Cardiomyopathy and Myocarditis Flashcards
(19 cards)
Cardiomyopathy
Definition
Literally means:
‘—-disease’
Cardio ∙ myo ∙ pathy
Without qualification, it is not a —
Many types of heart— /— result in ‘‘cardiomyopathy’
A —/—-abnormality of heart muscle, ie. the—
Strictly does not include — heart disease BUT…..
Uncommon but devastating disease that can occur in — people.
heart muscle
diagnosis
disease/injury
primary/intrisic
myocardium
ishcmeic
young
3 major clinicopathologic groups
— [CMD]
— [CMH]
—
- Some — can lead to mixed types
Others
Selective right ventricular —
[ARVD]
Ventricular Fibrillation [ — Syndrome]
—
Long – [Romano-Ward]
dilated
hypertrophic
restricitve
mutation
arrthomogenic
brugada
metabolic
long QT
Dilated Cardiomyopathy (CMD)
Primary
— cardiac — & subsequent —
— onset —
Four chamber —
& — , often of— cause
Aetiology
Most 2° — !
25% —
Impairment of cardiac muscle — production or
transmission
Disturbance of myocellular
—- metabolism
Listed CMD1A-Z followed by
CMD1AA – CMD 1NN etc
progressive
hypertrophy
dilation
gradual
heart failure
hypertrophy and dilation
unknown
ischaemia
genetics
force
ca++
Dilated Cardiomyopathy [CMD]
Acquired
-Possible pathological myocardial insults:
—
Previous — – e.g. coxsackie—
— – e.g. —
— reaction
- —- in Genes known to cause
Genetic Cardiomyopathy may pre-dispose to — disease
alchohol
myocarditis
virus b
drugs eg, chemotherapy
immunologic
polymorphism
aquired
Dilated Cardiomyopathy [CMD]
Early:
Impaired —
End stage
Ejection Fraction of —
that is EF and what is the normal EF:
cardiac output = HR X SV ( L/min)
stroke volume = end diastolic volume - end systolic volume
- factors include: preload/afterload/contractility
- ejection fraction = — / — for example 70/120 = 58% ( >/ =50%)
- HF with reduced or preserved EF
left ventricular contractility
25%
stroke volume / end diastolic volume
Dilated Cardiomyopathy [CMD]
— common form of cardiomyopathy (— % of
cases)
Occurs at – age, most common age — yrs
— developing —
May be — or – [ —]
— > —
Fundamental defect is ineffective — → poor cardiac output
50% of patients die < 2 years
25% survive > than 5 years
Death → progressive — , or
complications such as —
— frequently necessary
most
90%
any
20-60
slowly
heart failure
sporadic or familial ( genetic )
men > women
ventricular contraction
cardiac failure
arrythmias
heart transpantation
Dilated Cardiomyopathy [CMD]
Pathology
Heart - — , – & — , — (Poor— )
— of all 4 chambers
Weight exceeding — What is
normal ?
— of the ventricular wall
—- are common – risk
of emboli Why?
Functional —-
Why?
—are usually not obstructed
Dilated Cardiomyopathy [CMD]
Pathology - Microscopic
Individual cardiac muscle
cells vary in size
Myocyte –
Interstitial —
Scant mononuclear — infiltrate
heavy flabby large hyopcotractile
poor pump
dilatation
900g
thining
mural thrombi
mitral regurgitation
coronary arteries
hypertrophy
fibrosis
inflammatory
Hypertrophic Cardiomyopathy (CMH)
Hypertrophic Obstructive (HOCM)
Myocardial hypertrophy
Abnormal —
Intermittent — obstruction
In contrast to DCM, powerful — contractions that rapidly excel blood from ventricles
But, – walled ventricles → diastolic filling impaired
Remember SV = EDV-ESV
diastolic filling
venticular outflow
hyperkinetic
stiff
Hypertrophic Cardiomyopathy [CMH]
Aetiology
— in 50% of cases (—- trait)
Mutations in genes encoding
— proteins
Prognosis varies with the — defect
Related to inefficiency of —
utilization
Reduced ATP: Interferes with
— reuptake
Triggers Ca++ dependent hypertrophy
& arrhythmia
Genes involved in
Sarcomeric contractile proteins
Z-disk proteins
Calcium-induced calcium release
ATP generation systems
Membrane & Basal lamina components
e.g. Duchenne MD
Mitochondrial Function
>25 CMH genetic Subtypes
familial
autosomal dominant
sactomeric contractile
genetic
atp
ca++
Hypertrophic Cardiomyopathy [CMH]
Clinical:
—
–> Myocardial — common, without coronary artery disease – why ?
