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Posts Tagged ‘congenital heart disease’

I stumbled upon a TV reality show where a mother of a child  was crying inconsolably ,whom she lost when it fell  into a open  bore-well pit .She was blaming it as her fate and the hole was sent by Lord Yama (God of death )

I  just got  curious ! This article was written  in a flash.

I used to get questions from anxious parents  of  children with holes in the heart .(Asymptomatic small VSDs or ASDs  who come for  periodic echo-cardiograms) .I reassure and  convey  the message , most of these  holes are tiny and will close automatically and they need not worry.Even if it doesn’t, it poses little problem.

 

asd clsoure

But .after watching  that haunting TV show, I have started to warn  the parents  that  holes in the heart may not be that dangerous  but  be wary of  holes in the roads  and unclosed bore wells  in our country !

Every single parent was amused  with  my statement  ! Some how it appeared sense to me !

I made a mini google research. It is estimated thousands of bore-wells are dug every day and kept open in allover the country side .It is like live land mines .  Some press reports  suggest at least one child dies in India every day due to uncovered bore-wells and man holes ! (May be really true !)

The following are  some of the  samples.

One

borewell deaths 4

One more

borewell deaths  3

Two

You can understand the gravity of the problem , one engineer has devised a special  child retrieval Robot for such accidents.

baby snare bore well cath lab snares asd vsd device av loop how to snare a device

Three

A news report  in Times of India  .What shall we do about these ?

asd vsd borewells pits holes in the heart

It is a horrific truth in this  civilised world .Nature creates  holes  in the heart due to defective gene in some . It appears  less dangerous to me. After all a hole in the heart  threatens only one life,while  a hole in the road  kills many people.

As a cardiologist  , I am saying  this with anguish as our  team  along with  surgeons  work  over time to close intra-cardiac  holes  with device ,  the  holes in the road are often  callously  kept open   forming   death traps  for our children .The men responsible for such things deserves no  pardon.

The story is never ending . . .

borewell deaths 2

Post-amble:
Doctors are not just the  noble professional who provide relief  from  illness . They  have  other social responsibilities  too !
I believe ,  as physicians bound by Hippocratic  oath , we should  help tackle issues that threaten  our pubic health  system , especially in this case were the victims are  vulnerable children !
In fact ,  pediatricians should come forward to work with Govt to  improve safety  issues for children  and  orthopedicians  should help  prevent road traffic accident while  cardiologists  shall  work with the Govt to prevent  heart disease . . .  etc etc .
Though  bore-well deaths are  a pan India  phenomenon , One state in India ( TN)   has taken a   new initiative recently  and has  banned digging  bore-wells without prior permission and stipulated strict guidelines .Others can take a cue from here !

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Life  is nothing , but the intervening time between the first heart  beat and the last heart beat of an individual  !

Human heart is not an ordinary organ.  Right from the day 22  of fetal life , when the cardiac jelly  begins to beat till  the early  new-born period ,  (In some, even longer !)  it continues to evolve.

We know  heart as a hemodynamic organ . But ,  in the fetus   it is  dynamic in  a  different sense (Embryo-dynamics) . As the mother’s  heart  takes care of the fetal circulation ,  the fetal heart concentrates  on it ‘s  own growth  .The heart learns the  lessons of life in a hard way  , it has to survive the next 70 -80 years independently .

The complexity is enormous . The cardiac muscle  comes  from  mesenchyme, the conducting system  comes from ectoderm .Systematic  events  like , looping  , partition  , regression  of the heart tube should occur at critical times .  Apart from this , the venous and arterial  connections ( Aorta IVC,SVC)  develop concomitantly and has to fuse with respective  chambers without any error.

It is a wonderful biological marvel  happening inside every fetus without the help of any architect  !

The changes do not stop at birth. It continues , well  after delivery . One  hole gets closed(PFO), one conduit disappears (PDA) .One chamber regress (RV) . We, expect all these things to happen in a  meticulous and  sequential  way.

Yes , it happens in most. But , in many with altered bio – genetic forces  things   fail to unfold  in  the programmed way.    It is not at all a surprise ,  to find some common aberration .

So , when some body is born  with a congenital heart defect , don’t blame  it on God .He does his job , in billions and billions of heart in the right manner .

It is our ancestral gene  code that gets awry in a few  !

In Hindu  philosophy  the  defects  we inherit  are the wages we pay for our ancestral misdeeds  .


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Some journals do a great work silently .Impact factors are a non issue for them

It is the content that matters . Pediatric cardiology is one such journal !

Of course , they don’t publish papers  that have  greatest  significance to mankind !

like Telmisartan is not inferior to Ramipril in the mangement of hypertension

and Fondapaurinox  is as effective as regular Heparin   . . . etc  . ..etc

They dedicate themself in the decoding the mysteries of congenital and acquired heart disease of children .

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Atrial  septal defects  are one of the commonest forms of congenital heart disease.

  • The commonest being the ositum secundum ASD     ( Which is in fact is a defect in the development of septum primum)
  • The next common is ostium primum defect which is a part of AV canal or atrio ventricular septal defect.

Other forms of ASD include

  • SVC type /Also  called sinus venosus type of ASD .
  • IVC type
  • Coronary sinus defect -Also called partial or complete forms of unroofed coronary sinus

asd svc asd sinus venosus

SVC type ASDs

They are in the strict sense can not be called as ASD. This is because there is no defect in  any of the  embryological  inter atrial septal component.

There is no direct communication between RA and LA, instead   a window  or passage of communication   between pulmonary vein and SVC. Right upper lobe pulmonary vein  is usually the culprit .Some times more than one PV  communicates with SVC.

The exact area of this PV-SVC window occur between anterior surface of right upper lobe PV with postero lateral surface of SVC.

PAPVC partial anomalous pulmonary venous drainage can be considered an integral part of this defect as RUPV is linked with SVC.

Can we have a combination of SVC ASD and OS ASD ?

This is possible .But two embryological errors need to occur. This is often seen as a large OS ASD with deficient or absent superior rim. So whenever superior rim of IAS is deficient a PAPVC and a SVC ASD should be looked for.

Clinical features

  • SVC type ASDs  generally shunt lesser blood than OS ASDs. (Often<2:1) .This is because it is not the LA that is communicating with RA instead only a
  • It is usually a single PV (some times 2) that shunts from left to right.

There is a distinct possibility of missing this lesion in routine echo.Minimal RA,RV enlargement may give us a clue.The classical subcostal or  4 chamber  view in echocardiography may not visualise  these defects.

So, whenever one encounters mild dilatation  of RA and RV and the IAS appears  intact, a meticulous search and a focused echo in the superior aspect of IAS is warranted. Angled  superior views may pick up this defect.A transesophageal echocardiogram (TEE) is often required to  confirm it.

Therapeutic issues

  • Device closure is not possible
  • Surgery involves little more technicality than ASD OS.
  • Small defects can be patch closed.
  • Some times the SVC has to be disconnected from the PV and anastamosed separately on right atrial appendage. SVC resection  will  aid the surgeon in proper patch closure.
  • Post operative follow up is necessary as SVC obstruction or PV obstruction may be a delayed consequence

References

svc asd sinus venosus

http://ats.ctsnetjournals.org/cgi/content/full/59/6/1588?ijkey=4f42649bc5805f3cf7d60f5728ec7f871356277b

http://asianannals.ctsnetjournals.org/cgi/content/full/10/3/231

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