Cardiomyopathy and myocarditis Flashcards
What is dilated cardiomyopathy?
- dilation of the ventricles and thinner ventricular walls, hence affecting ventricular function
- strength of muscle contraction will be weaker
- can be primary/end result of any pathological insult to the myocardium
Causes of dilated myopathy
- Genetic and familial: SCN5A gene, muscular dystrophy
- inflammatory: viruses, autoimmune, postpartum
- toxic; drugs, exogenous chemicals, alcohol, endocrine
- Injury, cell loss, scar replacement
- Ischaemia/valvular causes
Irreversible/reversible causes of dilated cardiomyopathy
-alcohol
-endocrine
-tropical disease
-post partum
-haemaochrmatosis ( ion storage disease)
sarcoid
Symptoms of dilated cardiomyopathy
Slow onset
- dyspnoea
- fatigue
- orthopneoa
- PND
- ankle oedema
- weight gain of fluid overload
- cough
PMX of dilated cardiomyopathy
- systemic illness
- travel
- vascular disease
- thyroid, neuromuuscular disease
Examination of cardiomyopathy (findings)
-poor superficial perfusion
-thready pulse
0irregular in AF
-sob at rest
-narrow pulse pressure
-high JVP
-may have TR waves?
-displaced apex
-S3, S4
-MR murmur
=pumnary oedema, PE, ankle oedema, sacral odema, acites, hepatomegaly
Investigation of dilated cardiomyopathy
- ECG
- CXR
- N termial prp BNP?
- Bloods ( FBC, U+E)
- ECHO
- CMRI
- Coronary angiogram
- Biopsy
Treatment -Non specific
Correct:
- anaemia
- exacerbating drugs ( NSAIDS)
- endocrine disturbance
- reduce fluid/salt intake
- manage weight
- reduce alcohol intake
Treatment - Specific
- ACEI
- ATII blockers
- diuretics
- beta blockers
- spironlactone
- anticoagulants
- cardiac transplant
- SCD risk assestment with ICD/CRT-DP implant ( pacing systems)?
What is restrictive and infiltrative cardiomyopathy?
- when ventricle has reduced compliance so cannot fill well.
- stiffness
Causes of restrictive and infiltrative cardiomyopathy
-Non infiltrative: familial, forms of HCM, scleroderma, diabetic, pseudoxanthoma elasticum
Infiltrative; amyloid, sarcoid
Storage disease; haemachromatosis, fabry disease
Endomyocardial; fibrosis, carcinoid, radiation, drug effects
Evalutation of restrictive and infiltrative cardiomyopathy
- ECG
- CXR
- N termial prp BNP?
- Bloods ( FBC, U+E ; look out for sarcoid + haemachromatosis)
- autoantivoies for sclerotic CT diseases
- amyloid requires non cardiac biopsy to establish diagnosis
- fabry; low plasma alpha galactosidase A activity
- ECHO
- CMRI
- Coronary angiogram
- Biopsy (more helpful but high false negative rate)
Treatment - Specific for restrictive infiltrative cardiomyopathy
- limited diuretics as low filling pressures cause problems
- beta blockers limited
- anticoagulants
- cardiac transplant
- SCD risk assestment with ICD/CRT-D/P implant ( pacing systems)?
- if Fe overload, specific forms of amyloid of fabrys then specific treatment are available
- endomyocardial fibrosis has little specific
Prognosis of restrictive and infiltrative cardiomyopathy
-if irreversivle poor prognosis
What is hypetrophic cardiomyopathy
-part of heart becomes thickened idiopathic. This results in heart being less able to pump properly
Causes of hypertrophic cardiomyopathy
Genetics
-sacromere gene defect which is autosomal dominant.
