High risk features in pulmonary arterial hypertension impacting agent selection*
High risk features in pulmonary arterial hypertension impacting agent selection*
Clinical and echocardiographic¶ evidence of RV failure
PaCO2 <32 mmHg
RAp >20 mmHg
CI <2 L/min/m2
Rapid disease progression
WHO functional class III/IV
6MWD <300 meters
Peak VO2 <10.4 mL/kg/min
Elevated BNP/NT-proBNP
RV: right ventricle; PaCO2: partial arterial pressure of carbon dioxide; RAp: right atrial pressure; CI: cardiac index; WHO: World Health Organization; 6MWD: 6-minute walk distance; VO2: oxygen consumption on cardiopulmonary exercise testing; BNP: brain natriuretic peptide; NT-pro BNP: N-terminal pro-brain natriuretic peptide; CTD: connective tissue disease; PAH: pulmonary arterial hypertension. * While factors such as age >50 years, CTD PAH, and failure to improve or clinical worsening during treatment do not affect initial agent selection, they may influence the decision to add or change therapy. ¶ Examples include pericardial effusion, RV dysfunction, and right atrial enlargement.