Pulmonary Endarterectomy (PEA)
PEA is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension (CTEPH, group 4) as it is a potentially curative option [Class of recommendation I, Level of evidence C]. As a rule, a patient should not be considered inoperable as long as the case has not been reviewed by a multidisciplinary and surgical team with experience in this particular intervention. A centre can be considered to have sufficient expertise in the field if it performs at least 20 PEA operations per year with mortality rate < 5%. The selection of patients for surgery should be based on the extent and location of the organized thrombi, on the degree of PH, and on the presence of co-morbidities. PAH speciﬁc drug therapy may be indicated in selected CTEPH patients such as patients not candidates for surgery, patients with residual PH after pulmonary endarterectomy or if pre-operative treatment is deemed appropriate to improve hemodynamic.
Balloon atrial septostomy (BAS)
The creation of an interatrial right-to-left shunt can decompress the right heart chambers, and increase left ventricle preload and cardiac output. The recommended technique is graded balloon dilation atrial septostomy. This intervention is palliative. BAS should be avoided in end-stage patients presenting with a baseline mean RAP of > 20 mmHg and O₂ saturation adt rest of < 80% on room air.
Double lung transplantation (or heart-lung transplantation) should be considered for PAH patients deteriorating under maximal specific drug therapy or with pulmonary veno-occlusive disease (PVOD, group 1’).