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Posts Tagged ‘non dilated cardiomyopathy’

Human heart is a compact elastic organ .  We know elasticity is lost when it is stretched beyond a point.This is what happens in dilated  cardiomyopathy .When the heart muscle fibers stretch  too long from the baseline  it loses its ability to contract and relax   efficiently.In fact  , after a cut of point even if it comes the original length the elastic fibers are fractured and suffer from irreversible damage.

Among  the systolic vs diastolic dimension it is the diastolic dimension that becomes important in defining dilatation.

When do you say a ventricle is dilated?

  1. When the EDD (End diastolic dimension) > 60mm
  2. EDD > 56mm
  3. EDD > 10 % from baseline
  4. EDD > 25 %  from baseline

Any of the above can be right.

The normal human ventricle measures  between 35 to 55mm in diastole .

Currently accepted  definition for enlargement of heart is EDD  of 56 mm and above. Some believe  in a more strict criteria of 60mm.

Consider the following situation

A man with 35mm EDD   can increase 20mm ( ie 60 % )  from of his baseline  and still be  labeled as  normal LV  dimension ! . If the above patient  is  destined to develop dilated cardiomyopathy    his  heart  would  begin  its  final  journey  slowly but   surely ( from 35 mm  ! ) . So ,  according to current criteria  we can diagnose  DCM only after it travels the half way towards hell .   What a way to define DCM  ! Be cautious LV dimensions can fool  you  . . .

If the EF is low and symptoms develop early ,  one may recognise  the above  entity ( at least erroneously !) as non dilated cardiomyopathy or RCM.

If  the patient is relatively asymptomatic and   if we   overlook  the  baseline  LV parameters ,  we are likely  to miss  most of the early  DCMs

Final message

We need to stress the importance of baseline LV dimension in defining DCM  . It is proposed  from this  site ,  an increase of 25 %   and above from baseline  can be   included as an   additional  criteria  for  LV dilatation . This  could  help us understand   the early muscle dynamics in DCM.

Un-Answered questions

  1. How to diagnose  early DCM ?
  2. When does the EF begins to decline in DCM ?
  3. What is the relation between EDD and EF %?
  4. Is HF with preserved LV function ( previously called diastolic dysfunction ) is the earliest point in the natural history of  DCM
  5. Is there a overlap  between non dilated cardiomyopathy , RCM and early phase of DCM ?

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y

A classical restrictive cardiomyopathy . Both atria balloon out as they face stiff resistance from the ventricles

Restrictive cardiomyopathy(RCM) is a common form of myocardial disease which was difficult to recognise  in the past.It was also commonly confused with constrictive pericarditis. Today , one can easily recognise this entity.A simple clue is bi- atrial enlargement  with relatively normal ventricle size.The above case is a classical form of RCM.

In late stages of RCM ,both LV, RV begins to dilate and can mimic a dilated cardiomyopathy. Doppler filling pattern  , and tissue Doppler motion of mitral annulus are recently validated methods to identify RCM. Still ,  2D features  are  very useful .This implies , the anatomical changes in the chamber size  are as important as physiological AV filling profiles. It is generally believed , physiological impairment precedes anatomical defects.( But not proven concept yet ! )

Note: The ventricles are not dilated and retain good systolic function

In the above patient  , etiology could not be confirmed and was labeled as idiopathic RCM  as tests for amyloid and eosinophilic infiltrations were negative.

M -Mode of the same patient confirms good systolic function

One would  call  for doppler mitral filling profile here ,  to confirm restrictive physiology ( A short DT , Short IVRT  . A reversal in  pulmonary veins , E/E’ ratio etc etc ) But all these are redundant here.

How is RCM different from non dilated cardiomyopathy ?

A new entity is being recognised in the cardiac  muscle disease.This is often referred to as NDCM (Non dilated cardiomyopathy)  .Global  systolic LV dysfunction  with normal  LV dimension.This a similar to the terminology MDCM (Minimally dilated  cardiomyopathy  where LV dimension increases  not more than  15 % of basal size ) .

This is seen in CKD and diabetic individuals.Atria may be enlarged .Diastolic dysfunction may co exist.  It is no surprise,  this entity closely mimics RCM. But in RCM LV systolic function  is not greatly compromised till late stages , while NDCM it begins with systolic dysfunction. This is the only difference .There can be overlaps .

Many strongly believe ,  both  RCM  and NDCM are one and the same entities ,  that present in different time frames in their natural history.

