Cardiopulmonary exercise test (CPET) has been gaining importance as a method of
functional assessment in Brazil and worldwide. In its most frequent applications, CPET …

OBJECTIVE Choices of pharmacologic therapies for pulmonary arterial hypertension (PAH)
are ideally guided by high-level evidence. The objective of this guideline is to provide …

Rationale: The relationship between the initial treatment strategy and survival in pulmonary
arterial hypertension (PAH) remains uncertain. Objectives: To evaluate the long-term …

Rationale: Incident pulmonary arterial hypertension was underrepresented in most
pulmonary hypertension registries and may have a different disease profile to prevalent …

Objective Pulmonary arterial hypertension (PAH) is a severe, life‐limiting complication of
systemic sclerosis (SSc). Guidelines recommend early detection and management of SSc …

Exercise stresses the pulmonary circulation through increases in cardiac output (Q.) and left
atrial pressure. Invasive as well as noninvasive studies in healthy volunteers show that the …

Rationale: Patients with idiopathic pulmonary arterial hypertension (IPAH) present
circulating autoantibodies against vascular wall components. Pathogenic antibodies may be …

Rationale: Pulmonary hypertension (PH) associated with fibrotic idiopathic interstitial
pneumonia (IIP; idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia) …

Objective Pulmonary arterial hypertension (PAH) affects up to 15% of patients with
connective tissue diseases (CTDs). Previous recommendations developed as part of larger …

Rationale: Activin A receptor type II-like kinase-1 (ACVRL1, also known as ALK1) mutation is
a cause of hereditary hemorrhagic telangiectasia (HHT) and/or heritable pulmonary arterial …