Cardiomyopathy, Restrictive Cardiomyopathy, RCM

Buzz

- LV small
  - may be LVH
- LA large
- decrease in the ventricular filling
- EF maintained

Compared to Constrictive Disease

- contours of the ventricular cavities are
  maintained (do not appear indented- like in constriction)
- The degree of increase in atrial size is much greater in the RCM than in constrictive pericarditis (CP)
- myocardial thickness
  is frequently increased in the RCM but normal in the CP
- the pericardial thickness is usually normal, having a normal value
  of 2 mm or less

Definition

Restrictive cardiomyopathy, is defined as abnormal diastolic function in association with relatively well-preserved systolic function (at least in the early stages of the disease).

“heart muscle is structurally and functionally abnormal in the absence of coronary artery disease, arterial systemic hypertension, valvular disease, or congenital heart disease sufficient to cause the observed myocardial abnormality.^1″

Functionally hemodynamic picture strongly resembles constrictive pericarditis.

Precipitous rise of LV pressure with only small increases in volume. On cardiac catheterization, this phenomenon is characterized by a dip-and-plateau contour of early diastolic pressure traces.

RCM are usually characterized by normal or small LV cavity size (<40 mL/m²)

Types

Amyloidosis

Sarcoidosis

Loefflers

Iron Overload

Hypertrophic Cardiomyopathy

Chloroquin Induced Cardiomyopathy

Basically

infiltrative or storage diseases (e.g. amyloidosis and glycogen storage disease);

obliterative or endomyocardial diseases [e.g. endomyocardial fibrosis (EMF), related or not to hypereosinophilia].

Cause	Familial/ genetic	Non-familial/ non-genetic
Apparently Idiopathic
?Genetic origin	X
?Unknown origin		X
Amyloidosis
?AL/prealbumin		X
?Genetic (e.g. TTR)	X
?Senile		X
Other infiltrative diseases (such as Gaucher?s disease, Hurler?s disease)	X
Inflammatory cardiomyopathies with a restrictive haemodynamic component: Sarcoidosis, SSc		X
Storage diseases
?Haemochromatosis	X
?Fabry disease	X
?Glycogen storage disease	X
Pseudoxanthoma elasticum	X
Radiation therapy		X
Drugs		X
Endomyocardial diseases (with or without hypereosinophilia, carcinoid disease, drug induced)	X (rare)	X (frequent)
Miscellaneous (radiation, drug-induced, e.g. antracycline toxicity, serotonin, methysergide, ergotamine, mercurial agents, and busulfan)		X

RCM, restrictive cardiomyopathy; TTR, transthyretin; SSc, systemic sclerosis.

References and Links

Zuluaga, A et al Restrictive Cardiomyopathies:Evaluation Using Cardiac MRI and Multidetector CT

TCV
- Cardiomyopathy Map

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Cause
Familial/ genetic
Non-familial/ non-genetic




Apparently Idiopathic


?Genetic origin
X



?Unknown origin

X


Amyloidosis




?AL/prealbumin

X


?Genetic (e.g. TTR)
X



?Senile

X


Other infiltrative diseases (such as Gaucher?s disease, Hurler?s disease)
X



Inflammatory cardiomyopathies with a restrictive haemodynamic component: Sarcoidosis, SSc

X


Storage diseases




?Haemochromatosis
X



?Fabry disease
X



?Glycogen storage disease
X



Pseudoxanthoma elasticum
X



Radiation therapy

X


Drugs

X


Endomyocardial diseases (with or without hypereosinophilia, carcinoid disease, drug induced)
X (rare)
X (frequent)


Miscellaneous (radiation, drug-induced, e.g. antracycline toxicity, serotonin, methysergide, ergotamine, mercurial agents, and busulfan)

X



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Cause
Familial/ genetic
Non-familial/ non-genetic




Apparently Idiopathic


?Genetic origin
X



?Unknown origin

X


Amyloidosis




?AL/prealbumin

X


?Genetic (e.g. TTR)
X



?Senile

X


Other infiltrative diseases (such as Gaucher?s disease, Hurler?s disease)
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Inflammatory cardiomyopathies with a restrictive haemodynamic component: Sarcoidosis, SSc

