HYPERTROPHIC CARDIOMYOPATHY

Question 1

Cardiomyopathies

Answer 1

A group of diseases of the MYOCARDIUM affecting its mechanical (hypertrophic, dilated, restrictive cardiomyopathies), or electrical functions (Long QT syndrome)

Question 2

Definition (4)

Answer 2

1- Characterized my marked ventricular hypertrophy in the absence of abnormal loading conditions “hypertension and valvular diseases”
2- The hypertrophic ventricle has an impaired diastolic filling phase leading to reduced stroke volume
3- Most cases are familial, autosomal dominant, and caused by mutation in the genes encoding Troponin T and beta myosin (sacromeric proteins)
4- Most common cause of sudden death in young people

Question 3

Clinical Features (7)

Answer 3

1- Patients may be asymptomatic or have breathlessness, angina, syncope
2- Jerky pulse (rapid upstroke followed by a rapid fall)
3- Ejection systolic murmur (due to left ventricular outflow obstruction)
4- Pansystolic murmur (as a result of functional mitral regurgitation)
5- Bisferious pulse (two upstrokes of the carotid pulse)
6- Double apical pulsation
7- Standing and Valsalva maneuvers increase the intensity of these murmurs, unlike squatting and sustained handgrips which decrease them

Question 4

Complications (5)

Answer 4

1- Sudden death
2- Thromboembolism
3- Arrhythmias
4- Infective endocarditis
5- Heart failure

Question 5

Investigations (3)

Answer 5

1- ECG: signs of LVH (Deep S in V1 V2, tall R in V5 V6, both together > 35mm)
2- Echo: septum 1.5 times the thickness of the posterior wall
3- Genetic analysis

Question 6

Management (6)

Answer 6

1- Amiodarone: reduces risk of arrhythmias and sudden death
2- ICD: for high risk patients with 2 or more of:
- Massive LVH (>30mm on echo)
- FH of sudden cardiac death (<50 y/o)
- Non-sustained V.tach on 25-hr Holter monitoring
- Prior unexplained syncope
- Abnormal BP response on exercise (flat or hyporesponsive)
3- Beta blockers (best) and Verampil for chest pain and dyspnea
4- Vasodilators should be avoided bc they aggravate the ventricular outflow obstruction and so cause refractory hypotension
5- Catheter ablation of the septum
6- Surgical myomectomy (if all others fail)