Double outlet right ventricle is a group of conditions where both the blood vessels i.e. the vessel to the body (aorta) and the vessel to the lungs (pulmonary artery) arise from the right pumping chamber. For a refresher on the normal heart please refer to Blog post number 4.
Double outlet right ventricle is not one condition but a group of conditions where the common feature is that both the vessels arise from the right pumping chamber (RV). It is almost always associated with a hole in the wall between the pumping chambers (Ventricular Septal Defect or VSD) and sometimes there is obstruction in the blood flow to the lungs (Pulmonary stenosis or PS).
DORV is divided into several types depending on the arrangement and position of the two blood vessels and also on the position of the VSD. Below is a diagram showing the various positions of the VSD in relation to the two blood vessels.
The position of the VSD and the presence of obstruction to lung blood flow will determine whether a child is breathless or not and also whether there is 'cyanosis' (Refer to Blog post No 6). Some babies with DORV will need early surgery as they are found to be very blue due to severe obstruction to lung blood flow. These patients will need an initial 'shunt' operation to improve lung flow and then maybe a year or so later they can have the complete operation. The aim with surgery in DORV is to try to bring the heart back to normal. This is achieved using many surgical techniques
BT Shunt operation Complete Repair
Sometimes it is not possible to perform complete repair and a staged approach has to be taken to separate the blue and red blood in the heart (Glenn operation at 6-12 months of age followed by Fontan operation at around 5-7 years of age)
Glenn Operation Fontan Operation
Following surgery there needs to be close follow up at regular intervals. There is sometimes a need for re-operation in these patients.
Double outlet right ventricle is not one condition but a group of conditions where the common feature is that both the vessels arise from the right pumping chamber (RV). It is almost always associated with a hole in the wall between the pumping chambers (Ventricular Septal Defect or VSD) and sometimes there is obstruction in the blood flow to the lungs (Pulmonary stenosis or PS).
DORV is divided into several types depending on the arrangement and position of the two blood vessels and also on the position of the VSD. Below is a diagram showing the various positions of the VSD in relation to the two blood vessels.
The position of the VSD and the presence of obstruction to lung blood flow will determine whether a child is breathless or not and also whether there is 'cyanosis' (Refer to Blog post No 6). Some babies with DORV will need early surgery as they are found to be very blue due to severe obstruction to lung blood flow. These patients will need an initial 'shunt' operation to improve lung flow and then maybe a year or so later they can have the complete operation. The aim with surgery in DORV is to try to bring the heart back to normal. This is achieved using many surgical techniques
BT Shunt operation Complete Repair
Sometimes it is not possible to perform complete repair and a staged approach has to be taken to separate the blue and red blood in the heart (Glenn operation at 6-12 months of age followed by Fontan operation at around 5-7 years of age)
Glenn Operation Fontan Operation
Following surgery there needs to be close follow up at regular intervals. There is sometimes a need for re-operation in these patients.
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