Recommendations for chronic thromboembolic pulmonary hypertension(CTEPH)
| Statement | Class# | Level¶ |
| The diagnosis of CTEPH is based on the presence of pre-capillary PH (P̄pa ≥25 mmHg, Ppcw ≤15 mmHg, PVR >2 Wood units) in patients with multiple chronic/organised occlusive thrombi/emboli in the elastic pulmonary arteries (main, lobar, segmental, subsegmental) | I | C |
| In patients with CTEPH lifelong anticoagulation is indicated | I | C |
| Surgical pulmonary endarterectomy is the recommended treatment for patients with CTEPH | I | C |
| Once perfusion scanning and/or CT angiography show signs compatible with CTEPH, the patient should be referred to a centre with expertise in surgical pulmonary endarterectomy | Iia | C |
| The selection of patients for surgery should be based on the extent and location of the organised thrombi, on the degree of PH, and on the presence of comorbidities | Iia | C |
| PAH-specific drug therapy may be indicated in selected CTEPH patients such as patients not candidates for surgery or patients with residual PH after pulmonary endarterectomy | Iib | C |
PH: pulmonary hypertension; P̄pa: mean pulmonary arterial pressure; Ppcw: pulmonary capillary wedge pressure; PVR: pulmonary vascular resistance; CT: computed tomography; PAH: pulmonary arterial hypertension. #: class of recommendation; ¶: level of evidence.