Posts Tagged ‘rheumatic heart disease’

Rheumatic fever and RHD is still a major cause for  cardio vascular morbidity and mortality in India .It seems , improving quality of life  has little  impact on the incidence .(We could realise  this as  we sit in the cardiology OPD of a 200 year old hospital !) There is no country wide data on the true prevalence .  Our understanding of rheumatic heart disease is based on isolated  studies on localized populations .

Of late , cardiology   resources  in our country is diverted towards  much glamorous CAD the poor continue to  suffer with  RHD.

Just Imagine many   hospitals indulge in 1000s of PTCA every year  but hardly do  a  hand full of PTMCs.

How our cath  lab resources  are used   across the breadth and length of country needs some introspection (Currently , I believe we have about 750   labs ) .I think there should  be a binding legislation  in every cath lab .For every 10 PTCAs done   at least one PTMC must be done to heal the poor .( Like the Air-craft license  .You can’t fly only the lucrative metro sector is given only if IT  services less developed areas )

In this scenario ,  it is a pleasant  surprise  to find a  wonderful review article on RHD from the two pioneers ,  in lesser known medical journal IJMR  .

Review article rheumatic fever  Indian journal of medical researchEspecially heartening  is  the fact ,  it is a collective effort  from two  distant  regions of India ( Kochi from down south and  New Delhi In the north) . While politicians keep the divide , it is a great  work  of the authors , which  would help  youngsters  who  would like  to go on a national mission on eradication of RHD. .




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Mitral regurgitation is  one of  the most common lesion of rheumatic heart disease .Mechanism of MR in acute rheumatic fever is different from chronic rheumatic heart disease.

Acute Rheumatic fever

The following mechanisms contribute to MR of acute rheumatic fever

  1. Edema of leaflets (Carey Coombs murmur )
  2. Valvulitis
  3. Small verrucous  vegetations (See Image )
  4. Acute LV dilatation in fulminant cardiac failure.

* Note  : Acute rheumatic fever in its first episode can never  cause stenosis  however fulminant the fever may be  .There is no acute mitral stenosis .But ,  during recurrence and reactivation some amount of stenotic process may occur.  Still ,  recurrence and reactivation are more often related to significant MR rather than MS. ( Isolated mitral stenotic lesions  rarely  give h/o recurrent rheumatic fever )

Chronic rheumatic  heart disease

As the mitral valve gets progressively damaged  any combination of MS or MR occur .The following mechanism are involved in  the genesis of MR. (Pathology of Mitral stenosis is not discussed here)

  1. Chordal shortening, tethering , pulling , prevent proper co-optation
  2. Chordal lengthening
  3. Chordal disruption (Minor > Major )
  4. Prolapse of either AML or PML (Not both ,unlike myxamatous MVPS)
  5. Infective endocardits  of  leaflet
  6. Perforations of  leaflet
  7. Annular  dilatation
  8. Fibrosis of posteromedial/Antero-lateral   pap muscle(Rare )
  9. Left atrial pathology

* The direction and the  width of MR jet is  related to the mechanism of MR.

If there is chordal shortening due to fibrosis  of mitral valve  co -optation plane is altered . The degree of chordal shortening , pap muscle fibrosis (rare)  symmetry of chordal involvement determine the MR.

Rheumatic mitral valve prolapse

  • This could be  more common than we realise.
  • It can be true or pseudo.
  • True prolapse occur due to chordal weakening or lengthening .
  • In chordal disruption the leaflet tips usually become flail

Since rheumatic process fixes the PML first , the AML   appear to overshoot the plane of PML and   appear as prolapse.(Pseudo )

The sail like AML commonly  directs the jet posteriorly and laterally .(Murmur conducted to axilla and back )

It is rare for PML to prolapse in RHD , if  it does occur ,  it directs the jet anteriorly (murmur conducted to aortic area mimic AS !)

