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Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism. It is caused by persistent obstruction of pulmonary arteries by chronic organised fibrotic clots, despite adequate anticoagulation. The pulmonary hypertension is also caused by concomitant microvasculopathy which may progress without timely treatment. Timely and accurate diagnosis requires the combination of imaging and haemodynamic assessment. Optimal therapy should be individualised to each case and determined by an experienced multidisciplinary CTEPH team with the ability to offer all current treatment modalities. This report summarises current knowledge and presents key messages from the International CTEPH Conference, Bad Nauheim, Germany, 2021. Sessions were dedicated to 1) disease definition; 2) pathophysiology, including the impact of the hypertrophied bronchial circulation, right ventricle (dys)function, genetics and inflammation; 3) diagnosis, early after acute pulmonary embolism, using computed tomography and perfusion techniques, and supporting the selection of appropriate therapies; 4) surgical treatment, pulmonary endarterectomy for proximal and distal disease, and peri-operative management; 5) percutaneous approach or balloon pulmonary angioplasty, techniques and complications; and 6) medical treatment, including anticoagulation and pulmonary hypertension drugs, and in combination with interventional treatments. Chronic thromboembolic pulmonary disease without pulmonary hypertension is also discussed in terms of its diagnostic and therapeutic aspects., Competing Interests: Conflict of interest: E. Fadel, G. Martinez, G. Simonneau, K. Salaunkey, N. Screaton and P. Brénot have no conflicts of interest to declare. A. Vonk Noordegraaf is supported by the Netherlands CardioVascular Research Initiative (CVON-2012–08 PHAEDRA, CVON-2017-10 DOLPHIN-GENESIS) and the Netherlands Organization for Scientific Research (NWO-VICI: 918.16.610); his institute received speakers fees from Johnson & Johnson, MSD, Actelion, Bayer and Ferrer in the past 3 years; and he served as a member of the scientific advisory boards of Morphogen-X, Ferrer, Gossamer Bio Services Inc. and Johnson & Johnson. C.B. Wiedenroth has received speaker fees and/or consultant honoraria from Actelion, AOP Orphan Pharmaceuticals AG, Bayer AG, BTG, MSD and Pfizer. D.P. Jenkins is on the adjudication committees for the SELECT and Maciteph studies (Actelion), and has received fees for lectures on CTEPH from Actelion. E. Mayer has received speaker or consulting fees from Actelion/Janssen, Bayer and MSD. F.A. Klok has received research support from Bayer, Bristol-Myers Squibb, Boehringer Ingelheim, MSD, Leo Pharma, Actelion, the Netherlands Organisation for Health Research and Development, the Dutch Thrombosis Association, the Dutch Heart Foundation and the Horizon Europe Program, unrelated to the current work and paid to his institution. H.A. Ghofrani has received grants/research support from Actelion, Janssen, Bayer, Gossamer, Acceleron; honoraria or consultation fees from Actelion, Janssen, Bayer, Gossamer, Acceleron, Pfizer, Novartis and MSD; and participated in a company sponsored speaker's bureau for Actelion, Janssen, Bayer, Gossamer, Pfizer, Novartis and MSD. H. Matsubara has received honoraria for lectures from Janssen Pharmaceutical KK, Bayer Yakuhin Ltd, Nippon Shinyaku Co Ltd, Mochida Pharmaceutical Co. Ltd and Kaneka Medix Corporation. I.M. Lang has a financial interest with Actelion/Janssen, AOP Orphan, Medtronic, Daiichi Sankyo, Neutrolis, United Therapeutics and Ferrer that could be perceived as a real or apparent conflict of interest in the context of the subject of this article. J. Pepke-Zaba has received speaker or consulting fees from Actelion/Janssen, Bayer, MSD and Ferrer, and a research grant from MSD, all outside submitted work. M.C. McInnis reports honoraria from Bayer and Boehringer Ingelheim, unrelated to the current work. M. Delcroix reports research grants from Actelion/Janssen, speaker and consultant fees from Altavant, Acceleron, AOP, Daiichi Sankyo, Bayer, Ferrer, Gossamer and MSD, outside the submitted work, and all paid to her institution. She is holder of the Janssen Chair for Pulmonary Hypertension at KU Leuven. M.M. Hoeper reports fees for lectures and/or consultations from Acceleron, Actelion, Bayer, GSK, Janssen, MSD and Pfizer. M.M. Madani is a consultant for Actelion. M. Toshner reports grants and personal fees from Bayer, personal fees from MSD, grants and personal fees from Actelion, and personal fees from GSK within the last 5 years. N.H. Kim has served as consultant for Bayer, Janssen, Merck and United Therapeutics. S. Guth has received speaker fees and/or consultant honoraria from Actelion, Bayer, GSK, MSD and Pfizer. X. Jaïs reports grants from the French Ministry of Health and Bayer Healthcare, during the conduct of the study; grants from Acceleron, Janssen and MSD; personal fees from Janssen and MSD; and non-financial support from Janssen., (Copyright ©The authors 2023.)

