
Despite the recent development of several new therapies, pulmonary arterial hypertension (PAH) remains an incurable disease. Careful monitoring of disease progression is vital to ensuring that patients receive maximal medical therapy before the onset of overt right-sided heart failure. In part 1 of this article, I reviewed the role of the history and physical examination, chest radiography, electrocardiography, echocardiography, and pulmonary artery catheterization. In part 2, I focus on MRI, cardiopulmonary exercise testing (CPET), the 6-minute walk test, and biomarkers.