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Corrected transposition of great arteries :A rare review article !

Posted Sep 14 2010 10:18am

In this era of  technology hype , cardiology  journals are flooded with interventional   articles. Congenital heart disease has been pushed to the back ground .  CTGV* is a   fascinating  congenital heart disease (Of course ,not so fascinating  for the patient !)

*TGV and TGA are used interchangeably  .

It is a complex disorder of ventricular looping (L Looped ventricle LTGA )

This can occur in three forms

  • Isolated CTGV
  • CTGV with VSD or PS
  • CTGV as a part of complex  cyanotic heart disease.

The irony of this disorder is , it has two errors in development that tend to  neutralise  the hemodynamic  abnormality .

Atrio ventricular connection is abnormal (Discordant) . RA is  connected to LV and LA connected to RV . Still nothing alarming  happens  as LV is connected to  Pulmonary artery and RV is connected to Aorta .(Ventriculo arterial discordance)

In spite of this natural  hemodynamic  correction , one can not ignore this entity  ! as it is  anatomically  uncorrected  for the rest of the life.

Since morphologic RV acts as a systemic ventricle it is bound to  have difficulty in tackling the systemic pressure in later in  life .

Further , the two  complex  defects called  (also called as  ventricular inversion ) make sure that the conduction tissue and the AV valves   are distorted and  squeezed in the AV junctional arena with it’s unique double looping defect  .The his bundle inverses, the left AV valve often regurgitates .Complete heart block often ensues.

Management

  • Isolated CTGV are best left alone .
  • When VSD /PS are associated corrections are adviced
  • It is not simple surgery , one may require a technique called double switch .(I always wonder such surgeries are ever indicated in other  wise asymptomatic population with isolated CTGV)

Here is an article from “The  Heart“  that deals with the problem of CTGV , may be  . . . in a manner no other journal  has ever done !


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