State-of-the-Art Paper
Updated Evidence-Based Treatment Algorithm in Pulmonary Arterial Hypertension

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Uncontrolled and controlled clinical trials with different compounds and procedures are reviewed to define the risk-benefit profiles for therapeutic options in pulmonary arterial hypertension (PAH). A grading system for the level of evidence of treatments based on the controlled clinical trials performed with each compound is used to propose an evidence-based treatment algorithm. The algorithm includes drugs approved by regulatory agencies for the treatment of PAH and/or drugs available for other indications. The different treatments have been evaluated mainly in idiopathic PAH, heritable PAH, and in PAH associated with the scleroderma spectrum of diseases or with anorexigen use. Extrapolation of these recommendations to other PAH subgroups should be done with caution. Oral anticoagulation is proposed for most patients; diuretic treatment and supplemental oxygen are indicated in cases of fluid retention and hypoxemia, respectively. High doses of calcium-channel blockers are indicated only in the minority of patients who respond to acute vasoreactivity testing. Nonresponders to acute vasoreactivity testing or responders who remain in World Health Organization (WHO) functional class III, should be considered candidates for treatment with either an oral phosphodiesterase-5 inhibitor or an oral endothelin-receptor antagonist. Continuous intravenous administration of epoprostenol remains the treatment of choice in WHO functional class IV patients. Combination therapy is recommended for patients treated with PAH monotherapy who remain in WHO functional class III. Atrial septostomy and lung transplantation are indicated for refractory patients or where medical treatment is unavailable.

Key Words

algorithm
evidence-based treatment
pulmonary arterial hypertension

Abbreviations and Acronyms

CCB
calcium-channel blocker
ERA
endothelin receptor antagonist
ETA
endothelin receptor A
ETB
endothelin receptor B
HPAH
heritable pulmonary arterial hypertension
INR
international normalized ratio
IPAH
idiopathic pulmonary arterial hypertension
IV
intravenous
PAH
pulmonary arterial hypertension
PDE
phosphodiesterase
PH
pulmonary hypertension
RCT
randomized controlled trial
WHO
World Health Organization

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