ABSTRACT

Pulmonary hypertension (abnormally elevated pulmonary artery pressure; PH) is often identified during investigation of shortness of breath. Most often it is a result of underlying cardiac and chronic respiratory diseases. Although most commonly due to left heart disease, sometimes it is due to chronic pulmonary arterial disease (WHO Group 1 PH), for which targeted pulmonary vasodilators are efficacious. A systematic diagnostic approach is needed to determine the underlying diagnosis, leading to the optimal treatment, often in partnership with an expert PH centre.

KEY POINTS
    • Shortness of breath is a common presenting problem, for which pulmonary hypertension (PH) and its underlying causes should be considered.
    • The most common causes of PH in the community are left heart disease and lung disease.
    • Diagnostic criteria for PH have changed recently, with a lower mean pulmonary artery pressure of 20 mmHg now used to define PH.
    • GPs have an important role in the early detection, risk stratification and referral for further investigation and management of patients with suspected PH.
    • Although PH may be documented on transthoracic echocardiography, invasive right heart catheterisation is needed to diagnose Group 1 pulmonary arterial hypertension (PAH).
    • Specific pulmonary vasodilator therapy is only indicated for patients with Group 1 PAH and selected patients with Group 4 PH (chronic thromboembolic pulmonary hypertension; CTEPH).
    • Patients with suspected Group 1 PAH should be referred to a PH expert centre and those with suspected Group 4 PH referred to a specialist CTEPH centre.

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