Management of chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe and progressive disease. Three treatment modalities are available: pulmonary endarterectomy, balloon pulmonary angioplasty, and pulmonary hypertension (PH) medical therapy. Both mechanical therapies may also be considered in patients with chronic thromboembolic pulmonary disease without PH. Patients should be referred to expert centers, as full surgical evaluation is the primary target and further evaluation for additional or alternative treatment strategies by an experienced multidisciplinary team is mandatory. Surgery remains the treatment of choice for CTEPH. PH medical therapies are available for inoperable patients, or those with residual PH postprocedure. Furthermore, interventional therapy is recommended in inoperable patients with appropriate target lesions. Multimodal treatment strategies are common in inoperable patients. In recent years, there is a growing expertise in combining all 3 modalities in carefully selected patients. In the current era, the peri-interventional mortality risks are low (<3% for pulmonary endarterectomy and <1% for balloon pulmonary angioplasty in expert centers), and outcomes are excellent for the vast majority of patients with CTEPH.