This is a heart defect that is present since birth (congenital heart disease). Normally left atrium (LA) and left ventricle (LV) connects with each other and LV gives rise to aorta that supplies blood to body. Similarly right atrium (RA) and right ventricle (RV) connects with each other and RV gives rise to pulmonary artery that connects with both lungs.
When there is connection of LA and RV and RV gives rise to aorta (AV-VA discordance), and RA connects with LV and LV gives rise to pulmonary artery (AV-VA discordance), it is known as ccTGA (in cases where liver is on right side and stomach is on left side that is normally situated). Above mentioned combination can be changed in cases of abnormal location of liver and stomach.
ccTGA can be associated with large ventricular septal defect (VSD), obstruction as the level of pulmonary artery (PS), ECG changes most commonly in the form of complete heart block (CHB) etc.
Patients are generally asymptomatic when there is no VSD or pulmonary stenosis and blood oxygen level is normal and detected incidentally on routine examination.
When associated with large hole (VSD) and without PS, signs of heart failure will be there. And with severe PS, cyanosis will be there.
There is a risk of complete heart block in this heart disease.
In asymptomatic patient with no VSD, PS etc., patient is advised medical follow-up with the advice of ECG and ECHO annually. When is severe valvular regurgitation (tricuspid valve), replacement of tricuspid valve is generally advised (Physiological repair).
The most desirable surgery in case of ccTGA is ‘’DOUBLE SWITCH OPERATION (DSO)’’in which surgeon do Atrial switch and arterial switch operation (ASO).
Atrial switch means baffling of systemic and pulmonary venous channels into LA and RA respectively. ASO means coronary transfer from aorta to pulmonary artery.
Ventricular septal defect has to be closed at the time of DSO.
If there is association of severe PS along with VSD then VSD surgical closure along with placement of an artificial tube from LV to aorta (Rastelli operation) is advised.
If two ventricle repair is not possible then univentricular palliation in the form PA banding or BT shunt, followed by Glenn shunt and then Fontan completion at 3-4 years of age to be carried out.
If patient is having CHB, then it has to be treated by placement of permanent pacemaker into the body.