Pulmonary hypertension comprises a diverse group of conditions that lead to increased right ventricular afterload. Management and prognosis vary according to the cause. Patients at high risk (e.g. those with systemic sclerosis) or with unexplained progressive dyspnoea should be screened with echocardiography. Early referral to a specialist centre is key to timely investigation and management.
- The prognosis and management of pulmonary hypertension vary, depending on the underlying cause.
- Diagnosis relies on systematic evaluation of patients with dyspnoea and screening of those at high risk (e.g. patients with systemic sclerosis, congenital heart disease or previous pulmonary embolism).
- Specialist referral centres provide access to individualised work-up and management for patients; early referral is key to optimising outcome.
- Novel drug therapies and pulmonary vascular interventions are available for specific subgroups of pulmonary hypertension, such as pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension; however, for most patients, treatment targets the underlying condition.