Cardiomyopathy, Myocarditis and pericarditis Flashcards
dilated cardiomyopathy
heart condition where the ventricular function is impaired
- Can be a primary problem or the end result of almost any pathological insult to the myocardium
Causes of dilated cardiomyopathy?
ischaemia
valvular
genetic + familial - muscular dystrophy
inflammatory, infectious, autoimmune, postpartum
toxic:- drugs, exogenous chemicals, endocrine
injury, cell loss, scar replacement
which gene commonly causes cardio myopathy if altered
SCN5A gene
which chambers of the heart can dilated myocardiopathy affect/
Both L and R ventricles mainly but can also affect atria
Can be one chamber but most often all chambers become dilated and functionally impaired
So systolic and diastolic dysfunction can occur
thrombosis in chambers is not uncommon
when is flow slower through a ventricle?
when it is dysfunctional/ not contracting properly
Reversible causes of dilated cardiomyopathy (6)
Alcohol endocrine tropical disease post partum - after having a baby - often fixes itself haemaochromatosis sarcoid
is DCM common?
no
incidence = 5-8 per 100000
symptoms of DCM
Progressive slow onset dyspnoea fatigue orthopnoea PND - paroxysmal nocturna dyspnoea ankle swelling weight gain of fluid overload cough
what are you likely to find on examination of a patient with DCM? (10)
Poor superficial perfusion
thready pulse, irreg if in AF
SOB at rest
narrow pulse pressure
JVP elevated+/- TR waves
displaced apex
S3 and S4
MR murmur often
pulmonary, sacral and ankle oedema pleural effusions
acites - protein containing fluid in abdomen
hepatomegally
Basic investigations to carry out for DCM diagnosis? (7)
ECG - almost always abnormal
CXR - fluid overload, pleural effusion
ECHO - simplest, least invasive
basic bloods - FBC
CMRI - best imaging modality - show scar burden in myocardium and maybe see thrombus
coronary angiogram
sometimes a biopsy depending if caught at early stage or not
general treatment of DCM
Correct anaemia
Remove exacerbating drugs eg NSAIDs - these retain salt/water so not good if already have fluid overload
Correct any endocrine disturbance
Advise on fluid and salt intake, reduce it
Advise on managing weight to identify fluid overload
HF nurse referral
specific treatment of DCM
- ACE Inhibitors, - Angiotensin II blockers - diuretics
- Beta blockers
Spironolactone - Anticoagulants as required
- Sudden Cardiac Death risk assessment with ICD or CRT-D/P implant
- Cardiac transplant – last resort, if everything else fails
prognosis of DCM? which cause of DCM shows worst outcome
generally poor and often influenced by the causes
Cardiomyopathy due to HIV
restrictive and infiltrative cardiomyopathy - what is it? prevalence?
not a high prevalence - cardiologist might see 1 a year?
heart is restricted - has to stretch to accommodate blood and increased venous return - ventricle can’t fill well due to reduced compliance
impairment of diastolic function
about 50% related to specific clinical conditions, the rest remain unknown
2 causes of infiltrative cardiomyopathy?
sarcoid - inflammation of organs
amyloid - protein collection in tissues/organs
storage diseases that can cause restrictive and infiltrative cardiomyopathy
haemachromatosis
Fabry’s disease - glycogen storage disease
endomyocardial causes of restrictive and infiltrative cardiomyopathy?
Fibrosis, carcinoid, radiation, drug effects
is there increased or reduced compliance in Restrictive and infiltrative cardiomyopathy?
reduced
is relaxation of the ventricular wall an active or passive process?
active
requires functioning intact myocytes
investigations carried out for restrictive and infiltrative cardiomyopathy (10)
repeated ECG - note if there is Left bundle branch block (LBBB) -cardiac conduction abnormality seen on ECG. In this condition, activation of the left ventricle of the heart is delayed, which causes the left ventricle to contract later than the right ventricle.
CXR
BNP - increased in heart failure
Basic bloods FBC, U+E, be on the look out for sarcoid and haemachromatosis
Auto antibodies for sclerotic CT diseases
Amyloid needs non cardiac biopsy to help establish the diagnosis
Fabry; low plasma alpha galactosidase A activity
Echo
CMRI, probably best imaging modality
Biopsy more helpful but still has high false negative rate
what are some specific measures put in place for restrictive and infiltrative cardiomyopathy
Limited diuretic use as low filling pressures will cause problems
Beta blockers limited ACE Inhibitor use
Anticoagulants as required
Sudden Cardiac Death risk assessment with Implantable cardiac defibrillator or Cardiac resynchronization therapy pacemaker implant
Cardiac transplant
If iron overload, specific forms of amyloid or Fabrys then specific treatments are available
Endomyocardial fibrosis has little specific treatment
hypertrophic cardiomyopathy:- what is it? prevalence? genetic basis?
thickened heart muscle
impaired relaxation is a common feature. Ventricles can’t hold much blood, and the walls can’t relax properly and may stiffen. Behaves in restrictive manner
relatively high prevalence 1:500
over 1500 genes now identified - sarcomere gene defect
autosomal dominant but gene can be expressed differently and not known how until time passes
what parts of the heart thicken in hypertophic cardiomyopathy?
doesn’t have to be whole heart, can be part of it
Can be apical, septal or generalised
if septal hypertrophy what can an additional defect in the mitral valve lead to?
