Diagnostiek en behandeling van pulmonale hypertensie

• Pulmonary hypertension is defined as a mean pulmonary artery pressure of more than 25 mmHg.
• There are many possible causes of pulmonary hypertension; pulmonary hypertension has a poor prognosis, irrespective of the underlying cause.
• The most frequent causes of pulmonary hypertension are left heart failure and lung disease.
• The diagnostic work up of pulmonary hypertension starts with investigations into left heart failure and lung disease.
• If these reveal no cause, ventilation perfusion scintigraphy should be carried out so that chronic thrombo-embolic pulmonary hypertension can be demonstrated or excluded.
• In most cases, chronic thrombo-embolic pulmonary hypertension can be treated curatively by means of pulmonary endarterectomy.
• If chronic thrombo-embolic pulmonary hypertension is also ruled out, then we speak of pulmonary arterial hypertension.
• Prostacyclins, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and soluble guanylyl cyclase stimulators have been shown to be effective in patients with pulmonary arterial hypertension. This condition should be treated at specialist centres.

Conflict of interest and financial support: ICMJE forms provided by the authors are available online along with the full text of this article. Current research into pulmonary hypertension is financed by a grants from NWO (VIDI grant 917.96.306) and CVON (PHAEDRA).