James R. Klinger, MD

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.

Careful monitoring of disease progression is vital to ensuring that patients with pulmonary arterial hypertension receive maximal therapy before the onset of overt right-sided heart failure. Routine follow-up includes the evaluation of symptoms, functional class, and exercise capacity and assessment of pulmonary pressures and right ventricular (RV) function. Transthoracic echocardiography (TTE) offers a noninvasive and fairly reliable technique for monitoring pulmonary artery pressure (PAP) and structural changes of the right side of the heart. However, TTE does not reliably assess cardiac output, right-sided filling pressures, or pulmonary venous pressure. Pulmonary artery catheterization may be particularly useful in patients who have inconsistent findings, such as a reduction in PAP measured by TTE in the presence of worsening symptoms or other signs of disease progression. An increase in RV end-diastolic pressure, usually above 10 mm Hg, is a concern and warrants consideration of additional therapy even if other hemodynamic and clinical parameters are unchanged. (J Respir Dis. 2009;30(1-2)