Left ventricular outflow tract obstruction
common further heart/vessel problems associated with hypertrophic cardiomyopathy
Coronary arteries also affected with small vessel narrowing and consequent ischaemia and fibrosis
arrhythmias are common
symptoms of hypertrophic cardiomyopathy
Breathless, palpitations, syncope, exertional symptoms, Sudden Cardiac Death
Asymptomatic for many fatigue dyspnoea anginal like chest pain exertional pre syncope syncope related to arrhythmias or LVOT obstruction
examination findings:- hypertrophic cardiomyopathy
Can be none!
Notched pulse pattern
Irreg pulse if in AF or ectopy
Double impulse over apex, thrills and murmurs, often dynamic, LVOT murmur will increase with valsalve and decrease with squatting
JVP can be raised in very restrictive filling
investigations/tests for hypertrophic cardiomyopathy?
ECG - often abnormal but a few are normal where phenotype is poorly expressed in genotype +ve individuals
Echo - see thickness in muscle and dynamic effects of blood flow
CMRI
Risk stratification for Sudden Cardiac Death, may need ICD Holters repeatedly (monitors heart rate)
general measures suggested for hypertrophic cardiomyopathy
Avoid heavy exercise
Avoid dehydration
Explore FH and first degree relatives, ECGs and echoes may be required
Consider genetic testing
Regular follow up to re appraise the risks and progress
specific measures suggested for hypertrophic cardiomyopathy
Drugs to try and enhance relaxation, variable results but often if symptomatic, beta blockers, verapamil, disopyrimide
If in AF anticoagulate
Obstructive form; surgical or alcohol septal ablation
ICD if required based on risk stratification
myocarditis:- what is it? acute/chronic? prevalence?
can be acute or chronic
inflammation of the myocardium
canimpair myocardial function, conduction and generate arrhythmia
8-10 100000
most common is viral
common viruses causing myocarditis?
adenovirus parvovirus B19 influenza hep C epstein barr virus
pathology of myocarditis?
Infiltration of inflammatory cells into the myocardial layers, reduced function and heart failure, heart block as conduction system is involved and arrhythmias
symptoms of myocarditis
Heart failure with fatigue, SOB, Chest Pain in only 26%
Shorter course of a few weeks
May not have fever
Signs of Heart Failure
Expected investigation/test outcomes for myocarditis?
ECG usually abnormal
Biomarkers often elevated but not falling in a pattern consistent with MI
Echo, can get RWMA (regional wall motion abnormalities - LV wall motion abnormalities usually)
CMRI can see oedema in certain images
Low threshold for biopsy
Viral DNA PCR Auto antibodies Step antibodies Lyme B burgdorferi HIV
general measures/management for myocarditis
Supportive with treatment of heart failure and support for brady and tachy arrhythmias
Immunotherpay if biopsy or other Ix point to a specific diagnosis
Stop possible drugs or toxic agent exposure
20% mortality at 1 year and 56% by 4 years.
pericarditis
Inflammation of the pericardial layers with or without myocardial involvement
causes of pericarditis (10)
idiopathic viral - infectious bacterial post MI perforation dissection of proximal aorta neoplasia - presence or formation of new, abnormal growth of tissue immune-inflammatory trauma congenital
symptoms of pericardial disease
Usually 1-2/52 week duration
chest pain with pleuritic features and postural features, sitting forward usually improves it lying back makes it worse
Fever
signs of pericardial disease
Raised temp
pericardial rub Left Sternal Edge
look for JVP as if an effusion is present and substantial or haemodynamically relevant then it will be raised
low BP
muffled HS and raised JVP should make you consider not just pericarditis but effusion
High fever and very unwell despite no effusion may suggest bacterial
investigations for pericardial disease
ECG and echo,
troponin (protein in muscle fibres) may be raised if myocardial involvement too (myocardial injury)
general measures/management for pericardial disease:- viral, idiopathic and bacterial
Viral is conservative - non invasive treatment
idiopathic gets colchicine and limited use of NSAIDs
Bacterial must be drained even if small effusion and antimicrobials, high death rate
If large effusion present and some haemodynamic effects then drain
what signs and symptoms would a patient present with if tamponade was present
signs:- raised JVP, low BP, +/- rub or muffled HS. Large drop in stroke vol, systolic BP and pulse wave
amplitude during inspiration (pulsus paradoxus)
symptoms:- fatige, SOB, dizzy with low BP, occasionally chest pain.
tests/investigations:- tamponade
urgent echo
CXR - show large cardiac shadow
drainage - treatment
mechanical circulatory support
what does persistant effusion need
a surgical pericardial window made to allow flow to abdomen
constrictive pericarditis
rare, impaired filling although myocardium is normal most of the time
causes: - idiopathic, radiation, post-op, autoimmune, renal failure, sarcoid
symptoms: - fatigue, SOB, cough
signs: - more of RHF with oedema, ascites, high JVP, jaundice, hapetomegally, AF, TR, pleural effusion
investigations: - echo, RH catheter to differentiate from restrictive cardiomyopathy - v difficult
treatment: - careful and limited diuretics and pericardectomy
