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Posts Tagged ‘left to right shunt’

PAH  is  the major determinant of surgical outcome of left to right shunts. In this  modern era of cardiac care  allowing a child  with   left to right shunt   to progress to a  stage of   Eisenmenger syndrome  is  considered  as a  huge medical failure . But  , this is still rampant in many of the developing countries .

Cardiologists are divided over the issue of  operability of Eisenmenger syndrome .The confusion is largely due to the conflicting data of outcome in these patients. While  there is strong   data  when  PVR exceeds  SVR  ,  the death is imminent in the post operative period .

What has complicated the issue is   there are  many case reports  where severe PAH patients have been successfully operated. Most would think it is a statistical exception and one can  not alter the traditional criteria based on few case reports.

But ,it remains an irony as on 2009 ,  we do not have a proper methodology to assess reversibility of PAH in Eisenmenger syndrome . Further ,  there is a  significant number of  patients with high PVR  , who continue to experience  an  unabated left to right shunting .  We do not have an answer  for either the mechanism of such shunts and  how to manage these patients.

Click over the slide  to view full  PPT  presentation in PDF format .

This short paper was presented in the Annual scientific sessions of cardiological society of India 2009 regarding the usefulness of a new parameter to assess reversibility of PAH. This may not be called as  a study rather a report of  our experience  in  five  patients  with eisenmenger syndrome

Download the full PPT presentation in PDF  format.

pulmonary artery pulse pressure

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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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Atrial septal defect is one of the common congenital heart disese. Surgical correction or device closure is indicated in all patients  with significant shunts. Statistically for every  ASD diagnosed  with  more than 2:1 shunt there must be  is atleast three pateints  with ASD with less than 2:1 shunt in general population. Do we diagnose it ? . Some may be miss diagnosed as PFO.

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