Table 3.
Clinical features |
Exclusion of other causes of PH |
● Chronic thromboembolism: angio-CT, V-Q scintigraphy |
● Left heart disease: echocardiography, right heart catheterization |
● Sleep breathing disorders |
Risk factors for PAH |
● HIV infection |
● BMPR2 mutations |
● Portal hypertension |
● Connective tissue disease |
Pulmonary function testing |
● Forced spirometry |
● CO diffusing capacity (DLCO) |
● Arterial blood gases: PaO2 and PaCO2 |
Imaging techniques |
● High-resolution CT scan: assessment of extent and severity of emphysema |
Right heart catheterization |
● Mean PAP |
● Cardiac index |
Cardiopulmonary exercise testing |
● Cardiovascular limitation pattern |
● Preserved ventilatory response |
● Gas exchange inefficiency: low end-tidal CO2, high VE/VCO2 slope |
Circulating biomarkers |
● BNP or NT-proBNP |
Abbreviations: PH, pulmonary hypertension; angio-CT, computed tomography angiogram; V-Q, ventilation and perfusion; PAH, pulmonary arterial hypertension; HIV, human immunodeficiency virus; BMPR2, bone morphogenetic protein receptor type 2; PAP, pulmonary artery pressure; VE, minute ventilation; VCO2, carbon dioxide output; BNP, brain natriuretic peptide; NT-proBNP, N-terminal prohormone of brain natriuretic peptide.