Pulmonary arterial hypertension: features and management
Pulmonary arterial hypertension (PAH) may be defined as a sustained elevation in mean pulmonary arterial pressure of greater than 25 mmHg at rest or 30 mmHg after exercise.
Features
Management should first involve treating any underlying conditions, for example with anticoagulants or oxygen. Following this, it has now been shown that acute vasodilator testing is central to deciding on the appropriate management strategy. Acute vasodilator testing aims to decide which patients show a significant fall in pulmonary arterial pressure following the administration of vasodilators such as intravenous epoprostenol or inhaled nitric oxide
If there is a positive response to acute vasodilator testing
If there is a negative response to acute vasodilator testing
Features
- exertional dyspnoea is the most frequent symptom
- chest pain and syncope may also occur
- loud P2
- left parasternal heave (due to right ventricular hypertrophy)
Management should first involve treating any underlying conditions, for example with anticoagulants or oxygen. Following this, it has now been shown that acute vasodilator testing is central to deciding on the appropriate management strategy. Acute vasodilator testing aims to decide which patients show a significant fall in pulmonary arterial pressure following the administration of vasodilators such as intravenous epoprostenol or inhaled nitric oxide
If there is a positive response to acute vasodilator testing
- oral calcium channel blockers
If there is a negative response to acute vasodilator testing
- prostacyclin analogues: treprostinil, iloprost
- endothelin receptor antagonists: bosentan
- phosphodiesterase inhibitors: sildenafil