Pathology: Left ventricular outflowtract obstruction due to asymmetrical hypertrophy of the ventricular septum.Leading cause of cardiac death in young.
Aetiology: Autosomal dominant withpositive family history but 50% are sporadic
Symptoms: Cardiac death may be firstsymptom, syncope, dyspnoea, angina, palpitations
Signs: ‘a’ wave in JVP,double apex beat, harsh ejection systolic murmur
Investigations: ECG:Deep T wave inversion and ST depression
Echo: Asymmetrical septal hypertrophy, smallleft ventricular cavity with
hyperdynamic function, impaired diastolic function,dilated atria.
Treatment: Medical: Beta blockers or calcium channel antagonist for symptomcontrol.
Controlled exercise.
Surgical: septal myomectomy, implantabledefibrillator, pacemaker
Complications: Heart failure or sudden death
Prognosis: 6% mortality/year in those<14 years.
50% of deaths occur during strenuous exercisereflecting need for controlled exercise treatment
