Patient discussions

Low-level graded aerobic exercise (e.g., walking) should be encouraged, and supervised exercise training is recommended.[3]​​[35][56][58]​​[59] [ Cochrane Clinical Answers logo ] ​​​​​ There is a lack of evidence for a direct impact of exercise training on survival and outcome in pulmonary hypertension. However, there are studies showing a beneficial effect on prognostically important parameters. The European Respiratory Society has identified a strong need to establish specialised rehabilitation programmes for patients with PAH to enhance access to this treatment intervention, which appears to be effective, cost-efficient and safe.[58]​ Patients should avoid heavy physical activity, isotonic exercises, and high altitudes. Some patients may require oxygen on commercial aircraft; European Society of Cardiology/European Respiratory Society guidelines recommend in-flight oxygen administration for patients using oxygen or whose arterial blood oxygen pressure is <8 kPa (60 mmHg) at sea level.[3]

Patients should follow a sodium-restricted diet.[105]​ Electrolytes should be monitored in patients receiving diuretics, particularly at the onset of therapy and after changes in dosing.

Recommended immunisations against influenza, coronavirus disease 2019 (COVID-19), and pneumococcal pneumonia should be given.

Patients should avoid vasoconstricting sinus or common cold preparations and anorexigens.

Pregnancy should be avoided, and women of child-bearing age should be counselled about the risks associated with becoming pregnant.[3][35]​ Those who present during pregnancy, or who become pregnant, should be treated by a multidisciplinary team experienced in managing pulmonary hypertension in pregnancy.

The risk-benefit ratio of elective surgery needs to be carefully considered.​[106]

Patients may need psychosocial support, including advanced care planning with referral to specialist palliative care services at the right time.[3]

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