It is rare to see a  perfect  central jet in RHD  . presence of  Central jet is a good sign to consider mitral valve repair.

Myocardial involvement in RHD.

Even though rheumatic fever is a classical  example for  pan-carditis , it is surprising   to note (Of course fortunately !)   how  myocardium escapes in the  chronic process of RHD.

Is it really true  ,  myocardium do not get involved in chronic RHD ?

Clinical cardiologists rarely discuss this issue. Pathogists indeed have documented significant lesions within myocardium  . Involvement of left atrial myocardium and  rarely  ventricular myocardium in the sub mitral  zone  can influence the  degree of  MR

* Even in acute rheumatic fever with fulminant carditis , myocardial involvement is  disputed by many  ! . My belief is ,  there will   definitely a subset  in   both acute and  chronic  forms of   RHD   , in which myocardium  gets  involved . In our institute LV dysfunction associated with RHD occur in  up to  5 % of  RHD population .

Importance of knowing the mechanism of MR

Two aspects  appear important

1. Is there a potentially  reversible component in pathology so that we can  wait  before intervention  ?

I have seen children referred for mitral valve replacement due to severe MR  . In due course   MR regress by the time they reach the tertiary center (waiting period included ) At least one child i remember,  the MV surgery was canceled  due to spontaneous regression MR.

It was later found the MR was  more of valve inflammation than degeneration .

* Always think about the possibility of reversible rheumatic MR  in every severe isolated  MR in children (Do not apply this rule in adults or in combined MS or MR  )  Do a ESR, ASO and start an  intensive anti inflammatory therapy  , aspirin with strict penicillin prophylaxis .With this  one can definitely postpone the surgery  in few cases  and  may avoid it altogether !

2. Surgical implication

If we could delineate  the  exact pathology of MR   it will facilitate  the   repair . Annular  reduction and  neo  chordae  etc . Of course ,the surgery could be  very  difficult in scarred mitral valves ,  Dr Sampath kumar *of AIIMS  New delhi , India  would  feel other wise !

*A pioneer in mitral valve repair in chronic  RHD (See reference 2 )

Questions  that need  answers

How is balloon/Surgery  related injury different from rheumatic process ?

Why is  rheumatic  mitral vale  prone for bacterial infection ?

What is the relationship  between  extent of  aortic valve involvement and  degree of mitral valve involvement in RHD ?



2. http://icvts.ctsnetjournals.org/cgi/reprint/5/4/356


There two popular books exclusively  for cardiac pathology


2. Renu Virmani

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Rheumatic heart disease is rampant in India.  Advanced forms of mitral stenosis are  still common.

Critical mitral stenosis with LA clot formation is often seen.

But here is a  women in late twenties  presenting for the first time with syncope .

And what  you see inside is  not a fiction  . . .

Left atrial clot occupying the whole cavity ! Where is the blood bound for left ventricle ?

4 chamber view

Luckily the clot is   so  big and MVO is less than 1 sq cm. It is highly unlikely the LA clot can negotiate the orifice.

Small fragments can dislodge .This patient developed syncope whenever she bends and lie down at a particular position.

What needs to be done in this patient  ?

Can it be lysed ? No ,Emergency surgery is required with concomitant mitral valvotomy or mitral valve replacement.

Is there a temporary aortic filter available to prevent systemic emboli from heart  ?

Distal protection devices are  available only temporarily in the coronaries and  carotids during interventional procedures.There is no aortic protection devices  for LA,LV clots in high risk patients .  When IVC filters  are used  block a potential pulmonary   clot why not aortic filters  for preventing systemic emboli  ?

Why we have not thought about  this  . . . is  surprising . May be intensive anti coagulation is as effective .

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A young women  with Rheumatic heart disease .


LA aneurysm

Giant left atrium in rheumatic MS . http://www.drsvenkatesan.com

Mitral regurgitation is significant .www.drsvenkatesan.com


When do you call  a left atrium as   giant  ?  When it is referred to as  Aneurysmal dilatation ?

