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The tricuspid valve is absent and the right ventricle is usually small. There is usually a reduced blood flow to the lungs.
Increasing the blood flow to the lungs is necessary by a shunt procedure when symptoms warrant. In small babies, this is likely to be an arterial shunt connecting one of the arm arteries to the lung artery. In older infants and young children, a venous shunt can be created joining the upper body vein (the superior vena cava) directly into the lung artery (a Glenn shunt).
Definitive surgery involves directing blood returning to the heart from the body directly into the lung artery. Initially, this involved connecting the right atrium to the lung arteries and closing the hole between the filling chambers. This is the Fontan: procedure. Over the years, there have been many modifications of this operation improving the pattern of blood flow from the veins into the lung artery (total cavopulmonary connection).
For these operations to be successful, they require good size lung arteries with low pressures within them, a good functioning left ventricle (main pump chamber) with no leaking of the mitral valve.
This is a big operation. The recovery period is slower than for many other forms of cardiac surgery. Chest drains frequently need to be left in for a week or two as the body gets used to having high pressures within the veins. There is gradual and continued improvement in well being over many months after discharge. Medicines are usually required also for many months, some units thinning the blood with Aspirin, others formally anti-coagulate the patient long-term with Warfarin.
Often a small communication (called a ‘fenestration’ or ‘window’) is created in the wall between the flow pattern to the lungs and the left filling chamber (left atrium). This acts as a ‘safety valve’ and allows some blood to short circuit the lungs. It is useful in the early postoperative course and often gets spontaneously smaller with time. However, if a significant flow of blue blood into the other side of the heart persists, then this fenestration can be plugged later with a catheter technique.
Children frequently remain with a high colour and blue hands and feet and remain a little tired on strenuous exercise. Most however, are able to play games at school and enjoy normal activities. In some children, further operations to improve and streamline blood flow into the heart are necessary. Irregularities of heart rhythm requiring long term medications may occur in young adult life.