Treatment options for pulmonary arterial hypertension

In this video Cardiologist A/Prof Rachael Cordina discuss the treatment options currently available for Pulmonary Arterial Hypertension (PAH) and the difference between monotherapy versus combination therapy. The PAH Patient Charter outlines the standards of holistic care that you should expect to receive. This includes your healthcare team listening to your preferences and involving you in decision-making, and being informed of the full range of evidence-based treatment options available to you. Pulmonary arterial hypertension (PAH) treatments primarily come in tablet form, with three different drug classes available. Patients may be prescribed up to three types of medications. In more advanced cases, nebulised medications or continuous treatments administered through a drip may be recommended. Although using a pump for continuous medication might seem daunting, most patients manage well with the right training.

For other types of pulmonary hypertension, such as those associated with severe lung disease or low oxygen levels, long-term home oxygen therapy might be necessary. Conditions like sleep apnoea should be managed with a CPAP machine to help control pulmonary pressures. It’s also important to optimise other heart conditions, such as atrial fibrillation, and manage fluid balance with diuretics.
In the past decade, the number of available PAH medications has significantly increased. Recent trials have shown that using multiple drugs early in treatment is more effective in reducing disease progression, the need for transplantation, and the risk of death. Therefore, patients with idiopathic pulmonary hypertension or connective tissue disease-related PAH are often started on more than one drug early in their treatment.

If a patient is frail, has other health conditions, or has mild disease, a single drug may be introduced initially. However, if there is no significant improvement in walking distances, heart function, or blood tests, a third tablet may be added quickly. In high-risk cases, doctors might recommend adding a continuous drip medication, which is the most powerful therapy available. Overall, early treatment with multiple medications can improve outcomes for PAH patients.