It is all semantics. Whenever LA becomes more than 6 cm ,  at least in two diameters  many prefer to call it giant .

In India , 6 cm LA is such a common finding , we have kept a  cut off at an  arbitrary 9 cm .

What factors determine a LA to dilate like a balloon ?

The exact mechanism is not known.It could be  the  intrinsic weakness of LA wall ,  as very few with RHD develop this. Many LAs resists dilatation even in the midst of extreme LA pressure. But , it is a well-known fact , mitral regurgitation provokes greater LA dilatation than MS alone .This implies volumetrics  play a major role than  pressure dynamics  in determining LA size. Acuteness of hemodynamic insult is  inversely proportional to LA size.

By the way, what is the purpose of  recognizing  the LA as Aneurysmal ?

  • In plain X -ray chest , LA may  form the right heart border  over shooting the RA.
  • When LA becomes huge  , there is a  chance for mechanical complications  like dysphagia, phrenic nerve , bronchial compression etc .
  • Giant LA invariably increases the chance of LA clot.

Electro-physiological Issues

  • Atrial fibrillation , a usual accompaniment of giant LA ,  is often refractory . There is no  purpose  to convert to sinus rhythm . In fact ,  one should not attempt this. There was a time when surgical incisions  ,corridors , mazes were quiet popular.Now it is believed all these are adding further injury to the ill-fated LA .Electro-physiologists should be restrained . Pulmonary vein ablation should never be attempted in such cases as the focus of AF is elsewhere .

Implication in cath lab

During PTMC LA size can be an issue  as the plane of IAS is distorted and make things difficult for septal puncture . Further the balloon , guidewire  may often slip  back into RA .

Implication for the surgeon.

For the surgeon the implication could be more. As a cardiologist I can’t comment about that .One thing we have observed is when LA becomes huge , the size of mitral annulus is too fictitious and funnily enough we have recorded up to 6 cm of mitral annulus . No valve is available for this size . We learnt from the surgeons ,   large LA  rarely pose a  problem as they suture the much  smaller valve in a larger annulus .(Which  makes the task  that easier )

Does the LA size regress after surgery ?

In many  it does regress  , in as many it doesn’t. We have seen giant LAs continuing to trouble the patient even after a successful mitral valve replacement.

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Planimetery is the age old method to measure the mitral valve  area( MVA) by echocardiography.


  • Simple modality
  • 2D echo  is enough
  • Doppler errors avoided
  • In the presence of MR, planimetery orifice has an edge over other methods


  • Optimal gain setting becomes  important .There is  significant inter and intra observer variability.
  • Shape of the orifice is not constant  ( MVO is funnel like) . Narrowest diameter is usually measured.
  • Planimetery is  a purely an anatomical orifice,while blood flows through both primary and secondary mitral orifices .Sub valvular fusion makes secondary MVO the  narrowest point  . Measuring it becomes difficult as it has no defintion of border.
  • Gross errors possible in calcified valve.
  • In post commissurtomy  the  lateral extent of split is often  not tractable

How to improve the accuracy of planimetery ?

Color Doppler aided  2D  planimetery . This can improve some of the limitations , as  it provides a hemodynamic MVO(Some what physiological ) Of course  , pressure halftime derived MVO is purely a physiological orifice .

Other options to measure MVO

  1. Pressure half time
  2. Continuity equation
  3. PISA method

Advantages and disadvantages  of Pressure half time derived MVO will be posted soon.

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RHD is the scourge of mankind . While a part of the world is suffering from glamorous  lifestyle disease ,a  significant  population (The country I live – India , included)  is suffering due to  “lack of any style” in their  life !  exposed to  primitive streptococcal infections  and end up in deadly destructive heart valve disease .

