C17: Restrictive and Other cardiomyopathies Flashcards
another name for restrictive cardiomyopathy (RCMO)
infiltrative CMO
define RCMO
referes to diseases other than ischemic heart disease which cause increased resistance to Lv filling due to increased myocardial stiffness
what is RCMO associated w/
increased LV end-diastolic pressure
increased mean LA pressure
can systolic fxn be preserved w/ RCMO
in some cases
main cause of RCMO
abnormal infiltration, storage or fibrosis w/in the myocardium
classifications of RCMO
non-infiltrative
infiltrative
storage disease
endomyocardial
examples of non-infiltrative causes of RCMO
idiopathic familial hypertrophic CMO - separate class but can cause restrictive physiology so could fit here too scleroderma diabetic CMO (fibrosis)
examples of infiltrative causes of RCMO
amyloidosis sarcoidosis hurlers disease loefflers disease (WBCs deposited)
examples of storage disease causes of RCMO
hemochromatosis (iron storage disease)
fabry disease (fat deposits)
GSD
examples of endomyocardial causes of RCMO
endomyocardial fibroelastosis
carcinoid
radiation
chemo
describe infiltrative RCMO
disease of the myocardium secondary to a disease that produces histologic changes to the myocardium by depositing substances inside or in/b/w muscle cells… usually due to genetic or metabolic disorder
can infiltrative RCMO lead to restrictive filling patterns
why
yes
material deposited restricts LV filling by making it stiffer
does non-infiltrative disorders cause abnormal myocardium and increased stiffness
yes
clinical findings of RCMO
diastolic dysfunction and backwards heart failure:
fatigue, edema, SOBOE (due to increased LVEDP + LAP)
mod P HTN
RCMO can be hard to distinguish from which other pathology of the pericardium
constrictive pericarditis
in early stages of RCMO, what is systolic FXN like
later?
may be normal
reduced
which infiltrative disease doesn’t affect the LV
ARVD - arrhythmogenic RV dysplasia
RCMO 2D findings
usually normal LV cavity size
increased wall thickness
biatrial enlargement
sm pericardial effusion is common
RCMO on ECG
atrial and ventricular arrhythmias (afib common)
low voltage QRS
conduction defects
RCMO on chest Xray
cardiomegaly
pulmonary congestion/effusion
RCMO on cardiac MRI
may see the extent of the infiltration, better tissue characterization than echo
medical treatment for RCMO
treat underlying etiology diuretics ACE inhibitors anticoagulants anti-arrhythmics
surgical treatment for RCMO
limited…
pacemaker/defib
heart transplant
most common type of infiltrative RCMO
amyloidosis
describe amyloidosis
deposits of amyloid protein into the extracellular space b/w monocytes… produces a stiff myocardium and prevents effective filing
what is amyloidosis often misdiagnosed as in early stages
LVH due to HTN
what is needed to diagnose amyloidosis
cardiac biopsy and pathological evaluation for a definitive diagnosis….
what organs does amyloidosis affect
heart, liver, kidneys, spleen, skin
4 types of amyloidosis
describe them
primary/idiopathic - more common in males
secondary - associated w/ other inflammatory disorders
familial (hereditary)
senile - common in males over 70
amyloidosis on the ECG
- low voltage on ECG from myocardial damage
- pseudo-infarction pattern - abnormal Q waves due to myocardial fibrosis
- a fib
- ventricular arrhythmias
- conduction defects (LBBB)
what is a very important and sensitive sign for amyloidosis on 2D and ECG
LVH on 2D and low voltage on ECG
medical treatment for amyloidosis
digitalis, digoxin (slow HR)
diuretics
vasodilators (to decrease afterload)
anticoagulants
surgical treatment for amyloidosis
bone marrow stem cell transplant pacemaker or defib heart transplant (poor long term results)
echo 2D features of amyloidosis
granular/speckled appearance of the myocardium concentric LVH and RVH wall motion abnormalities in late stages asymmetrical septal thickening mild-mod biatrial enlargement pericardial effusion evidence of P HTN
how does all thickness in amyloidosis correlate w/ Diastolic Dysfunction
thicker = more severe DD
IVS or LVPW thickness of what value will result in restrictive filling patterns in most patients
> 15 mm
w/ amyloidosis, s prime of the LV will often be what value
< 5 cm/s
LV systolic FX w/ early amyloidosis
late?