Chronic thromboembolic pulmonary hypertension (CTEPH) is an under recognized complication of pulmonary embolism with significant morbidity and mortality when left untreated. There have been ongoing advances in the treatment of CTEPH with potentially excellent outcomes for patients when diagnosed and treated in a timely fashion. Although ventilation-perfusion scintigraphy remains critical in screening for CTEPH, the radiologic diagnosis involves multiple modalities from digital subtraction angiography to high quality computed tomography pulmonary angiography. State of the art imaging in CTEPH now also includes dual energy computed tomography, lung subtraction iodine mapping, and magnetic resonance imaging., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: Right-to-left ventricle diameter ratio (dRV/dLV) on CT pulmonary angiography (CTPA) is a predictor of outcomes in non-operated chronic thromboembolic pulmonary hypertension (CTEPH) patients. The purpose of this study is to evaluate the performance of a novel machine learning (ML) algorithm for dRV/dLV measurement in operated CTEPH patients and its association with post-operative outcomes., Methods: This retrospective study reviewed consecutive CTEPH patients who underwent pulmonary endarterectomy between 2013 and 2017. ML calculated dRV/dLV on pre-operative CTPA and compared with manual measures. Associations of dRV/dLV with patient characteristics and post-operative outcomes were evaluated including intensive care (ICU) and hospital length of stay (LOS) using multivariable linear regression analysis. Prolonged LOS was defined as greater than median., Results: ML segmented the ventricles in 99/125 (79%) patients. The most common cause of failure was misidentification of the moderator band as the interventricular septum (7.9%). Mean dRV/dLV by ML was 1.4 ± 0.4 and strongly correlated with manual measures ( r = 0.9-0.96 p < 0.0001). dRV/dLV was moderately correlated with measures of pulmonary hypertension on right heart catheterization and RV dilatation on echocardiogram ( r = 0.5-0.6, p < 0.0001). dRV/dLV ≥ 1.2 was associated with proximal Jamieson type disease ( p = 0.032), longer cardiopulmonary bypass ( p = 0.037), aortic cross-clamp ( p = 0.022) and circulatory arrest ( p < 0.001) at surgery and dRV/dLV ≥ 1.6 with post-operative ECMO ( p = 0.006). dRV/dLV was independently associated with prolonged ICU LOS (OR = 3.79, 95% CI 1.1-13.06, p = 0.035)., Conclusion: dRV/dLV was associated with CTEPH severity and independently associated with prolonged ICU LOS. This CT parameter may therefore assist in perioperative planning. Further refinement of the ML algorithm or CTPA technique is required to avoid errors in ventricular segmentation., Advances in Knowledge: Automated right-to-left ventricle ratio measurement by machine learning is feasible and is independently associated with outcome after pulmonary endarterectomy.

