A Practical Clinical Approach to the Diagnosis and Treatment of Patients with Pulmonary Hypertension

Pulmonary hypertension is defined by a mean pulmonary artery pressure of >25 mmHg at rest or 30 mmHg during exercise. There are many causes and currently diseases causing the condition are classified into five groups. The greatest elevation in pulmonary arterial pressure is found among those disorders in group 1 (known as pulmonary arterial hypertension [PAH]) and research and targeted therapy has focused on this group in particular, although patients in group 4 (chronic thromboembolic PH [CTEPH]) also receive advanced pulmonary vasodilator therapy. The symptoms of PH are often vague and the diagnosis is frequently missed or delayed. Efforts are therefore being made to improve awareness of PH among clinicians to enable prompt referral to a PH unit to confirm the diagnosis and instigate appropriate therapy. Multi-disciplinary team (MDT) discussion is necessary if patients with PH require surgical intervention or become pregnant. For patients in the other PH groups, treatment is usually concentrated on the primary disorder. A small number of patients with PAH will respond to calcium-channel-blocking agents. Specific targeted therapy is often given in combination depending on the patients functional performance status. Available agents include phosphodiesterase type V inhibitors, endothelin receptor antagonists, prostglandin analogues and nitric oxide. Many novel agents are under review. For carefully selected patients surgical options, include lung transplantation, pulmonary thromboendarterectomy and atrial septostomy.