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Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease with poor prognosis. The progression mechanisms of CTEPH have not been well-understood. Balloon pulmonary angioplasty (BPA) has been spread for the treatment of inoperable CTEPH. However, the efficacy of BPA for pulmonary blood flow dynamics is still unclear. Methods: We analyzed blood flow dynamics of pulmonary arteries using computational fluid dynamics (CFD) with 4 normal patients and 4 CTEPH patients before and after BPA. Results: In the patients with CTEPH, flow velocity and wall shear stress (WSS) in pulmonary arteries were significantly decreased compared with normal subjects. The blood flow velocity and WSS were significantly increased after BPA treatment (Fig.1). Although stagnation area of blood flow was widely observed in pulmonary artery of CTEPH patients, this area was significantly reduced after BPA (Fig.2). BPA treatment significantly increased the WSS at the distal portion of pulmonary artery and the vessel diameter also increased significantly (Fig.3). Conclusion: In the analysis using CFD, it seems that stagnation of blood flow plays a crucial role in the progression of CTEPH. BPA improved the stagnation of blood flow and WSS. Recovery of pulmonary blood flow dynamics might decrease thrombogenicity and lead to vascular endothelial function recovery.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism and a major cause of chronic PH leading to right heart failure and death. Lung ventilation/perfusion scintigraphy is the screening test of choice; a normal scan rules out CTEPH. In the case of an abnormal perfusion scan, a high-quality pulmonary angiogram is necessary to confirm and define the pulmonary vascular involvement and prior to making a treatment decision. PH is confirmed with right heart catheterisation, which is also necessary for treatment determination. In addition to chronic anticoagulation therapy, each patient with CTEPH should receive treatment assessment starting with evaluation for pulmonary endarterectomy, which is the guideline recommended treatment. For technically inoperable cases, PH-targeted medical therapy is recommended (currently riociguat based on the CHEST studies), and balloon pulmonary angioplasty should be considered at a centre experienced with this challenging but potentially effective and complementary intervention., State of the art and research perspectives in chronic thromboembolic pulmonary hypertension, including treatment algorithm http://ow.ly/C3Iy30mfUly

Background: Riociguat is approved in Japan for treatment of pulmonary arterial hypertension and inoperable or persistent/recurrent CTEPH after pulmonary endarterectomy. Aim: Obtain post-marketing information on riociguat safety in treatment of CTEPH in routine clinical practice in Japan. Methods: Multicentre, prospective, non-interventional study in patients treated with riociguat for inoperable or persistent/recurrent CTEPH (NCT02117791). Primary outcomes: treatment-emergent adverse events (AEs) and adverse drug reactions over mandatory 12 months’ observation and up to 7 years thereafter. Results: Interim data are available for 684 patients (median age 69.0 years; 75.3% female; 89.6% inoperable; 9.8% persistent/recurrent). Median treatment duration with riociguat 358 (range 2–1128) days; median dose 5.8 mg/day. Prior use of an ERA in 29.8% of patients, PCA in 29.8%, PDE5i in 23.2% and balloon pulmonary angioplasty (BPA) in 27.2%. Concomitant ERA in 29.7%, PCA in 27.5%, PDE5i in 1.5%. AEs were reported in 188 patients (27.5%), drug-related AEs in 118 (17.3%; Table) and serious drug-related AEs in 15 (2.2%): 24 (3.5%) withdrew because of BPA and 44 (6.4%) because of AEs. Conclusions: The AE profile of riociguat in clinical practice in Japan appears consistent with clinical trials globally and in Japan. The most common AEs are consistent with the vasodilating action of riociguat.

Background: It is reported that there is no benefit of pulmonary hypertensive medical therapy (PHT) in patients undergoing pulmonary endarterectomy (PEA), except improved total pulmonary resistance with epoprostenol (Kurt W et al. Circulation 2009). They cautioned that the use of PHT delayed the time to referral for PEA. However, the effects of PHT on long-term prognosis after PEA remain to be examined. Methods: We retrospectively studied 149 patients (57±16 years) with chronic thromboembolic pulmonary hypertension undergoing PEA at Bologna University Hospital. The prevalence of PHT and its impact on haemodynamics and long-term outcomes were studied. Results: Sixty-one patients were untreated (non-tp), whereas 88 (59%) were treated with some PHT, consisting of monotherapy in 73; sildenafil in 47 (sil) and bosentan in 26 (bos), and combination therapy in 15 (combo). At baseline, there were no significant changes in age, sex, mean pulmonary arterial pressure (mPAP) or exercise capacity among 4 groups. During a mean follow-up of 60±44 months, 27 patients died. Five-year survival from PEA was 80, 87, 96 and 80% in non-tp, sil, bos and combo groups (P=0.36). Pre-PEA treatments with sil and combo significantly improved mPAP (50.0±8.9 to 46.0±9.7, 53.1±10.7 to 47.4±8.7 mmHg, P Conclusions: Pre-PEA treatments with sildenafil did not affect the long-term prognosis after PEA, but may be effective for stabilising haemodynamics before PEA without delaying the time to surgery.

Chronic thromboembolic pulmonary hypertension (CTEPH) is thought to result from incomplete resolution of pulmonary thromboemboli that undergo organisation into fibrous tissue within pulmonary arterial branches, filling pulmonary arterial lumina with collagenous obstructions. The treatment of choice is pulmonary endarterectomy (PEA) in CTEPH centres, which has low post-operative mortality and good long-term survival. For patients ineligible for PEA or who have recurrent or persistent pulmonary hypertension after surgery, medical treatment with riociguat is beneficial. In addition, percutaneous balloon pulmonary angioplasty (BPA) is an emerging option, and promises haemodynamic and functional benefits for inoperable patients. In contrast to conventional angioplasty, BPA with undersized balloons over guide wires exclusively breaks intraluminal webs and bands, without dissecting medial vessel layers, and repeat sessions are generally required. Observational studies report that BPA improves haemodynamics, symptoms and functional capacity in patients with CTEPH, but controlled trials with long-term follow-up are needed. Complications include haemoptysis, wire injury, vessel dissection, vessel rupture, reperfusion pulmonary oedema, pulmonary parenchymal bleeding and haemorrhagic pleural effusions. This review summarises the available evidence for BPA, patient selection, recent technical refinements and periprocedural imaging, and discusses the potential future role of BPA in the management of CTEPH.

Background: We have recently shown that balloon pulmonary angioplasty (BPA) improves peak oxygen uptake (peak VO2) early after the procedure, along with hemodynamics, in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH), without postprocedural deconditioning. However, the combined effects of BPA and the following cardiac rehabilitation (CR) on exercise capacity and quality of life (QOL) remain to be examined. Methods: We prospectively enrolled 35 consecutive patients (68±10 years) with inoperable CTEPH who underwent a series of BPA (3.7±2.0 procedures). Just after the final BPA (6.4±2.5 days), 13 out of 35 patients participated in 12-week CR program, consisting of walking, bicycle, and resistance training. Serial changes of cardiopulmonary exercise testing (CPX), hemodynamics, and QOL were assessed before the first BPA, after the final BPA (the beginning of CR), and the end of CR. Results: In 35 patients, BPA significantly improved hemodynamics (mean pulmonary arterial pressure, 35±11 to 24±6 mmHg), heart failure (HF) symptoms and signs, exercise intolerance (normalized peak VO2, 64±13 to 72±11%, P