Table 33—

Recommendations for chronic thromboembolic pulmonary hypertension(CTEPH)

The diagnosis of CTEPH is based on the presence of pre-capillary PH (pa ≥25 mmHg, Ppcw15 mmHg, PVR >2 Wood units) in patients with multiple chronic/organised occlusive thrombi/emboli in the elastic pulmonary arteries (main, lobar, segmental, subsegmental)IC
In patients with CTEPH lifelong anticoagulation is indicatedIC
Surgical pulmonary endarterectomy is the recommended treatment for patients with CTEPHIC
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 endarterectomyIiaC
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 comorbiditiesIiaC
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 endarterectomyIibC
  • PH: pulmonary hypertension; pa: 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.