Chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by obstruction of large pulmonary arteries by acute or recurrent emboli, organisation of these clots, and vascular remodeling of occluded as well as non-occluded peripheral arteries. Up to 4% of patients surviving from an acute embolic event will eventually develop chronic pulmonary hypertension. Major goals of the diagnostic work-up of pulmonary hypertension include the determination of its cause, the evaluation of its functional and haemodynamic repercussions, and if thromboembolic disease is present, the exact mapping of the pulmonary vascular bed obstruction. Pulmonary endarterectomy is the treatment of choice for selected patients. Therapeutic alternatives include lung or heart-lung transplantation, pulmonary angioplasty and pharmacological treatment with pulmonary vasodilators.