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.