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
what are the investigations that must be carried out for DCM?
- ECG- noting LBBB
- echocardiogram
- cardiac MRI
- coronary angiogram
- sometimes biopsy
- CXR
- basic bloods FBC and U & E
- N terminal pro Brain Natiuretic peptide
what will show up on an echocardiogram in DCM?
dilatation of the left and/or right ventricle
what will show up on an CXR in DCM?
generalised cardiac enlargement
what is a cardiac MRI useful for in DCM?
identifying other aetiologies of LV systolic dysfunction (e. MI) or abdominal myocardial fibrosis
why is a coronary amgiogram carried out for DCM?
exclude coronary artery disease
what are the treatments of DCM?
treat heart failure with; spiranolactone oral nitrates ACE-i Beta Blockers Digoxin warfarin
corrct anaemia
correct endocrine disturbance
reuce fluid and salt intake
cardiac transplant
what is alcohol heart disease?
excessive alcohol consumption can lead to thiamine deficiency, direct toxicity and the adulaterations of alcohol causes effects
it presents similarly to dilated cardiomyopathy
what are the general meausures to control symptoms of DCM?
avoid heavy exercise
avoid dehydration
what are the infiltrative causes of restrictive and infiltrative cardiomyopathy?
amyloid sarcoid
what are the non-infiltrative causes of restrictive and infiltrative cardiomyopathy?
familial forms of HCM sclerodoma diabetes pseudoxanthoma elasticum
what are the storage diseases that can cuase restrcictive and infiltrative cardiomyopathy?
haemachromatosis
Fabry disease
what are the endo myocrdial caused of restrictive and infiltrative cardiomyopathy?
fibrosis
carcinoid
radiation
drug effects
what is the pathology of restrictive and infiltrative cardiomyopathy?
inability to fill the ventricles as they have reduced compliance. This leads to atrial dilatation as they gave to work harder and atrial fibrillation.
what are the investigations carried out for restrictive and infiltrative cardiomyopathy?
- repeated ECG (noting LBBB and other conduction defects)
- ECHO
- cardiac MRI
- biopsy sometimes useful for amyloid and sarcoid
- CXR
- N termial pro Brain Natriuretic Peptide
- Basic bloods, U and E and look out for sarcoid and haemochromatosis
- autoantibodies for sclerotic CT diseases
- low plasma alpha galactoside A activity for Fabry
what are the treatments for restrictive and infiltrative crdiomyopathy?
- anticoagulants if AF
- limited diuretic as low filling pressures cause problems
- Beta blockers
- limited ACEi
- ICD or CRT-D/P implant rare as prognosis is so low
- cardiac transplant
what is myocarditis?
acute or chronic inflammation of the myocardium
what other heart conditions can myocarditis cause?
myocardial function impairment
conduction impairment- heart block
generate arrhthmia
what are the causes of myocarditis?
viral - common bacterial fungal and parasitic - uncommon toxins eg. cocoaine hypersensitivity autoimmune activation
what are the symptoms of myocarditis?
assymptomatic heart failure fatigue SOB chets pain in a 1/4 patients fever
what are the signs of myocarditis?
tachycardia
soft heart sounds
prominent third heart sound
pericardial rub
what can chronic myocarditis lead to?
enlarged and hypertrophied heart and dilated cardiomyopathy
what arethe investigations crried out for myocarditis?
ECG
ECHO
cardiac MRI
biopsy
troponin raised but unlike in MI it doesnt fall viral dna pcr autoantibodies strep antibodies lyme B burgdorferi HIV
what are the general meaures for the treatment of mocarditis?
- treatment of heart failure
- treatment of brady/tachy arrhythmias
- immunotherapy
- stop possible drugs bringing about inflammation
what is pericarditis?
inflammation of the pericardial layers (with or without the myocardium)
what are the most common causes of pericarditis?
viral bacterial post MI perforation dissection of proximal aorta neoplasia autoimmune response
what are the symptoms of pericarditis?
chest pain:
relieved by sitting forward
execerbated by movement, repsiration and lying down
fever
what are the signs of pericarditis?
pericardial rub fever raised JVP (if effusion causinf haemodynamic effects) low BP muffled heart sounds
what are the investigations carried out for pericarditis?
ECG- ST segment raised and concave and PR dependent
echo
troponin (raised if myocardium involved)
CXR
what is the treatment of viral pericarditis?
conservative
what is the treatment of idiopathic pericarditis?
colchicine
limited use of NSAIDS
what is the treatment of bacterial pericarditis?
drain pericardial effusion
antimicrobials after
what is tamponade?
haemodynamically significanr pericardial effusion
what are the causes of pericardial effucion?
often same as pericrditis
what are the symptoms of pericardial effusion?
fatigue
SOB
dizzy with low BP
occasionally chest pain
what are the signs of pericardil effusion?
pulsus paradoxus JVP raised low BP rub muffled HS
what is pulsus paradoxus?
an abnormally large decrease in stroke volume, systolic blood pressure and pulse wave amplitude during inspiration
what are the tests carried out for pericardial effusion?
echo
CXR
what is the treatment of pericardial effusion?
drainage
underlying cause
what are the causes of constrictive pericarditis?
idopathic radiation post surgery autoimmune renal failure sarcoid.
what is the pathology of constrictive pericarditis?
impaired filling although myocardium is normal
what are the symptoms of contrictive pericarditis
fatigue
SOB
cough
what are the signs of constrictive pericarditis?
RHF with oedema ascites high JVP jaundice hepatomegaly AF TR pleural effusion pericardial knock
what are the investigations for constrictive pericarditis?
echo
right heart catheter to differentiate from RCM
ECG
CXR
what is the treatment of constrictive pericarditis?
limited diuretic
pericardectomy
