Patient discussions

Low-level graded aerobic exercise (e.g., walking) should be encouraged, and supervised exercise training is recommended.[3]​​[35][57][59]​​[60] [ 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 specialized rehabilitation programs for patients with PAH to enhance access to this treatment intervention, which appears to be effective, cost-efficient and safe.[59]​ 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.[106]​ Electrolytes should be monitored in patients receiving diuretics, particularly at the onset of therapy and after changes in dosing.

Recommended immunizations 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 childbearing age should be counseled 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.​[107]

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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