Hypertrophic Cardiomyopathy

Hypertrophic Cardiomyopathy

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

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