Double Outlet Ventricle

It can be either double outlet left or right ventricle. Double outlet right ventricle is more common.

In this, both aorta and pulmonary artery arises from a single ventricle. Ventricles are the chambers that are 2 in number and situated in lower part of heart and separated by a ventricular septum.

Normally, aorta arises from left ventricle and pulmonary artery arises from right ventricle but in case of double outlet left or right ventricle, both almost arises from a single chamber. In this situation there is 100% commitment of one vessel to a ventricle and another vessel most of the times has 50% commitment to each ventricle.

When this is associated with large hole in the heart with no pulmonary stenosis (PS), they often come with signs of cardiac failure. These can be in the form of poor weight gain, shortness of breath, etc.

And when there is an association of severe pulmonary stenosis, patient can come with history of lips and fingers discoloration.

Double outlet right ventricle (DORV) can be either subaortic type or subpulmonic type (Taussig-Bing malformation). Patient present early in later variety.

Management depends on echo assessment regarding the routability of VSD to aorta in subaortic or VSD to pulmonary artery in Taussig-Bing malformation. If routable then in subaortic type only VSD needs to closed (preferably in first 6 months of life).

In Taussig Bing type, VSD is routed to pulmonary artery and switch operation at great artery level (arterial switch operation) is carried out.

If ventricular septal defect is not routable then treatment has to be planned in the line of single ventricle pathway, meaning that child cannot be given 4 chamber circulation (normally 4 chamber circulation is there).

When there is no pulmonary stenosis, then we tighten the pulmonary artery by putting a band in main artery known as PA banding. In opposite situation with less blood supply to the lungs, Modified BT shunt needs to be done (stage I operation). Either of these needs to be done early in life.

After 6 months-one year of first stage, we need to done Glenn shunt as a second stage operation. In this operation, superior vena cava (SVC) is disconnected from heart and connected to pulmonary artery.

After 2-3 years of Glenn shunt,Fontan operation to be carried out as a third stage of operation. In this operation, inferior vena cava (IVC) is connected to right pulmonary artery with the help of artificial tube (CONDUIT). 

After all 3 stages, there is good outcome and child can live a good life. Without surgery, patient can die very early.