A new study, presented at the American Association for Thoracic Surgery annual meeting, found that patients treated surgically for segmental chronic thromboembolic pulmonary hypertension (CTEPH) had positive outcomes with the majority doing well in the long term.

In addition, the study found that the proportion of CTEPH patients with segmental disease increased dramatically during the study period – from 2005 to 2020. At the beginning of the study, roughly seven percent of patients were diagnosed with segmental disease. During the last five years of the study, the proportion rose to an average of 41% of patients.

Pulmonary endarterectomy (PEA) shows success in treating the condition and potentially offering a cure for both proximal and segmental disease. The study performed a comprehensive analysis of all CTEPH patients undergoing PEA at University of Toronto between August 2005 and March 2020. Follow-up was completed for all patients. Disease located at the segmental level and more distally was defined as Jamieson type 3 and compared to more proximal disease defined as Jamieson type 1 and 2 (Type 1-2).

At 12 months, 76% of patients with segmental disease who underwent PEA were returned to virtually normal function, with the remaining 24 % requiring some degree of pulmonary hypertension (PH) therapy and/or balloon angioplasty. Anecdotally, the study found that patients who were treated with PH therapy preoperatively were more likely to require continued PH therapy post-operatively.

According to Marc de Perrot, director of the CTEPH program at University of Toronto, while the proportion of patients with segmental disease has increased dramatically, PEA results in excellent long-term outcomes. “Segmental CTEPH is a surgical disease,” explained de Perrot. “With surgery, patients show 80% long-term survival and good quality of life. The most important factor is a multidisciplinary approach to reassess and provide additional treatment post-operatively if necessary.”