Risk Stratification at Early Follow-Up Determines PAH Prognosis

Study Questions:

Guidelines recommend a goal-oriented treatment approach in pulmonary arterial hypertension (PAH). To what degree does reaching a low-risk profile following treatment predict a good outcome?


A total of 530 PAH patients were included in the study. The great majority were idiopathic (IPAH), associated with connective tissue disease (APAH-CTD) or congenital heart disease. Follow-up assessments performed after a median of 4 (interquartile range, 3–5) months were available for 383 subjects. Patients were classified as “low risk”, “intermediate risk”, or “high risk” and the benefit of reaching the “low risk” group was estimated utilizing a number of low-risk parameters including World Health Organization functional class, 6-minute hall walk, N-terminal pro–B-type natriuretic peptide, echo right atrial (RA) area, presence of a pericardial effusion, and right heart catheterization mean right atrial pressure, cardiac index, and mixed venous oxygen saturation.


Survival differed (p < 0.001) between the risk groups at baseline and at follow-up. Survival was similar for patients who remained in or improved to the “low risk” group and similar for patients who remained in or worsened to the “intermediate risk” or “high risk” groups. Irrespective of follow-up risk group, survival was better (p < 0.001) for patients with a higher proportion of variables at low risk. Results were unchanged after excluding patients with IPAH >65 years at diagnosis, and when patients with IPAH or APAH-CTD were analyzed separately. Patients in the “low risk” group at follow-up exhibited a reduced mortality risk (hazard ratio, 0.2; 95% confidence interval, 0.1–0.4 in multivariable analysis adjusted for age, sex, and PAH subset), as compared with patients in the “intermediate risk” or “high risk” groups.


These findings suggest that comprehensive risk assessments and the aim of reaching a low-risk profile are valid in PAH.


The study validates the guidelines and supports the need for PAH patients to be evaluated in a PH center at least annually for risk stratification in the low-risk and more frequently in the intermediate- and high-risk patients.

Clinical Topics: Anticoagulation Management, Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Pericardial Disease, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Heart Failure and Cardiac Biomarkers, Pulmonary Hypertension

Keywords: Atrial Pressure, Cardiac Catheterization, Connective Tissue Diseases, Heart Defects, Congenital, Hypertension, Pulmonary, Natriuretic Peptide, Brain, Peptide Fragments, Pericardial Effusion, Primary Prevention, Prognosis, Risk Assessment

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