Describe the pathology of Hypertrophy cardiommyopathy
- myocyte hypertrophy and disarray
- segmental wall thickness >14mm or >12mm in primary relative
- can be apical, septal or generalised
- impaired relaxation so behaves in a restrictive manner
- if septal hypertrophy this can with mitral valve defect lead to LVOT obstruction
- CA also affected with small vessel narrowing and consequent ischaemia and fibrosis, arrythmias are common
Symptoms of hypertrophic cardiomyopathy
- asymptomatic
- fatigue
- dyspnoea
- angina
- chest pain (exertional)
- syncope related to arrhythmias
Examination of hypertrophic cardiomyopathy
- none
- notched pulse pattern
- irregular pulse if in AF or ectopy
- double impulse over apex, thrills and murmurs, often dynamic, LVOT murmur will will increase with valsalve and decrease with squatting
- JVP can be raised in very restrictive filling
Symptoms of hypertrophic cardiomyopathy
- breathlessness
- palpitations
- syncope
- exertional symptoms
- SCD
- asymptomatic
- fatigue, anginal chest pain, exertional pre syncope
Examination findings of hypertrophic cardiomyopathy
- none
- notched pulse pattern/irregular
- double impulse over apex, thrills, murmurs, dynamic, LVOT murmur will increase with valsalve and decrease with squatting
- JVP can be raised in a restrictive filling
Investigation of hypertrophic cardiomyopathy
- ECG ( abnormal but few normal where phenotype is poorly expressed in genotype +ve individuals)
- Echo
- CMRI
- risk for SCD, may need ICD
Non specific treatment for hypertrophic cardiomyopathy
- avoid heavy excercise/dehydration
- consider genetic testing
- regular FU to re appraise risks and progress
Specific treatmet for hypertrophic caridomyopathy
- drugs to try and enhance relaxation
- beta blockers, verapamil, disopyrimide
- if AF anricoagulate
- surgical/alcohol septal ablation
- ICD if required based on risk stratification
What is myocarditis
- inflammation of myocardium
- acute/chronic. Impaired myocardial function, conduction and generates arrythmia
Causes of myocarditis
- can eventually take on dilated cardiomyopathy appearance
- viral
Describe the pathology of myocarditis
-infiltration of inflammatory cells into myoscardial layers, reduced function and HF, heart block as confuction system is involed and arrhythmias
Symptoms of myocarditis
- HF
- fatigue
- SOB
- CP? ( 26%)
Investigation of myocarditis
- ECG ( abnormal)
- biomarkers elevated
- ECHO
- CMRI ( oedema)
- biopsy ( low threshold?)
- viral DNA PCR
- auto antibodies
- step antibodies
- Lyme B burgoderferi
- HIV
Treatment of myocarditis
- immmunotherapt if biopsy or others indicated diagnosis
- stop drugs / toxic agent exposure
What is pericardial disease/pericarditis
- reflected lining over epicardium and parietal
- inflammation of pericardial layers with/without myocardial involvement
Causes of pericardial disease/pericarditis
- idiopathic
- viral
- bacterial, post MI, perforation, dissection of proximal aorta
- neoplasia
Symptoms of pericardial disease
- chest pain with pleuritic features
- postual fetures?
- sitting forward improves it, vise versa
- fever
-pericardial rub LSE, raise JVP if effusion present, low BP, muffled HS,
Investigations of pericarditis
- ECG ( ST elevation and PR depression)
- ECHO
- high troponin if myocardial involvement
Treatment of pericarditis
- viral conservative
- idiopathic = colchicine + limited use of NSAIDS
- bacterial must be drained even if small effusion and antimicrobrials
- if large effusion, haemodynamic effects then drain
What is a pericardial effusion?
- where tamponade is present?
- build up of fluid in pericardial space
Symptoms of PE
- fatigue
- SOB
- dizziness
- low BP
- chest pain
- pulsus paradoxus
- JVP high
- low BP
- may/may not jhave pericardial rub /muffled HS
- pulmonary oedema (rare in pericardial effusion/tamponade)
Investigations of pericardial effusion
- ECHO ( cardiac shadow)
- CXR ( cardiac shadow)
- drainage
- send for MCS, neoplasic cells, protein and LDH ( most are exudates)
What is constrictive pericarditis
- thickening, fibrosis of pericardium which limits heart ability to function
- myocardium unaffected
Causes of contrictive pericarditis
- idiopathic
- radiation
- post surgery
- autoimmine
- renal failure
- sarcoid
Pathology of constrictive pericarditis
-impaired filling although myocardium is normal
Symptoms of constrictive pericarditis
- SOB
- cough
- fatigue
-RHF (oedema), ascites, high JVP, jaundice, hepatomegaly, AF, TR, pleural effusion, perickardal knock
Investigation + treatment of constrictive pericarditis
-ECHO
-right heart catherter
( to differentiate from restrictive cardiomyopathy)
-limited diuretic and pericardectomy
