Diagnosis and assessment of pulmonary arterial hypertension / 대한내과학회지

ABSTRACT

The diagnosis and assessment of pulmonary arterial hypertension (PAH) is a rapidly evolving area, with changes occurring in the definition of the disease, screening and diagnostic techniques, and staging and follow-up assessment. After 4th World Symposium on pulmonary hypertension (PH) which took place in Dana Point in early 2008, the definition of PH has been simplified (mean pulmonary artery pressure > or =25 mmHg) based on currently available evidences. The diagnosis of PH involves two stages: detection (determining the cause of a patient's symptoms, or to detect the presence of PAH in a high-risk patient) and characterization (determining the specific clinical context of the PH, including causal factors, associated diseases or substrates and hemodynamic perturbations). Echocardiography and right heart catheterization have been main diagnostic method in PAH. Also, there has been progress in imaging techniques and biomarkers used to screen patients for the disease and to follow up their response to therapy. Useful tools to predict outcome include functional class, exercise capacity, pulmonary hemodynamics, acute vasoreactivity, right ventricular function, as well as brain natriuretic peptide, endothelin-1, and so on. As new therapies have been developed for PAH, screening, prompt diagnosis, and accurate assessment of disease severity have become increasingly important. A clear definition of PH and the development of a rational approach to diagnostic assessment and follow-up using both conventional and new tools will be essential to advance proper treatment of patients.