Diuretics are indicated in patients with PAH who have signs of right-sided heart volume overload, as evidenced by lower extremity edema, ascites, hepatic congestion, or elevated jugular venous pressure. As patients with right-sided heart failure are preload dependent, however, caution must be used. Hypovolemia should be avoided, as this can lead to a drop in cardiac output and hemodynamic compromise.
For patients who progress or are poorly responsive to initial therapy, practitioners typically add agents from a different class. Agents within the same class (including phosphodiesterase-5 inhibitors and guanylate cyclase stimulators) should not be used together.
To decrease the risk of developing pneumonia (which is a recognized cause of death in patients with PAH), patients should receive vaccinations against influenza and pneumococcal pneumonia.
Balloon atrial septostomy involves creation of an atrial right-to-left shunt by graded balloon dilation of the atrial septum. The technique has been performed via a femoral catheter, with a Brockenbrough septal needle and Mansfield balloons to dilate the septostomy. This has been used with success in carefully selected patients who are symptomatic despite maximal medical therapy. The benefit (improved exercise function) occurs at the cost of a fall in arterial oxygen saturation. It is important to note that this should only be regarded as a palliative or bridging procedure (before transplantation), and is not part of recommended PAH treatment guidelines at this time.
Read more on the treatment of PAH.
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Cite this: Zab Mosenifar. Fast Five Quiz: Pulmonary Arterial Hypertension - Medscape - Jun 14, 2019.