What is the role of long-term monitoring in patients with idiopathic pulmonary arterial hypertension (IPAH)?

Updated: Jul 08, 2020
  • Author: Ronald J Oudiz, MD, FACP, FACC, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

Currently, no precise dosage adjustment algorithm is available for patients with IPAH who are on PAH-specific therapy. Monitor the patient with frequent physical examinations and focus the history on heart failure symptoms and adverse effects of medications.

Echocardiography has been used in several studies to serially monitor changes in the right ventricular–right atrial pressure gradient and the right and left ventricular chamber sizes. Findings from other noninvasive modalities (eg, electron-beam CT measurements of cardiac chamber sizes) correlate with hemodynamic improvements in pulmonary physiology.

More recently, cardiopulmonary exercise testing, serial invasive hemodynamic testing, and 6-minute walk testing have been used to monitor the disease status of patients with IPAH.


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