DORV - Double Outlet Right Ventricle

  • In 'Double Outlet Right Ventricle' (DORV) the two Great Arteries (Aorta and Pulmonary Artery) both originate from the right ventricle and blood from the left ventricle passes across a VSD into the RV to reach the great arteries. The lung circulation is often exposed to very high pressure and increased blood flow (as with a large VSD).

    In most cases the infant develops symptoms in the early weeks of life - either with 'Cyanosis' (Blueness of the skin) or with breathlessness and failure to gain weight normally. There are many different varieties of this abnormality. Many affected patients have associated defects in the heart or main arteries, including such problems as Pulmonary Stenosis (PS), Pulmonary Atresia, other valve abnormalities or Coarctation of the Aorta, etc. Such problems may cause severe obstruction to blood flow to the lungs or into the main circulation and may require urgent surgery.

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    Treatment, which is surgical, is aimed at repairing the defect and any associated problems, where that can be done. In many cases the heart can be made to function sufficiently well to eliminate symptoms and to allow normal schooling and near normal activities. Most cases are amenable to 'Complete Repair', though more than one operation may be needed.

    In a minority of the more complex cases complete repair may not be possible. In such cases treatment aims to make the heart work as effectively as possible.  This may involve several operations. For some children the final operation is called the  'Fontan Operation'. This leads to blue blood (low in oxygen) being channelled through the lungs, without any 'pump' driving it. The ventricles then pump the red blood (high in oxygen), round the body. This operation makes the child 'Pink' - but does not correct the original problem.