Table 25—

Recommendations for pulmonary arterial hypertension(PAH) associated with congenital cardiac shunts

The ERA bosentan is indicated in WHO-FC III patients with Eisenmenger's syndromeIB
Other ERAs, phosphodiesterase type-5 inhibitors, and prostanoids should be considered in patients with Eisenmenger's syndromeIIaC
In the absence of significant haemoptysis, oral anticoagulant treatment should be considered in patients with PA thrombosis or signs of heart failureIIaC
The use of supplemental O2 therapy should be considered in cases in which it produces a consistent increase in arterial oxygen saturation and reduces symptomsIIaC
If symptoms of hyperviscosity are present, phlebotomy with isovolumic replacement should be considered usually when the haematocrit is >65%IIaC
Combination therapy may be considered in patients with Eisenmenger's syndromeIIbC
The use of CCBs is not recommended in patients with Eisenmenger's syndromeIIIC
  • ERA: endothelin receptor antagonist; WHO-FC: World Health Organization functional class; PA: pulmonary arterial; CCB: calcium channel blockers. #: class of recommendation; : level of evidence.