HOCM
| Can cause Sudden cardiac death | |
|---|---|
| Etiology | asymmetric septal hypertrophy ⇒ LV outflow obstruction. Blunt cardiac trauma can lead to arrhythmia. |
| Epidemiology | Autosomal dominant inheritance, 50% sporadic. Typically younger male. |
| Clinical presentation | Dyspnoea, angina, palpitation, sudden syncope ⇒ in young people AF, a wave in JVP, systolic thrill lower L sternal edge (louder when standing). Murmur: harsh ejection systolic murmur Apex beat: double-apex beat Pulse: jerky, pulsus bisferiens (subaortic stenosis) |
| Pathogenesis | Disordered myocyte architecture, subaortic fibrosis |
| Diagnostic investigations | ECG: LVH; T wave inversion; deep, narrow Q waves in inferolat leads; WPW, ventricular ectopics; P mitrale Echo: asymmetric septal hypertrophy, hypercontractile LV post wall, diastolic dysfunction |
| Management | - aim ↓ ventricular contractility with beta blockers or verapamil; arrhy: amiodarone; paroxsymal AF: anticoagulate - consider ICD. - Poor prognosis if age <14, syncope at presentation, FHx |