Medications for PAH

Treatment options for Pulmonary Arterial Hypertension (PAH) can help slow the progression of the disease and improve symptoms. It is important to work with your specialist to create a treatment plan that is right for you.  

We spoke with Tracy Tse the respiratory pharmacy team leader at the Prince Charles Hospital about the different types of medications for PAH. Watch the webinar video or read more below. 

Treatment goals for PAH 

Pulmonary Arterial Hypertension (PAH) is caused by narrowing or blockage of the blood vessels (arteries) that carry blood from the heart to the lungs. This reduces the flow of blood and oxygen through the lungs which increases the pressure in these blood vessels. The heart needs to pump against the higher pressure to keep blood flowing through the lungs, making it harder for the heart to work. The goal of PAH medications is to prevent further damage to your pulmonary arteries and to improve symptoms. In many people the medications also dilate the lung blood vessels to improve blood flow. 

Treatment options 

Everyone’s disease is different, and treatment options will depend on the cause of PAH and the stage at which it is diagnosed. The treatment options for PAH have changed dramatically over the last ten years with the introduction of several new medications and treatments. Although there is currently no cure for this disease, it can often be well managed for many years with medications. 

Ongoing monitoring of your condition is required, such as regular blood tests to monitor haemoglobin, liver function, heart stress (measured with BNP) and iron levels. As a progressive disease, PAH will generally worsen over time without treatment and so early diagnosis and treatment is important. Taking your treatment as prescribed can help slow disease progression and improve symptoms. Different treatments have different side effects. 

There are several different medicines that PAH patients can take to open up the blood vessels to the lungs and reduce the work for the heart. These are usually taken as tablets but can also be given by intravenous infusion (into the vein) or breathed in as a mist (via an inhaler). Most people will be prescribed more than one medicine because combination therapy is more effective. 

Prostacyclins 

Prostacyclins help the blood vessels relax by mimicking prostaglandin, a substance in the blood that is often low in PAH patients. Treatments in this group include epoprostenol and iloprost.  

• Epoprostenol: given as a continuous drip into the vein.  
• Iloprost: taken through a nebuliser and breathed in five or six times a day. 

Endothelin receptor antagonists (ERAs) 

People with PAH have too much endothelin in their blood. ERAs block endothelin, a substance that helps the blood vessels constrict. Medications in this group are: macitentan, bosentan and ambrisentan. These tablets are taken every day.  

Phosphodiesterase type five (PDE-5) inhibitors 

PDE-5 inhibitors allow the lungs to make more of their own natural vasodilators, which relax the blood vessels and allow more blood to flow. These treatments include sildenafil and tadalafil which are taken every day. 

Soluble guanylate cyclase stimulators (sGC stimulators)  

sGC stimulators help in chemical reactions that improve blood flow in the lungs. This type of treatment includes a tablet called riociguat.  

Supportive therapy: 

Other medications can be used as supportive medications, and may include fluid tablets, blood thinners and medications to help relieve symptoms. 

Diuretics 

If the patient has fluid retention, diuretics remove excessive fluid. It is important to continue adhering to the food and salt restriction to make sure that it works effectively. Some of the examples include frusemide, bumetanide, or spironolactone. 

Iron Supplements 

A lot of people living with PAH have an iron deficiency which can reduce exercise capacity. Iron study is recommended every six months. 

Calcium channel blocker  

Only a small amount of people with PAH will need calcium channel blockers. These drugs help encourage more blood flow by relaxing blood vessel walls. Options can include Amlodipine and nifedipine. 

If you would like information and support on PAH click here or phone freecall 1800 654 301. 

Please note: the information in this blog is of general nature only. Speak to your treating healthcare team for specific advice on your health and wellbeing.