management of tet absent pulmonary valve in an adult

female 27 yr old  dx to have tet with absent pulmonary valve saturating in 80s, with some respiratory symptoms having delivered 3 times, with ps gd 90mmhg, moderate pr,  dilated pas 20-30mm size, whats the management plan

[Jeffrey R Boris, M.D.]

Dear Anamika,

Without evaluating you more and getting all of your information, it is difficult to say what the management plan is.  My guess is that you have not had ANY cardiac intervention?  You may be relatively balanced from a circulatory standpoint, although with your saturations in the 80's, you may be shunting more blood from right to left than you want to be.  My recommendation is that you be seen at a center that has adult congenital heart disease cardiologist and cardiac surgeons to fully evaluate your status and see if any intervention is needed or is feasible at this time.