cardiomyopathy and specific heart disease Flashcards
what is cardiomyopathy?
disease of the heart`
what are the main types of cardiomyopathy?
hypertrophic cardiomyopathy
dlated cardiomyopathy
restrictive cardiomyopathy
myocarditis
what are the main pericardial diseases?
pericaditis
constrictive pericarditis
pericardial effusion-leads to pericardial tamponade
what is hypertrophic cardiomyopathy?
inappropriate ventricular hypertrophy (myocardium hypertrophied without any other cause)
is hypertrophy of ventricles from aortic stenosis or hyertension hypertrophic cardiomyopathy?
no
is hypertrophy in hypertrophic cardiomyopathy symmetrical?
no (hypertrophy is symmetrical in appropriate hypertropy eg. hypertension)
where does hypertrophy normally occur in HCM?
the IV septum
apices
generalised hyertrophy
what is the aetiology of HCM?
autosomal dominant inheritance of a single point mutation of sarcomere protein. Phenotypic variation within and between families
what are the three main pathological outcomes if HCM?
- increased muscle mass
- mitral regurgitation
- increased risk if pulmonary oedema
what are the problems caused by increased muscle mass in HCM?
- decreased size of ventricle
- lower LV compliance
- blockage of ventricular outflow tract
how can HCM cause mitral regurgitation?
blockage of LV outflow tract caises Venturi effect
Mitral leaflets are sucked towards the IV septum so they cannot close together
how does HCM increase the risk of pulmonary oedema?
- reduced LV compliance means blood ejected less easily
- so causing higher pressure in the LV
- high pressure backs up to the pulmonary circulation
what are the symptoms of HCM?
asymptomatic for many dyspnoea chest pain palpitations (cardiac arrhythmias) syncope dizziness sudden death: life-threatening arrythmias
what are the signs of HCM?
- pansystolic murmur
- notched carotid pulse/steeprising irregular if in AF
- ejection systolic murmur
- fourth heart sound and double apical pulsation
- JVP raised in very restrictive filling
why does HCM cause a pansystolic murmur?
mitral regurgitation
why does HCM cause jerky carotid /steep rising pulse?
rapid ejection then sudden obstruction of outflow tract
why does HCM cause ejection systolic murmur?
LV outflow tract obstruction in systole
why is there a 4th herat sound and double apical pulsation in HCM?
forceful atrial contraction
what are the investigations carried out for HCM?
ECG
echocardiogram
cardiac MRI
genetic analysis (confirm diagnosis)
what are the general aims if treatment of HCM?
relieving symptoms
preventing sudden death
what are the treatments relieving symptoms of HCM
Beta blockers
CCBs- verapamil
what are the trearments carried out to prevent sudden death from HCM?
alcohol ablation
surgical resection
implantable cardioverter-defibrillation (ICD)
what can put HCM patients at risk if sudden cardiac death?
- family history of sudden cardiac death
- gene specific
- syncope
- ventricular tachycardia
- exercise hypotension
- massive left ventricular hypertrophy
what is the definition of dilated cardiomyopathy?
heart muscle of LV becomes thin and stretched out so the heart is unable to pump effectively
what is the commonest cardiomyopathy and commonest cause of heart failure?
DCM
what is the aetiology of DCM?
Genetic and familial DCM: SCN5A gene, muscular dystrophy
Inflammatory, infectious, autoimmune, postpartum
Toxic; drugs, exogenous chemicals, endocrine
Injury, cell loss, scar replacement
what are trhe conditions that DCM commonly presents with?
heart failure cardiac arrhythmias conduction defects thromboembolism sudden death
what are the symptoms of DCM?
- dyspnoea
- fatigue
- peripheral oedema
- orthopnoea
- paroxysmal nocturnal dsypnoea
- weight gain
- cough
what are the signs of DCM?
thready pulse
irregular pulse if in AF
narrow pule pressure
poor superficial perfusion
diplaced apex beat
S3 and S4
MR murmur
pulmonary oedema
pleural effusion
SOB at rest
ankle oedema sacral oedema ascites hepatomegaly JVP elevated