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Storage diseases




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?Fabry disease
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?Glycogen storage disease
X



Pseudoxanthoma elasticum
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Radiation therapy

X


Drugs

X


Endomyocardial diseases (with or without hypereosinophilia, carcinoid disease, drug induced)
X (rare)
X (frequent)


Miscellaneous (radiation, drug-induced, e.g. antracycline toxicity, serotonin, methysergide, ergotamine, mercurial agents, and busulfan)

X



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    [prefix] => 
    [localName] => td
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DOMElement Object
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    [nodeValue] => Storage diseases
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    [textContent] => Storage diseases
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DOMElement Object
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DOMElement Object
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DOMElement Object
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DOMElement Object
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DOMElement Object
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    [prefix] => 
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DOMElement Object
(
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    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => Other infiltrative diseases (such as Gaucher?s disease, Hurler?s disease)
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DOMElement Object
(
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    [nodeValue] => X
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    [namespaceURI] => 
    [prefix] => 
    [localName] => td
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DOMElement Object
(
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    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => 
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DOMElement Object
(
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    [nodeValue] => ?Senile
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    [ownerDocument] => (object value omitted)
    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => ?Senile
)

DOMElement Object
(
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    [tagName] => td
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    [childElementCount] => 0
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    [nodeName] => td
    [nodeValue] => 
    [nodeType] => 1
    [parentNode] => (object value omitted)
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    [lastChild] => 
    [previousSibling] => (object value omitted)
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    [attributes] => (object value omitted)
    [ownerDocument] => (object value omitted)
    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => 
)

DOMElement Object
(
    [schemaTypeInfo] => 
    [tagName] => td
    [firstElementChild] => 
    [lastElementChild] => 
    [childElementCount] => 0
    [previousElementSibling] => (object value omitted)
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    [nodeName] => td
    [nodeValue] => X
    [nodeType] => 1
    [parentNode] => (object value omitted)
    [childNodes] => (object value omitted)
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    [lastChild] => (object value omitted)
    [previousSibling] => (object value omitted)
    [nextSibling] => (object value omitted)
    [attributes] => (object value omitted)
    [ownerDocument] => (object value omitted)
    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => X
)

DOMElement Object
(
    [schemaTypeInfo] => 
    [tagName] => td
    [firstElementChild] => 
    [lastElementChild] => 
    [childElementCount] => 0
    [previousElementSibling] => 
    [nextElementSibling] => (object value omitted)
    [nodeName] => td
    [nodeValue] => ?Genetic (e.g. TTR)
    [nodeType] => 1
    [parentNode] => (object value omitted)
    [childNodes] => (object value omitted)
    [firstChild] => (object value omitted)
    [lastChild] => (object value omitted)
    [previousSibling] => (object value omitted)
    [nextSibling] => (object value omitted)
    [attributes] => (object value omitted)
    [ownerDocument] => (object value omitted)
    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => ?Genetic (e.g. TTR)
)

DOMElement Object
(
    [schemaTypeInfo] => 
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    [lastElementChild] => 
    [childElementCount] => 0
    [previousElementSibling] => (object value omitted)
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    [nodeName] => td
    [nodeValue] => X
    [nodeType] => 1
    [parentNode] => (object value omitted)
    [childNodes] => (object value omitted)
    [firstChild] => (object value omitted)
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    [previousSibling] => (object value omitted)
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    [prefix] => 
    [localName] => td
    [baseURI] => 
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DOMElement Object
(
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    [nodeValue] => 
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    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
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)

DOMElement Object
(
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    [nodeValue] => ?AL/prealbumin
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    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => ?AL/prealbumin
)

DOMElement Object
(
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    [prefix] => 
    [localName] => td
    [baseURI] => 
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)

DOMElement Object
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    [attributes] => (object value omitted)
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    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
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)

DOMElement Object
(
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    [nodeValue] => Amyloidosis
    [nodeType] => 1
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    [lastChild] => (object value omitted)
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    [ownerDocument] => (object value omitted)
    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => Amyloidosis
)

