Breathlessness in PAH and scleroderma overview

Dr David Michail, Consultant Respiratory and Sleep Physician at Westmead Hospital, Sydney, provides an overview on breathlessness and PAH and discuss self-management options to help reduce the impact on day to day life. Dr. Michail will also provide an overview of scleroderma.

Pulmonary hypertension (PH) is a condition characterised by high blood pressure in the arteries of the lungs. It can be caused by various factors, including heart disease, lung disease, and blood clots. Diagnosing PH involves several tests, such as an echocardiogram and right heart catheterisation, to measure heart pressures and confirm the diagnosis.

Breathlessness is a common and distressing symptom of PH. It can result from increased ventilation needs due to lung or heart disease. The perception of breathlessness varies among individuals and can be influenced by neural pathways, emotions, and memories. Effective management of breathlessness includes both pharmacological and non-pharmacological approaches.

Pharmacological treatments for PH target three main pathways: endothelin, nitric oxide, and prostacyclin. Common medications include ambrisentan, macitentan, bosentan, sildenafil, tadalafil, and riociguat. In severe cases, intravenous medications may be used. It’s important to manage other factors like oxygen therapy, blood thinners, and psychosocial support.

Non-pharmacological strategies for managing breathlessness include using portable fans, practicing breathing techniques, and adopting energy conservation methods. Relaxed breathing, paced breathing, and pursed-lip breathing can help reduce breathlessness. Positioning techniques, such as leaning forward, can also be beneficial.

Exercise and physical activity are crucial for improving breathlessness and preventing deconditioning. Patients are encouraged to participate in exercise programs and use walking aids if needed. Nutrition is also important, as maintaining a healthy weight and muscle strength can improve overall quality of life.

For patients with systemic sclerosis, annual screening for PH is recommended, even if they are asymptomatic. Early detection and treatment can improve long-term outcomes. PH should be managed in specialised centres to ensure optimal care and risk assessment.

Overall, managing PH involves a combination of medical treatments, lifestyle adjustments, and support from healthcare providers and loved ones. Patients are encouraged to communicate openly with their healthcare team and seek support from organisations like the Lung Foundation.