early: often normal
late: LV may dilate and EF will be reduced
late stages of amyloidosis are indistinguishable from which other cardiomyopathy
DCM, b/c the thick LV muscle starts to thin and the LV dilates…. LA will also dilate
can HCM and amyloid coexist in the same patient
yes
define sarcoidosis
multiple granulomatous disease which involves the heart in 25% of patients
sarcoidosis is more common in which gender
female
which organs do the granulomas infiltrate w/ sarcoidosis
lungs (most common) lymph nodes liver spleen skin parotid glands heart
clinical features of sarcoidosis
progressive heart failure
diffuse fibrosis which leads to PHTN
sudden death
dysrhythmias, heart block (a lot of Av block due to infiltration of the septum)
which walls of the heart does sarcoidosis usually effect
basal inferolateral/lateral walls
how does the ECG occur w/ sarcoidosis
3rd degree AV block
ventricular dysrhythmias
pseudo-infarct patterns w/ prominent Q waves
Echo features of sarcoidosis
pericardial effusion
increased anterior wall RV thickness (> 5 mm)
pulmonary HTN
RV dysfunction due to Pulmonary artery hypertension
MR
TR
diastolic dysfunction
early US features of sarcoidosis
late
early: increased wall thickness
late: aneurysmal dilation of LV
segmental wall thinning
LV dilation and reduced EF
wall motion abnormalities
is sarcoidosis diagnosed by echo
no
what is hemochromatosis
iron storage disease affecting multiple organ systems that result in organ damage and malfunction
what is primary hemochromatosis
which gender does it affect more
genetic due to a metabolic syndrome
male (usually males >40 and women >50)
what is secondary hemochromatosis
iron overload due to repeated blood transfusion in patients w/ chronic anemia, prolonged hemodialysis or due to chronic liver disease
clinical findings of hemochromatosis
CHF cirrhosis of the liver impotence diabetes arthritis
how does hemochromatosis appear on the ECG
AF, low voltage, SVTs, atrioventricular conduction defects (AV block)
echo features of hemochromatosis
features relates to CMO
increased wall thickness
preserved systolic function
another name for endocardial syndromes
hypereosinophilic
what is an endomyocardial syndrome
causes
persistently elevated blood eosinophil counts which causes restriction due to:
Idiopathic
malignant
reactive
infectious (parasitic)
is it easy to see the endocardium w/ endomyocardial syndrome
yes
describe the evolution of endomyocardial syndrome
infiltration of eosinophils evolve to a thrombosis and cause scarring w/ risk of embolism
why does endomyocardial syndrome cause restriction
what effect does this have
due to fibrotic tissue that lines the myocardium
decrease in LV systolic function…. leading to CHF and death
clinical features of endomyocardial syndrome
SOB
neurological symptoms
what is needed to confirm the diagnosis of endomyocardial syndrome
biopsy
what is GSD
excessive storage of glycogen, hereditary cause
how does GSD affect the heart
- heart becomes enlarged and globular in shape
- thickened RV and LV and IVS w/ increased mass
other infiltrative cardiomyopathies
arrhythmogenic RV dysplasia
carcinoid heart disease
Fabrys disease
collagen disorders
describe arrhythmogenic RV dysplasia (ARVD)
how does it affect RV function and appearance
genetic condition w/ fibro-fatty infiltration of the RV
systolic and diastolic dysfunction
RV is dilated with larger trabeculations
can cause arrhythmias
doesnt affect the LV, its normal
another name for arrhythmogenic RV dysplasia (ARVD)
arrhythmogenic RV cardiomyopathy
describe LV non-compaction
how does it affect function
genetic condition w/ LV wall thickening and deep grooves b/w the trabeculations
hypokinetic
arrhythmias
and heart failure
which walls are affect w/ LV non-compaction
apex, mid lateral and mid inferior
describe the normal LV compaction process
occurs from epicardium to endocardium and base to apex… apex is last to compact
review chart at end of PP
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