| From your Guide Dr.MANI |  | |
Double Outlet Right Ventricle | ||
| From your Guide Dr.MANI | | |
Double Outlet Right Ventricle | ||
| |
What is Double Outlet Right Ventricle (DORV) ?
Some of the defects I have described are "simple", some are a little "complex" - but DORV is
something else. It is a common term that actually describes a wide spectrum of heart disease,
ranging from something similar to a Ventricular Septal Defect (VSD), through Tetralogy of Fallot
(ToF) to Transposition of the Great Arteries (TGA). It is sometimes like one, at other times like
another, and occassionally a mixture of some of them.
So if at first you are baffled, don't worry. I too was, and figured it out only after a long hard
struggle.
What is Double Outlet Right Ventricle ?
Normally, a ventricle has just ONE outlet. For the left ventricle, this is the aorta. For the right
ventricle it is the pulmonary artery. In DORV, both of these "outlet" blood vessels - aorta and
pulmonary artery - arise from the RIGHT VENTRICLE, either totally or to a great extent.
That sounds simple. What's so complex about that ?
Well, the complexity lies in the extreme variability of the position of the outlet vessels in relation to
each other, and the presence of additional defects. These include VSD, narrowing of the pulmonary
valve (Pulmonary Stenosis), abnormal attachments of the mitral or tricuspid valves and
abnormalities in the structure of the ventricles themselves.
The number of combinations possible are simply mind boggling. I will try and explain how exactly
this affects the outcome, and decision about surgical repair.
What happens in DORV ?
Most cases of DORV have a VSD. DORV is classified based on the relationship between the
VSD and the blood vessels. If the VSD is right under the aorta, it is called DORV with Sub-Aortic
VSD. If it lies under the pulmonary artery, it becomes DORV with Sub-Pulmonary VSD - also
called the TAUSSIG-BING anomaly. If the VSD is under both the arteries, it is called DORV with
Doubly Committed VSD. And sometimes, the VSD is remote from the arteries, and is known as
DORV with Non-Committed VSD.
This has a bearing on how the child with DORV behaves. When the VSD is right under the aorta,
the features are like that of a simple VSD. This means that the child is likely to get frequent chest
colds, and become breathless on exertion.
When in addition to this, there is narrowing of the pulmonary valve (Pulmonary Stenosis), the
course is similar to Tetralogy of Fallot (ToF). There is a "blue" discoloration - cyanosis.
Paradoxical embolism, squatting, and easy fatigue on exercise may be seen.
If the VSD is sub-pulmonary, the features are just like those of Transposition of Great Arteries
(TGA). Cyanosis, giddiness, fainting, and chest infections may occur.
When the VSD is doubly committed or non committed, clinical features are variable. In either
instance, the presence of pulmonary stenosis modifies the picture.
What factors affect treatment of DORV ?
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