Sequential multimodal therapy in patients with chronic thromboembolic pulmonary hypertension (CTEPH)

Abstract

Background: Management of CTEPH has recently evolved with the availability of three therapeutic options: pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and pulmonary arterial hypertension (PAH)-targeted medical therapy. In rare cases, patients may have surgically accessible lesions on one side with contralateral surgically inaccessible lesions and high pulmonary vascular resistance (PVR). We investigated the feasibility and effects of a sequential multimodal therapy in these patients. Methods: Six consecutive CTEPH patients with mixed anatomical lesions and PVR>800 dyn.s.cm-5 underwent a sequential multimodal therapy combining medical therapy, BPA and PEA between 2017 and 2019. BPA was performed on the inoperable side and PEA was then applied to the contralateral lung. Results: Median age at diagnosis was 74 (63-78; 3 males) years. Four patients were treated with upfront dual oral combination therapy and 2 patients received monotherapy for 5 (2-5) months prior to BPA. The median number of BPA sessions before PEA was 2. The median time from medical therapy to PEA was 8 months. At post-PEA evaluation, 6-min walk distance improved from 304 (274-354) to 419 (368-440) m (p=0.06), mean pulmonary artery pressure decreased from 51 (46-64) to 28 (19-33) mmHg (-45%; p=0.03) and PVR from 1050 (932-1510) to 323.5 (218-377) dyn.s.cm-5 (-69%; p=0.03). Three patients discontinued medical treatment after surgery. No patients died. Conclusions: Sequential multimodal therapy combining medical therapy, BPA and PEA may be a new treatment option for selected high-risk CTEPH patients with mixed anatomical lesions.