Chronic thromboembolic pulmonary hypertension (CTEPH) is part of group 4 of the pulmonary hypertension (PH) classification and generally affects more than a third of patients referred to PH centers. It is a three-compartment disease involving proximal (lobar-to-segmental) and distal (subsegmental) pulmonary arteries that are obstructed by persistent fibrothrombotic material, and precapillary pulmonary arteries that can be affected as in pulmonary arterial hypertension. It is a rare complication of pulmonary embolism (PE), with an incidence of around 3% in PE survivors. The observed incidence of CTEPH in the general population is around six cases per million but could be three times higher than this, as estimated from PE incidence. However, a previous venous thromboembolic episode is not always documented. With advances in multimodality imaging and therapeutic management, survival for CTEPH has improved for both operable and inoperable patients. Advanced imaging with pulmonary angiography helps distinguish proximal from distal obstructive disease. However, right heart catheterization is of utmost importance to establish the diagnosis and hemodynamic severity of PH. The therapeutic strategy relies on a stepwise approach, starting with an operability assessment. Pulmonary endarterectomy (PEA), also known as pulmonary thromboendarterectomy, is the first-line treatment for operable patients. Growing experience and advances in surgical technique have enabled expansion of the distal limits of PEA and significant improvements in perioperative and mid- to long-term mortality. In patients who are inoperable or who have persistent/recurrent PH after PEA, medical therapy and/or balloon pulmonary angioplasty (BPA) are effective treatment options with favorable outcomes that are increasingly used. All treatment decisions should be made with a multidisciplinary team that includes a PEA surgeon, a BPA expert, and a chest radiologist.

BACKGROUND: Balloon pulmonary angioplasty (BPA) is an alternative therapy in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or residual/recurrent pulmonary hypertension (PH) after surgery. The aim of this study was to assess the short-term efficacy and safety of a BPA program.
METHODS: This prospective single-center study included all BPA sessions performed in CTEPH patients between 2017 and 2019. Clinical assessment including WHO functional class, plasma biomarkers, 6-min walk test (6MWT) and right heart catheterization was performed at baseline and six months after the last BPA session.
RESULTS: A total of 57 BPA sessions were performed in 11 CTEPH patients (64% with inoperable disease, 82% under pulmonar vasodilator therapy). Nine patients completed both the BPA program and a minimum six-month follow-up period. There were significant improvements in WHO functional class (p=0.004) and 6MWT (mean increase of 42 m; p=0.050) and a trend for significant hemodynamic improvement: 25% decrease in mean pulmonary artery pressure (mPAP) (p=0.082) and 42% decrease in pulmonary vascular resistance (PVR) (p=0.056). In the group of patients with severely impaired hemodynamics (three patients with mPAP >40 mmHg), the reduction was significant: 51% in mPAP (p=0.013) and 67% in PVR (p=0.050). Prostacyclin analogs and long-term oxygen therapy were withdrawn in all patients. Minor complications were recorded in 25% of patients. There were no major complications or deaths.
CONCLUSIONS: A BPA strategy on top of pulmonary vasodilator therapy further improves symptoms, exercise capacity and hemodynamics with an acceptable risk-benefit ratio in patients with inoperable CTEPH or residual/recurrent PH after surgery.

BACKGROUND: Balloon pulmonary angioplasty (BPA) is an alternative therapy in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or residual/recurrent pulmonary hypertension (PH) after surgery. The aim of this study was to assess the short-term efficacy and safety of a BPA program.
METHODS: This prospective single-center study included all BPA sessions performed in CTEPH patients between 2017 and 2019. Clinical assessment including WHO functional class, plasma biomarkers, 6-min walk test (6MWT) and right heart catheterization was performed at baseline and six months after the last BPA session.
RESULTS: A total of 57 BPA sessions were performed in 11 CTEPH patients (64% with inoperable disease, 82% under pulmonary vasodilator therapy). Nine patients completed both the BPA program and a minimum six-month follow-up period. There were significant improvements in WHO functional class (p=0.004) and 6MWT (mean increase of 42 m; p=0.050) and a trend for significant hemodynamic improvement: 25% decrease in mean pulmonary artery pressure (mPAP) (p=0.082) and 42% decrease in pulmonary vascular resistance (PVR) (p=0.056). In the group of patients with severely impaired hemodynamics (three patients with mPAP >40mmHg), the reduction was significant: 51% in mPAP (p=0.013) and 67% in PVR (p=0.050). Prostacyclin analogs and long-term oxygen therapy were withdrawn in all patients. Minor complications were recorded in 25% of patients. There were no major complications or deaths.
CONCLUSIONS: A BPA strategy on top of pulmonary vasodilator therapy further improves symptoms, exercise capacity and hemodynamics with an acceptable risk-benefit ratio in patients with inoperable CTEPH or residual/recurrent PH after surgery.