Remember demand & supply see IHD lecture
—– ventricular
—- sudden
-> — especially
angina
ishcarmia
arrythmia
death
athletes
Hypertrophic Cardiomyopathy [CMH]
Pathology - Gross:
Massive myocardial —
Disproportionate— thickening
—obstruction
Pathology – Gross & Microscopic:
Heart weight excess —
—may also be dilated
Extensive myocyte —
Most prominent in the — and –
hypertrophy
ventricular septal
LV outflow
800g
left atrium
hypertrophy
left ventricle &
interventricular septum
Restrictive Cardiomyopathy (RCM)
Pathophysiology:
Primary decrease in — which leads to:
— impeded
Impaired — during –
— function unaffected
Ventricles are of – size
Remember SV = ESV - ESV
– common than DCM, HCM
Majority patients >–yrs
ventiucalr compliance
diastolic relaxation
LV ventricular filling
diastole
LV systolic
normal
less
>60
Restrictive Cardiomyopathy (RCM)
Caused by any process that reduces — :
Non-infiltrative:
—
— ( – factors less well defined)
Infiltrative:
—
—
— tumour
Storage
Haemochromatosis
Radiation —
myocardial compliance
idiopathic
familial
genetic
amyloidosis
sarcoidosis
metastic
fibrosis
Restrictive Cardiomyopathy (CMR)
Pathology Gross & Microscopic:
Stiff thickened —
Massive L —
Endo-myocardium replaced by — material
–> Pink and exhibits apple-
green birefringence when
stained with Congo – Red
and examined under
polarised light to indicate
presence of Amyloid
–> Heart stained with PERL’s stain to show massive Iron
accumulation in Heart –
appearances of
Haemochromatosis
LV wall
atrial dilatation
amorphous
check stains slide 20,21
Myocarditis
— Inflammation of the – that results in – to cardiac –
Does not include inflammation post —
Outcome (as with any inflammatory process):
Process – or becomes – with — [if not dead in
acute phase !]
Clinically:
Variable → depending on acute— and—
Mild – / – changes
— - common
— onset heart failure
– cardiac death
primary
myocardial
injury
cardiac myocyte
post infract
resolves
chronic w fibrosis
acute state and cause
mild fever ad ECG changes
arrhythmia
delayed
sudden
Myocarditis
Causes:
1- — :
Viruses – CMV, Coxsackie,
Bacteria - Neisseria
Fungi - Candida
Protozoa – Toxoplasmosis
2- — mediated:
Post – Infectious
viral
streptococcal
SLE
Drug hypersensitivity – eg. chemo
Transplant rejection
3- — but probably immune
Sarcoidosis
Giant cell myocarditis
4- —-
5- —
infection
immune
unknown
idiopathic
radiation
SARS-CoV2 - ALL NEW
More evidence needed especially from autopsy studies
Direct v Indirect Injury ?
So far
* Myocardial infiltration by macrophages & CD4+ T
lymphocytes
* Myocyte damage & lymphocytic myocarditis
* SARSCoV-2 viral particles identified in cardiac
macrophages
* Long term consequence awaited – Troponin –
Cardiac MRI
* Consequence of Therapy in a few ??
- myocarditis and COVID-19:
SARS-CoV2 - ALL NEW
More evidence needed especially from
autopsy studies
Direct v Indirect Injury ?
So far
* Early Shock State
* Myocardial infiltration by macrophages &
CD4+ T lymphocytes
* Micro-thrombosis – small coronary vessels
* SARSCoV-2 viral particles identified in cardiac
macrophages
* Long term consequence awaited – Troponin –
Cardiac MRI
* Consequence of Therapy in a few ??
* Cardiac dilatation & spotty necrosis
* Consider Takotsubo cardiomyopathy [apical
left ventricular ballooning & MRI
demonstrating lack of myocardial
enhancement (thus excluding viral
myocarditis)
Myocarditis
Pathology determined by –
Acute — myocarditis
Myocardium oedematous
Lymphocytic infiltrate, mononuclear
cells
Non ischaemic necrosis
Viral inclusion rarely may be seen
(eg. In CMV)
Later in the disease:
Myocardial –
Ventricular —
— :
May see neutrophils, abscess
— myocarditis:
Multinucleate giant cells prominent
cause
viral
fibrosis
dilation
bacterial
giant cell
conclusion :
( Take Home Message
Genetics Critical especially in Sudden Adult Death Syndrome)
Cardiomyopathy - Sudden death or cardiac failure
Dilated
Hypertrophic
Restrictive
etc
Myocarditis
Remember all the I’s — Infective, Inflammatory, Ischaemic, Immune,
Irritant, Iatrogenic, Idiopathic
Outcome Resolution or Chronic cardiac failure due to healing by
fibrosis
