Pulmonary hypertension can be a devastating disease that is easily missed in the early stages because of its typically nonspecific presentation with gradually increasing dyspnoea. With advances in management, most forms of pulmonary hypertension are treatable. Early diagnosis and treatment are key to improving functional and haemodynamic outcomes and survival.
- Pulmonary hypertension (PH) is characterised by an elevation in pulmonary artery pressure at rest and presents with progressive breathlessness as the cardinal symptom.
- PH can be associated with more than 50 different conditions, which are categorised into five groups based on pathophysiological mechanisms; accurate classification is crucial to management as treatment options and goals vary between groups.
- As symptoms of PH can be vague or nonspecific, diagnosis is often delayed, with patients typically progressing to advanced disease before diagnosis.
- Identifying patients early in the disease course can improve functional and haemodynamic outcomes.
- Group 1 PH (pulmonary arterial hypertension [PAH]) is rare; if it is suspected then prompt referral of the patient to a specialist with an expert interest in PH is recommended.
- Long-term management of patients with PAH, Group 4 (chronic thromboembolic) or Group 5 (unclear or multifactorial) PH should be delivered or co-ordinated through a designated PH clinic or service.