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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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