you can cautiously try with a small dose of specific pulmonary vasodilators type sildenafil or bosentan and see how your patient reacts. Some patients do however deteriorate and show signs of pulmonary edema, requiring additional treatment and cessation of antipulmonary hypertensive treatment. 

We previously suggested trying iNO to see how the patient reacts in adults and then if ok moving to sildenafil (attached). At a conference last week where this was discussed and some advocate adding in epoprostenol after sildenafil on board. Having background diuretics a good idea. Will keep an eye on this thread to see if we can take some messages from paeds in to adults.

Article Pulmonary veno-occlusive disease presenting with recurrent p...