Therapeutic alternatives in chronic thromboembolic pulmonary hypertension: from pulmonary endarterectomy to balloon pulmonary angioplasty to medical therapy. State of the art from a multidisciplinary team

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease with a very complex pathophysiology differing from other causes of pulmonary hypertension (PH). It is an infrequent consequence of acute pulmonary embolism that is frequently misdiagnosed. Pathogenesis has been related to coagulation abnormalities, infection or inflammation, although these disturbances can be absent in many cases. The hallmarks of CTEPH are thrombotic occlusion of pulmonary vessels, variable degree of ventricular dysfunction and secondary microvascular arteriopathy. The definition of CTEPH also includes an increase in mean pulmonary arterial pressure of more than 25 mmHg with a normal pulmonary capillary wedge of less than 15 mmHg. It is classified as World Health Organization group 4 PH, and is the only type that can be surgically cured by pulmonary endarterectomy (PEA). This operation needs to be carried out by a team with strong expertise, from the diagnostic and decisional pathway to the operation itself. However, because the disease has a very heterogeneous phenotype in terms of anatomy, degree of PH and the lack of a standard patient profile, not all cases of CTEPH can be treated by PEA. As a result, PH-directed medical therapy traditionally used for the other types of PH has been proposed and is utilized in CTEPH patients. Since 2015, we have been witnessing the rebirth of balloon pulmonary angioplasty, a technique first performed in 2001 but has since fallen out fashion due to major complications. The refinement of such techniques has allowed its safe utilization as a salvage therapy in inoperable patients. In the present keynote lecture, we will describe these therapeutic approaches and results.

Automated summary

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and complex disease that differs from other causes of pulmonary hypertension (PH). It is associated with coagulation abnormalities, infection or inflammation, although these disturbances may not be present in many cases. CTEPH is characterized by thrombotic occlusion of pulmonary vessels, varying degrees of ventricular dysfunction, and secondary microvascular arteriopathy. It is classified as World Health Organization group 4 PH and can be surgically cured by pulmonary endarterectomy (PEA). However, not all CTEPH cases can be treated with PEA due to the heterogeneity of the disease. PH-directed medical therapy traditionally used for other types of PH has been proposed and utilized in CTEPH patients. Balloon pulmonary angioplasty, a technique first performed in 2001, has seen a revival as a salvage therapy in inoperable patients. In this keynote lecture, the therapeutic approaches and results of these treatments will be described.