While , the  privileged  cardiologists make merry in cath labs  ,  a  few toil in the deep sub Sahara forests and the tropics  of Cambodia  to bring some sense and balance  to  cardiology literature.

Mankind will benefit  much more  these committed souls* than the pseudo-research that is happening world wide !

Of course ,  we should thank the NEJM to publish  such  precious articles !

The summary of this article goes something  like this

Echocardiography ,  if used widely in general population  ( more specifically in children)  can identify rheumatic fever early with high degree of precision .With penicillin prophylaxis we can reduce the RHD burden of our planet significantly.

The message may be simple, but in the modern world  people’s suffering  is not due to lack of  sophisticated health tools , but  “lack of common sense “

This article came in 2007, unfortunately it has not  generated the  desired  impact  among the cardiac care takers  .(While  a  mediocre ,  drug eluting stent can reach 1000 cath labs in 100 countries in a matter  of weeks!)

The World health organisation (WHO) is yet to formally adopt this new  echocardiographic criteria to diagnose Rheumatic fever .We expect  the WHO , to urgently formulate new guidelines for early detection of rheumatic fever .(It is better , we stop hanging on to Jones for over  half  century !)

Will the WHO be proactive ?

If a portable echo costing few thousand dolors can save  millions of life , let the WHO the bring an ordinance to supply  (liberally ) manpower and  machines  to  all those poor countries  which lack in basic health service , but infested with  free supply of  deadly land mines , outsourced arms and ammunition !

This study was performed in  Maputo Heart Institute  Mozambique , and Cambodia .



* Three cheers to Eloi Marijon, M.D., Phalla Ou, M.D., David S. Celermaje .

Chain of Hope

Travel online to Africa : A journey into human side of cardiology  . . .

The charity  that has adapted  Maputo Heart Institute  Mozambique

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                                       Left atrium is the posterior most chamber of the heart.  It is almost a mid line structure.  The normal size of left atrium is about 4 / 4 cm. Normal left atrial volume is 46ml in men and 38 ml in women .(Atrial volume in a normal adult population by two-dimensional echocardiography Y Wang, Chest, Vol 86, 595-601.)  Left atrium  is not an easy chamber to identify in the  X ray chest as it does not form  the cardiac border.( Except a small circumference of left atrial appendage.(LAA)

Left atrium can enlarge in multiple directions.Generally it dilates in the path of least resistance.


  • It is believed left atrial appendage  enlargement occur early .  LAA enlargemnet seen as a fullness beneath the pulmonary artery shadow. It may be the earliest finding of LAE in X ray. ( This may appear as straight left heart border , as in classical  mitral stenosis where MPA is also enlarged). The LAA enlargement is not necessarily in  in proportion  with LAE.
  • LA could  also enlarge posteriorly by pushing the esophagus towards the spine.This is visible only in barium swallow.
  • Then LA can enlarge either to left or right ( Usually towards right) and  reach the right heart border or over shoot it and form the right heart border by itself.This occurs very late in the course.
  • The other direction  LA goes on to enlarge is superiorly. When LA enlarges superiorly it hits on the left main  bronchus and lifts it.This is measured by the widened subcarinal angle which is normally less than 75 degrees.
  • LA can enlarge anteriorly  sometimes , but it is resisted by right ventricle but rarely right ventricle yields to the LA push and produce a left parasternal lift which could be mistaken  for RV enlargement.
  • Inferior enlargement can not happen in a significant way as it is limited by the AV groove and strong fibrous skeleton. 

With the advent of echocardiography X ray assessment of LA is redundant .(Academic value and in fellows training programs).The upper limit of normal LA size is around 4.5cm.

LA enlargement is commonly seen in

  • Rheumatic mitral stenosis, regurgitation. Gross enlargement up to 10 cms are common.
  • Hypertensive heart disese.
  • Cardiomyopathy, especially restrictive where both atria enlarge.

In all these conditions if  atrial fibrillation occurs  LA size increases further.

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