Table 11—

Recommendations for right heart catheterisation(RHC; A) and vasoreactivity testing (B)

 RHC is indicated in all patients with PAH to confirm the diagnosis, to evaluate the severity and when PAH specific drug therapy is consideredIC
 RHC should be performed for confirmation of efficacy of PAH-specific drug therapyIIaC
 RHC should be performed for confirmation of clinical deterioration and as baseline for the evaluation of the effect of treatment escalation and/or combination therapyIIaC
 Vasoreactivity testing is indicated in patients with IPAH, heritable PAH and PAH associated with anorexigen use to detect patients who can be treated with high doses of a CCBIC
 A positive response to vasoreactivity testing is defined as a reduction of pa ≥10 mmHg to reach an absolute value of pa ≤40 mmHg with an increased or unchanged COIC
 Vasoreactivity testing should be performed only in referral centresIIaC
 Vasoreactivity testing should be performed using nitric oxide as vasodilatorIIaC
 Vasoreactivity testing may be performed in other types of PAHIIbC
 Vasoreactivity testing may be performed using i.v. epoprostenol or i.v. adenosineIIbC
 The use of an oral or i.v. CCB in acute vasoreactivity testing is not recommendedIIIC
 Vasoreactivity testing to detect patients who can be safely treated with high doses of a CCB is not recommended in patients with other PH groups (groups 2, 3, 4 and 5)IIIC
  • PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; CCB: calcium channel blocker; pa: mean pulmonary arterial pressure; CO: cardiac output; PH: pulmonary hypertension. #: class of recommendation; : level of evidence.