TY - JOUR T1 - Chronic thromboembolic pulmonary hypertension: do we need a new definition? JF - European Respiratory Journal JO - Eur Respir J SP - 1401 LP - 1403 DO - 10.1183/09031936.00177514 VL - 44 IS - 6 AU - Marc de Perrot AU - Eckhard Mayer Y1 - 2014/12/01 UR - http://erj.ersjournals.com/content/44/6/1401.abstract N2 - Chronic thromboembolic pulmonary hypertension (CTEPH) is the end result of persistent obstruction of the pulmonary arteries following episodes of acute and/or recurrent pulmonary emboli [1]. Pulmonary hypertension probably stems from a smaller pulmonary arterial cross sectional area due to thrombus organisation and a redistribution of blood flow through the patent pulmonary arterial bed producing an additional microvasculopathy [2]. Consequently many patients develop pulmonary hypertension despite adequate anticoagulation [3]. Once pulmonary hypertension occurs, the prognosis is poor due to right heart failure with a survival similar to other forms of pre-capillary pulmonary hypertension [4]. Patients suffering from CTEPH typically present with progressive dyspnoea that worsens over a period of months to years [5]. Dyspnoea is related to hypoxaemia, dead space ventilation and right heart dysfunction, severely affecting the quality of life [6, 7].Pulmonary endarterectomy is the treatment of choice for CTEPH. The surgery leads to major clinical improvement due to improved haemodynamic parameters and oxygenation, and a reduction in dead space ventilation [7, 8]. The surgery is curative in a large proportion of patients with resolution of the pulmonary hypertension. Over the past 20 years, the technique of pulmonary endarterectomy under deep hypothermic circulatory arrest has been refined and can currently be performed … ER -