Mgt:
IV vasodilator challenge (prostacyclin, NO, adenosine) in order to decide definitive treatment
Definitive treatment:
If responsive to vasodilator challenge, start Calcium channel blockers. Try for at least 3-6months, if no response use prostacyclin
If no response to vasodilator challenge, other modalities including Transplant depending on the NYHA classification
Anticoagulation for all patients (Warfarin)
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