Recommendations for pulmonary arterial hypertension(PAH) associated with congenital cardiac shunts
| Statement | Class# | Level¶ |
| The ERA bosentan is indicated in WHO-FC III patients with Eisenmenger's syndrome | I | B |
| Other ERAs, phosphodiesterase type-5 inhibitors, and prostanoids should be considered in patients with Eisenmenger's syndrome | IIa | C |
| In the absence of significant haemoptysis, oral anticoagulant treatment should be considered in patients with PA thrombosis or signs of heart failure | IIa | C |
| The use of supplemental O2 therapy should be considered in cases in which it produces a consistent increase in arterial oxygen saturation and reduces symptoms | IIa | C |
| If symptoms of hyperviscosity are present, phlebotomy with isovolumic replacement should be considered usually when the haematocrit is >65% | IIa | C |
| Combination therapy may be considered in patients with Eisenmenger's syndrome | IIb | C |
| The use of CCBs is not recommended in patients with Eisenmenger's syndrome | III | C |
ERA: endothelin receptor antagonist; WHO-FC: World Health Organization functional class; PA: pulmonary arterial; CCB: calcium channel blockers. #: class of recommendation; ¶: level of evidence.