It consists of large ventricular septal defect with severe pulmonary stenosis (PS) with anterior mal-alignment of conal septum. Due to severe PS, blood supply to the lungs is very less and patient becomes very blue. Blueness severity will depend on severity of PS. Blueness is assessed clinically and by using saturation monitor.
When TOF shall be treated?
It depends on symptomatology of the patient. If spo2 is less than 70-75%, early surgery in the form of Blalock-Taussig shunt (BT shunt) can be done or else we can wait till child is 5-6 kg. At that time final correction can be done if cardiac anatomy is suitable for that. In total repair, VSD is closed and RVOT is reconstructed in such way that PS is also get relieved. Surgery is to be done by opening the chest only.
Prognosis of TOF repair?
Good in long term
Is pulmonary valve sacrificed during total repair?
It depends on size of pulmonary annulus, if it is adequate for age then there is no need to sacrifice the valve or else we need to sacrifice this valve.
What will happen when pulmonary valve is sacrificed?
There will be pulmonary regurgitation and gradual dilatation of right ventricle. Later on there may be requirement of replacement of pulmonary valve.
TOF with large VSD
TOF with Pulmonary Stenosis