Objective: Determine the long-term outcome and need for additional therapy after pulmonary endarterectomy (PEA) for segmental chronic thromboembolic pulmonary hypertension., Methods: Retrospective analysis of a prospective cohort of 401 consecutive Canadian patients undergoing PEA between August 2005 and March 2020 in Toronto. The outcome of segmental disease defined as Jamieson type 3 was compared with more proximal disease defined as Jamieson type 1 and 2. The cohort was divided into 3 intervals to analyze the trend over time: 2005-2010, 2011-2015, and 2016-2020., Results: Type 3 disease accounted for 41% of patients undergoing PEA durig 2016-2020 compared with 7% in 2006-2010. Total pulmonary vascular resistance improved by 505 ± 485 dynes/s/cm -5 in type 3 disease and by 593 ± 452 dynes/s/cm -5 in type 1 or 2 disease (P = .07). Mortality after PEA was similar between type 3 and type 1 and 2 disease at 30-days (2.8% vs 2.3%; P = .8) and at 1 year (7.7% vs 5.5%; P = .4). At 5 years, the survival was lower in type 3 disease (80% vs 91% in type 1 or 2 disease; P = .002). Type 3 disease was an independent predictor for the initiation of pulmonary hypertension-targeted medical therapy after PEA with a cumulative incidence of 38% at 10 years compared with 20% in type 1 and 2 disease (P < .0001). Post-PEA balloon pulmonary angioplasty was predominantly performed in type 3 disease (8% vs 1% in more type 1 or 2 disease; P = .0002)., Conclusions: PEA achieved excellent early and long-term results in segmental chronic thromboembolic pulmonary hypertension. However, patients with segmental disease are at increased risk of requiring additional therapy after PEA and should be carefully monitored., (Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Pulmonary endarterectomy (PEA) is a curative procedure for patients with chronic thromboembolic pulmonary hypertension. Body composition and exercise capacity have been associated with adverse outcomes in patients undergoing cardiothoracic operations, but their significance with PEA is unclear. We evaluated the association of body composition and 6-minute walk distance (6MWD) with disease severity, hospital length of stay, discharge disposition, and postoperative functional recovery., Methods: This was a retrospective, single-center cohort study of patients who underwent PEA (January 2014-December 2017). Body composition (skeletal muscle mass and adiposity cross-sectional area) was quantified using thoracic computed tomography with sliceOmatic (TomoVision, Magog, QC, Canada) software. Body mass index was calculated. Association of body composition measures and 6MWD with clinical outcomes was evaluated using multivariable regression models., Results: The study included 127 patients (42% men), aged 58 ± 14 years; body mass index was 31 ± 7 kg/m 2 and 6MWD was 361 ± 165 m). Muscle and 6MWD were associated with disease severity measures. Of those surviving hospitalization (n = 125), a greater 6MWD was associated with a shorter hospital stay (1.9 median days per 100 m; p < .001) and higher likelihood of being discharged directly home from hospital (odds ratio, 2.1 per 100 m; P = .004), independent of age, sex, and body mass index. Those with a lower preoperative 6MWD (per 100 m) had a greater increase in their postoperative 6MWD (52 m; P < .0001), independent of age, sex, and body mass index. Body composition measures were not associated with hospital outcomes or exercise capacity in the first year postoperatively., Conclusions: Exercise capacity was a more prognostic marker of PEA outcomes compared with body composition. Future research is needed to explore pre-PEA rehabilitation strategies., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

ISHLT members have recognized the importance of a consensus statement on the evaluation and management of patients with chronic thromboembolic pulmonary hypertension. The creation of this document required multiple steps, including the engagement of the ISHLT councils, approval by the Standards and Guidelines Committee, identification and selection of experts in the field, and the development of 6 working groups. Each working group provided a separate section based on an extensive literature search. These sections were then coalesced into a single document that was circulated to all members of the working groups. Key points were summarized at the end of each section. Due to the limited number of comparative trials in this field, the document was written as a literature review with expert opinion rather than based on level of evidence., (Copyright © 2021 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)