DOMElement Object
(
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    [tagName] => td
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    [lastElementChild] => 
    [childElementCount] => 0
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    [nodeName] => td
    [nodeValue] => X
    [nodeType] => 1
    [parentNode] => (object value omitted)
    [childNodes] => (object value omitted)
    [firstChild] => (object value omitted)
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    [previousSibling] => (object value omitted)
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    [attributes] => (object value omitted)
    [ownerDocument] => (object value omitted)
    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => X
)

DOMElement Object
(
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    [tagName] => td
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    [childElementCount] => 0
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    [nodeName] => td
    [nodeValue] => 
    [nodeType] => 1
    [parentNode] => (object value omitted)
    [childNodes] => (object value omitted)
    [firstChild] => 
    [lastChild] => 
    [previousSibling] => (object value omitted)
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    [attributes] => (object value omitted)
    [ownerDocument] => (object value omitted)
    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => 
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DOMElement Object
(
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    [lastElementChild] => 
    [childElementCount] => 0
    [previousElementSibling] => 
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    [nodeValue] => ?Unknown origin
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    [childNodes] => (object value omitted)
    [firstChild] => (object value omitted)
    [lastChild] => (object value omitted)
    [previousSibling] => (object value omitted)
    [nextSibling] => (object value omitted)
    [attributes] => (object value omitted)
    [ownerDocument] => (object value omitted)
    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => ?Unknown origin
)

DOMElement Object
(
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    [tagName] => td
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    [lastElementChild] => 
    [childElementCount] => 0
    [previousElementSibling] => (object value omitted)
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    [nodeName] => td
    [nodeValue] => 
    [nodeType] => 1
    [parentNode] => (object value omitted)
    [childNodes] => (object value omitted)
    [firstChild] => 
    [lastChild] => 
    [previousSibling] => (object value omitted)
    [nextSibling] => (object value omitted)
    [attributes] => (object value omitted)
    [ownerDocument] => (object value omitted)
    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => 
)

DOMElement Object
(
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    [firstElementChild] => 
    [lastElementChild] => 
    [childElementCount] => 0
    [previousElementSibling] => (object value omitted)
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    [nodeName] => td
    [nodeValue] => X
    [nodeType] => 1
    [parentNode] => (object value omitted)
    [childNodes] => (object value omitted)
    [firstChild] => (object value omitted)
    [lastChild] => (object value omitted)
    [previousSibling] => (object value omitted)
    [nextSibling] => (object value omitted)
    [attributes] => (object value omitted)
    [ownerDocument] => (object value omitted)
    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => X
)

DOMElement Object
(
    [schemaTypeInfo] => 
    [tagName] => td
    [firstElementChild] => 
    [lastElementChild] => 
    [childElementCount] => 0
    [previousElementSibling] => 
    [nextElementSibling] => (object value omitted)
    [nodeName] => td
    [nodeValue] => ?Genetic origin
    [nodeType] => 1
    [parentNode] => (object value omitted)
    [childNodes] => (object value omitted)
    [firstChild] => (object value omitted)
    [lastChild] => (object value omitted)
    [previousSibling] => (object value omitted)
    [nextSibling] => (object value omitted)
    [attributes] => (object value omitted)
    [ownerDocument] => (object value omitted)
    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => ?Genetic origin
)

DOMElement Object
(
    [schemaTypeInfo] => 
    [tagName] => td
    [firstElementChild] => 
    [lastElementChild] => 
    [childElementCount] => 0
    [previousElementSibling] => 
    [nextElementSibling] => 
    [nodeName] => td
    [nodeValue] => Apparently Idiopathic
    [nodeType] => 1
    [parentNode] => (object value omitted)
    [childNodes] => (object value omitted)
    [firstChild] => (object value omitted)
    [lastChild] => (object value omitted)
    [previousSibling] => (object value omitted)
    [nextSibling] => (object value omitted)
    [attributes] => (object value omitted)
    [ownerDocument] => (object value omitted)
    [namespaceURI] => 
    [prefix] => 
    [localName] => td
    [baseURI] => 
    [textContent] => Apparently Idiopathic
)

Buzz

Compared to Constrictive Disease

References and Links

TCV