Table 1. Tools and variables for detecting disease progression
Clinical assessment
    Symptoms (NYHA or WHO functional class)
    Rate of progression
    PAH aetiology
    Signs of right ventricular failure
Functional (exercise) capacity
    6-min walk distance
    Cardiopulmonary exercise test
        V′O2,peak
        V′E/V′CO2 slope
        Changes in blood pressure
Evaluation of right ventricular (dys)function
    Right heart catheterisation
        Right atrial pressure
        Cardiac index
        Cardiac output
    Echocardiography
        Pericardial effusion
        Tei index (or myocardial performance index)
        Tricuspid annular plane systolic excursion
        Right atrial area
        Left ventricular eccentricity
    Biomarkers
        NT-proBNP/BNP
        Troponin
        Uric acid
        Sodium
    Cardiac magnetic resonance imaging
        Right ventricular size and function
        Cardiac output
        Stroke volume

  • NYHA: New York Heart Association; WHO: World Health Organization; PAH: pulmonary arterial hypertension; V′O2,peak: peak oxygen uptake; V′E: minute ventilation; V′CO2: carbon dioxide production; NT-proBNP: N-terminal pro-brain natriuretic protein; BNP: brain natriuretic protein.