How should I manage a patient with confirmed pulmonary hypertension who is experiencing worsening symptoms?

Management of a patient with confirmed pulmonary hypertension (PH) who is experiencing worsening symptoms requires urgent referral to a specialist pulmonary hypertension centre for comprehensive assessment and tailored treatment, as PH is complex and often managed in secondary care NICE CKS.

Initial management includes confirming the diagnosis and assessing the severity and cause of PH, as symptoms can be non-specific and may overlap with other conditions NICE CKS. Specialist centres will perform detailed investigations such as right heart catheterisation, echocardiography, and functional assessments to guide therapy Ntiloudi et al. 2024.

Optimising treatment of any underlying or associated conditions is critical. For example, if PH is secondary to chronic lung disease such as COPD, ensure optimal management of the lung disease including smoking cessation, pharmacotherapy, and pulmonary rehabilitation NICE NG115. Diuretics may be used symptomatically to control oedema related to right heart failure, but specific pulmonary vasodilator therapies should be initiated only under specialist guidance NICE NG115.

For patients with worsening symptoms, specialist centres may consider advanced therapies including pulmonary vasodilators (e.g., endothelin receptor antagonists, phosphodiesterase-5 inhibitors), anticoagulation, or surgical options such as pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension (CTEPH) Delcroix et al. 2023Swisher & Weaver 2023.

Oxygen therapy should be considered if hypoxaemia is present, but ambulatory oxygen is only recommended after specialist assessment to determine benefit NICE NG115. Pulmonary rehabilitation is advised to improve exercise capacity and quality of life NICE CG163.

Symptom management, including relief of dyspnoea, may involve palliative care approaches such as low-dose opioids and psychosocial support, especially in advanced disease stages NICE CKS. Regular monitoring of functional status, fluid balance, and cardiac rhythm is important to detect complications such as arrhythmias or heart failure NICE CKS.

In summary, worsening symptoms in pulmonary hypertension necessitate urgent specialist referral for comprehensive evaluation and initiation or escalation of targeted therapies, alongside optimisation of comorbidities and supportive care measures NICE CKS; Ntiloudi et al. 2024Delcroix et al. 2023Swisher & Weaver 2023.