac death

Ñ    Genotype

Ñ    Abnormal blood pressure response during exercise

Ñ    Massive left ventricular hypertrophy

Ñ    Non-sustained ventricular tachycardia on Holter monitoring

Two or more of the above risk factors are associated with an increased risk of sudden death

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