Double Inlet Left Ventricle

  • (Sometimes called 'Single Ventricle')

    This is one of a group of very complex defects where both Atriums connect to the same ventricle and/or one ventricle is absent or very tiny. 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. 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 sever obstruction to blood flow to the lungs or into the main circulation and may require urgent surgery.

    The RV (usually very small) may be on the opposite side of the heart to normal. This is similar in some respects to the situation with "Corrected Transposition. 

    Treatment is aimed at repairing the associated problems, where that can be done and performing staged surgery (often with several operations being needed) to make the heart work effectively. The basic defect cannot be repaired, but in many cases the heart can be made to function sufficiently well to eliminate cyanosis and to allow normal schooling and near normal activities.

    For many 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 large ventricle then pumps the red blood (high in oxygen), round the body. This operation makes the child 'Pink' - but does not correct the original problem. Not all affected children need to have a Fontan operation. If they are well, with less drastic surgery, they may not have this 'Final' big operation. Some children may be unsuitable for a Fontan operation and alternative forms of treatment may be offered.