Cardio

Hypertrophic Cardiomyopathy

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Pathology

Left ventricular outflow tract obstruction due to asymmetrical hypertrophy of the ventricular septum. Leading cause of cardiac death in young.

Aetiology

Autosomal dominant with positive family history but 50% are sporadic

Signs

‘a’ wave in JVP, double apex beat, harsh ejection systolic murmur

Symptoms

Cardiac death may be first symptom, syncope, dyspnoea, angina, palpitations

Investigations

ECG: Deep T wave inversion and ST depression
Echo: Asymmetrical septal hypertrophy, small left ventricular cavity with 
hyperdynamic function, impaired diastolic function, dilated atria.

Treatment

Medical: Beta blockers or calcium channel antagonist for symptom control. 
Controlled exercise.
Surgical: septal myomectomy, implantable defibrillator, pacemaker

Prognosis

6% mortality/year in those <14 years. 
50% of deaths occur during strenuous exercise reflecting need for controlled exercise treatment
Heart failure or sudden death

Key Facts

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Key References

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