Pulmonary arterial hypertension treatment guidelines

Evidence-based treatment algorithm: adapted from the Fourth World Symposium on PH¹

Oral anticoagulants (E/B)— IPAH/HPAH Diuretics (E/A) Oxygen* (E/A) Digoxin (E/C) Supervised rehabilitation (E/B)		Supportive therapy and general measures		Avoid excessive physical exertion (E/A) Birth control (E/A) Psychological and social support (E/C) Infection prevention (E/A)
		Expert referral (E/A)
		Acute vasoreactivity test (A for IPAH) (E/C for APAH)
*To maintain oxygen at 92%.		NONRESPONDER

Strength of recommendation	WHO Class II	WHO Class III	WHO Class IV
A	Ambrisentan, Tracleer, sildenafil	Ambrisentan, Tracleer, epoprostenol IV, inhaled prostacyclin analog, sildenafil	Epoprostenol IV
B	Tadalafil	Tadalafil, treprostinil SC	Inhaled prostacyclin analog
C			Treprostinil SC
E/B		Treprostinil IV	Treprostinil IV
E/C			Ambrisentan, Tracleer, sildenafil, tadalafil

Notes on pulmonary arterial hypertension treatment algorithm

Drugs within the same grade of evidence are listed in alphabetical order by generic name and not order
of preference. Not all agents listed are approved or available in all countries.

Quality of evidence
Good	Evidence is based on good randomized, controlled trials or meta-analysis
Fair	Evidence is based on other controlled trials or randomized, controlled trials with minor flaws
Low	Evidence is based on nonrandomized, case-control, or other observational trials
Expert opinion	Evidence is based on the consensus of the carefully selected panel of experts in the topic field

Strength of recommendation
A	Strong recommendation
B	Moderate recommendation
C	Weak recommendation
D	Negative recommendation
I	No recommendation possible (inconclusive)
E/A	Strong recommendation on the basis of expert opinion only
E/B	Moderate recommendation on the basis of expert opinion only
E/C	Weak recommendation on the basis of expert opinion only
E/D	Negative recommendation on the basis of expert opinion only

Reflects therapies approved for PAH in the United States only.
Inhaled treprostinil was not approved at the time of publication.

Tracleer is indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) in patients with WHO Class II-IV symptoms, to improve exercise ability and decrease the rate of clinical worsening. Patients with WHO Class II symptoms showed reduction in the rate of clinical deterioration and a trend for improvement in walk distance. Physicians should consider whether these potential benefits are sufficient to offset liver injury in WHO Class II patients, which may preclude future use as their disease progresses.

Important safety information

Because of the associated risks, Tracleer may be prescribed only through the Tracleer Access Program.

Potential for serious liver injury (including, after prolonged treatment, rare cases of liver failure and unexplained hepatic cirrhosis in a setting of close monitoring)—Liver monitoring of all patients is essential prior to initiation of treatment and monthly thereafter.

High potential for major birth defects—Pregnancy must be excluded and prevented through the use of reliable forms of birth control; monthly pregnancy tests should be obtained.

Contraindicated for use with cyclosporine A and glyburide.

Please see full prescribing information including BOXED WARNING.

Read about patients at risk for PAH

1. Barst RJ, Gibbs JSR, Ghofrani HA, et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54(suppl):S78-S84.