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6 Cards in this Set

  • Front
  • Back

Define?

Marked ventricular hypertrophy in the absence of any abnormal loading conditions such as valvular disease / high BP


This impairs diastolic filling = Decreased Stroke Volume

Causes

Mostly Autosomal Dominant - Genes that encode sarcomeric proteins = Deficiency in Troponin T/ B-Myosin/A-Tropomyosin

Clinical Features?

SADP


Syncope


Angina


Dyspnoea


Palpitations

Complications?

Sudden Death


Arrhytmias


Heart Failure


JERKY PULSE


Thromboembolism


Ejection systolic murmur

Investigations

1) ECG = LV hypertrophy with no discernable causes = DIAGNOSTIC


2) Echo = LV Hypertrophy


3) MRI = Fibrosis

Management

1) Amiodarone


2) Implantable Cardiac Defirbillator


3) B-Blockers / ACE-I


4) Surgical Resection/ Alcohol Ablation / Dual Chamber Pacing