Cardiomyopathy - Hypertrophic Flashcards
Features of hypertrophic CM?
Impairs relaxation of ventricles
Systolic function is usually ok
What is the genetic aspect of hypertrophic CM?
There is a sacromere gene defect that is autosomal dominant
Yet 50% of cases are sporadic so there is only a half chance of getting the gene
How the gene is expressed is not known until time passes and the disease manifests
Pathology if HCM? What is happening?
Myocyte hypertrophy and disarray that is generalised, apical or septal
What is a major problem with septal hypertrophy?
LV outflow tract obstruction and MV defects
Leading cause of sudden death in young people
What else is affected in HCM?
Coronary arteries - ischameia and arrhythmias are common
What should be focused on in a history/what should you always ask?
Family history as it has a genetic aspect
Symptoms of HCM?
Asymptomatic for many until LVOT obstruction sudden death
Fatigue Dyspnoea Palpitations - AF Angina like pain Exertional pre-syncope Syncope related to arrhythmia's or LVOT obstruction
What can be seen on examination?
Can be no findings
Notched pulse pattern Irregular pulse if AFib Double impulse over apex Thrills Raised JVP if filling is very restricted
What can be heard on auscultation?
Dynamic murmurs
LVOT obstruction murmur which increases with valsalva maneuver and decreases with squatting
What investigations can be done?
ECG
Echo’s
Cardiac MRIs
Risk stratification for sequential compresion devices
What will an ECG show up?
Often will be abnormal
Can be normal in the few where the phenotype is poorly expressed in a positive genotype
What are echo’s looking for?
Asymmetrical septal hypertrophy
General measures of treatment?
Avoid heavy exercise and dehydration
Treatment if symptomatic?
BBlockers or CCBs (verapamil)
These aim to reduce ventricular contractility
Treatment if presenting with an arrythmia?
Amiodarone
Anticoagulate for paroxymal AF or systemic emboli