Final message

Diagnosing RCM is no longer difficult in established cases* . The message from  this article is , 2 D echo can  strongly suggest  the possibility of RCM (or even clinch it)  .  Never ever  diagnose RCM with normal 2 D echo. Doppler filling profiles are useful  additional tools . We  can not diagnose RCM with doppler features alone , but we can  be fairly certain about RCM when we encounter  typical bi-atrial enlargement and a normal LV by  2 D echo.

Caution : Patients  with longstanding atrial fibrillation of any cause , can dilate their atria and could mimic RCM .They can be some compromise in LV function due to chronic tachycardia .

* Recognizing RCM ,  very early in the course is still a problem . Here the  newer modalities like Phase  MRI, tissue doppler, speckle tracking, and velocity vector imaging may be useful.

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Dilated cardiomyopathy (DCM )  is the commonest  cardiac muscle disease .Diagnosis is simple and straight forward. All that we  require is,   documentation of LV dilatation and contractile dysfunction.The nomenclature of cardiomyopathy has remained a difficult exercise .This   is primarily  due to   iatrogenic  & intellectual confusion  among  cardiologists . They mixed up etiological and morphological entities together ,   later on  they  wanted to de-link  etiology from morphology  ,  ultimately they realised  when illness strikes the heart ,   it   can not differentiate the  morphology, etiology and pathology as we would want to   . So , whenever possible we have to label  cardiomyopathy with all components (Dilated cardiomyopathy due to alcohol with some restrictive features.)

This article  tries to evoke  some thoughts    about  why  LV dilatation  is central to the understanding of cardiomyopathy.

DCM is the prototype where LV dilates with global hypokinesia.The upper limit of   LV diameter is generally considered to be 56mm in diastole.  (Range 35 -56mm) .This cut off  point is too empirical  for the simple reason, the  left ventricle can dilate   up to  50 %  from it’s basal diameter and still technically  be within normal limits.( A 3.5 cm LV ( end diastolic diameter ) can dilate to 5.6 cm ,i e  a 50 % dilatation , still LV  has not reached the upper limit of normal  )

Even as we do not have a clear  answer to the above issue , we  recognise  left ventricle muscle can hypertrophy, progressively  dilate , transiently dilate, fail to  dilate ,  regressively  dilate  or  hypotrophy .These changes can be dynamic and heavily influenced by hemodynamic and local pathologic factors like fibrois, interstitial proliferation etc. Meanwhile , the pharmacological ,   surgical /catheter injuries we  inflict    , modifies  the muscle behavior in a positive or negative manner.

In this back ground ,  we have found a new entity called NDCM .

Apart from  DCM, a newer form of  cardiomyopathy  is being recognized  .This is often referred to as NDCM (Non dilated cardiomyopathy)  .Global  systolic LV dysfunction  with normal  LV dimension.This a similar to the terminology MDCM (Minimally/Mildly  dilated  cardiomyopathy  where LV dimension  do not  increase  beyond   15 %  upper limit of normal  ) .

This is seen in CKD and diabetic individuals.Atria may be enlarged .Diastolic dysfunction may co exist.  It is no surprise,  this entity closely mimics RCM. But in RCM LV systolic function  is not greatly compromised till the  late stages , while NDCM it begins with systolic dysfunction. This is the only difference .There can be overlaps .

MDCM was reported in 1990  . Keren gave a excellent insight about the condition  ,  It is unfortunate it failed to take off as a popular  clinical entity .  Cardiologists are argued to use this term liberally in their clinical practice .

Final message

Cardiology is not  that  simple as one would like to ! The two components of cardiomyopathy ie   LV dilatation and LV dysfunction can be temporally dissociated  one may precede the other. To  complicate the matter further, one of them may not manifest at all !

Few ,  still consider many of the RCMs and NDCM are one and the same entities that present in different time frames in their natural history.

So the simplified  concept  to decode the cardiomyopathy conundrum  could be

  • When both  dilatation and dysfunction occur it is classical DCM
  • When dysfunction  alone occur without dilatation it is NDCM
  • When both dysfunction  and dilatation are less it is RCM*(Relaxyl dysfunction must)
  • When dilatation is mild and dysfunction is severe it  is MDCM

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How will you refer to a ventricle which is not dilated but still has severe global contractile dysfunction ?

Traditionally cardiomyopathy is classified as

  • Dilated (DCM)
  • Hypertrophic(HCM)
  • Restrictive (RCM)

But there is large group of pateints who do not show any of the above features and still have global hypokinesia  contractile dysfunction. this group has been largely ignored .It could constitute up to 25%of all cardiomyopathy.there can be some overlap between non dialted cardiomyopathy and RCM.

We report our experience here with

non dilated cardiomyopathy click to download PPT

non dilated  cardiomyopathy

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