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.
- 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 !