Life with Pulmonary Arterial Hypertension: Q & A with Dr Zoe Brown

In this session Dr Zoe Brown, Rheumatologist, answer our community’s questions about Pulmonary Arterial Hypertension (PAH). The session covers exercise and PAH, oxygen, medication and parenthood. Pulmonary arterial hypertension (PAH) is a serious condition affecting up to 10% of patients with connective tissue diseases. Early diagnosis through annual reviews and echocardiography is crucial. Exercise is vital for managing PAH, with supervised programs improving breathlessness and exercise capacity. Supplemental oxygen can benefit those with low blood oxygen levels, which can help to enhance exercise performance and quality of life. Pregnancy poses high risks for women with PAH, which can require careful management and counselling. It is important to dicsuss PAH and family planning with your healthcare team. Medications for PAH target blood vessel constriction, with treatments tailored based on risk levels. Ongoing research and specialised care are essential for the optimal management of PAH.

Pulmonary arterial hypertension treatments aim to improve exercise capacity, heart function, quality of life and survival. Oral phosphodiesterase inhibitors like sildenafil and tadalafil work on the nitric oxide pathway to dilate blood vessels, potentially improving heart function and preventing vessel thickening. These medications may cause side effects like flushing, dizziness, headache, and gastrointestinal upset. Endothelin receptor antagonists, such as macitentan and bosentan, block vessel constriction and prevent thickening, requiring regular monitoring for anaemia and liver function. The prostacyclin pathway, targeted by new oral agents like selexipag, also helps dilate blood vessels. It is important to discuss any side effects and potential changes with your healthcare professional. In Australia, selexipag is reserved for patients on combination therapy. PAH can result from heart and lung diseases, blood clots, or conditions like idiopathic pulmonary fibrosis and sarcoidosis. General care measures, including supplemental oxygen and pulmonary rehabilitation, can be essential for managing PAH, alongside addressing other health conditions like iron deficiency and thyroid disorders.