Life with pulmonary arterial hypertension

In this video Dr Zoe Brown, Rheumatologist, answer our community’s questions about Pulmonary Arterial Hypertension (PAH).

PAH can develop in up to 10% of patients with connective tissue diseases, such as rheumatoid arthritis. Early diagnosis is crucial, and annual reviews with echocardiography can help detect PAH early. Exercise is an important part of treatment, and supervised exercise programs or pulmonary rehabilitation can help manage breathlessness and improve exercise capacity. These programs typically run for six to eight weeks and may include one-on-one or group sessions.

Supplemental oxygen therapy can benefit those with low oxygen levels in the blood, improving exercise capacity and quality of life. However, if oxygen levels are adequate, additional oxygen may not be necessary. For patients with PAH, especially those with low oxygen levels during exercise, oxygen therapy can reduce breathlessness.

Pregnancy poses significant risks for women with PAH due to the extensive physiological changes that occur. The mortality rate for pregnant women with PAH remains high, so it is important to discuss family planning and contraception with healthcare providers. If a woman with PAH becomes pregnant, she should be linked with a specialist centre experienced in managing PAH during pregnancy. Certain PAH therapies may need to be stopped, and the timing and mode of delivery will be closely managed to minimise risks.

Following a PAH diagnosis, treatment with one or two drug therapies will be based on a comprehensive assessment of the patient’s risk level. Initial management includes advice on physical activity, supervised rehabilitation, and supportive therapies such as blood thinners and diuretics. Drug treatments target the narrowing of blood vessels in the lungs, with medications available to promote vessel opening and reduce pressure.

Oral treatments for PAH in Australia include phosphodiesterase 5 inhibitors (sildenafil and tadalafil) and endothelin receptor antagonists (macitentan and bosentan). These treatments have been shown to improve exercise capacity, heart function, and survival.