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Multicenter Study
. 2024 Oct 22;150(17):1354-1365.
doi: 10.1161/CIRCULATIONAHA.124.068610. Epub 2024 Sep 17.

Worldwide CTEPH Registry: Long-Term Outcomes With Pulmonary Endarterectomy, Balloon Pulmonary Angioplasty, and Medical Therapy

Marion Delcroix  1   2 , Joanna Pepke-Zaba  3 , Andrea M D'Armini  4   5 , Elie Fadel  6 , Stefan Guth  7 , Stephen P Hoole  3 , David P Jenkins  3 , David G Kiely  8 , Nick H Kim  9 , Michael M Madani  10 , Hiromi Matsubara  11 , Kazuhiko Nakayama  12 , Aiko Ogawa  11 , Jaquelina S Ota-Arakaki  13 , Rozenn Quarck  1   2 , Roela Sadushi-Kolici  14 , Gérald Simonneau  15 , Christoph B Wiedenroth  7 , Bedrettin Yildizeli  16 , Eckhard Mayer  7 , Irene M Lang  14
Affiliations
Multicenter Study

Worldwide CTEPH Registry: Long-Term Outcomes With Pulmonary Endarterectomy, Balloon Pulmonary Angioplasty, and Medical Therapy

Marion Delcroix et al. Circulation. .

Abstract

Background: The European Chronic Thromboembolic Pulmonary Hypertension (CTEPH) registry, conducted between 2007 and 2012, reported the major impact of pulmonary endarterectomy (PEA) on the long-term survival of patients with CTEPH. Since then, 2 additional treatments for inoperable CTEPH have become available: balloon pulmonary angioplasty (BPA), and an approved oral drug therapy with the guanylate cyclase stimulator riociguat. The current registry aimed to evaluate the effect of these new therapeutic approaches in a worldwide context.

Methods: Participation in this international global registry included 34 centers in 20 countries. Between February 2015 and September 2016, 1009 newly diagnosed, consecutive patients were included and followed until September 2019.

Results: Overall, 605 patients (60%) underwent PEA and 185 (18%) underwent BPA; 76% of the 219 remaining patients not receiving mechanical intervention (ie, neither PEA nor BPA) were treated with pulmonary hypertension drugs. Of patients undergoing PEA and BPA, 38% and 78% also received drugs for pulmonary hypertension, respectively. Median age at diagnosis was higher in the BPA and No PEA/BPA groups than in the PEA group: 66 and 69, respectively, versus 60 years. Pulmonary vascular resistance (PVR) was similar in all groups, with an average of 643 dynes.s.cm-5. During the observation period (>3 years; ≤5.6 years), death was reported in 7%, 11%, and 27% of patients treated by PEA and BPA, and those receiving no mechanical intervention (P<0.001). In Kaplan-Meier analysis, 3-year survival was 94%, 92%, and 71% in the 3 groups, respectively. PEA 3-year survival improved by 5% from that observed between 2007 and 2012. There was no survival difference in patients receiving vitamin K antagonists and non-vitamin K oral anticoagulants (P=0.756). In Cox regression, reduced mortality was associated with: PEA and BPA in the global cohort; history of venous thromboembolism and lower PVR in the PEA group; lower right atrial pressure in the BPA group; and use of pulmonary hypertension drugs, oxygen therapy, and lower right atrial pressure, as well as functional class in the group receiving no mechanical intervention.

Conclusions: This second international CTEPH registry reveals important improvement in patient survival since the introduction of BPA and an approved drug for pulmonary hypertension. The type of anticoagulation regimen did not influence survival.

Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT02656238.

Keywords: angioplasty, balloon; endarterectomy; hypertension, pulmonary; registries; venous thromboembolism.

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Conflict of interest statement

M.D. reports research grants from Janssen, speaker and consultant fees from Altavant, Acceleron, AOP, Bayer, Ferrer, Gossamer, INARI, Janssen, United Therapeutics, and MSD outside the submitted work, and all paid to her institution. She was holder of a Janssen chair for pulmonary hypertension at the KU Leuven. J.P.Z. reports research grants from MSD and consultant fees from Ferrer, Gossamer, Janssen outside submitted work. S.G. reports speaker fees or consultant honoraria from Actelion/Janssen, Bayer AG, MSD, and Pfizer. D.P.J. reports speaker fees and consultancy fees from Janssen outside this submitted work. D.G.K. reports speaker fees, consultancy fees, or funding to attend meetings from Acceleron, Altavant, Ferrer, Janssen, Gossamer, MSD and United Therapeutics and research support from Ferrer and Janssen, all outside of the submitted work. N.H.K. reports receiving speaker fees from Bayer and Janssen; consultancy fees from Bayer, Janssen, Merck, Polarean, Pulnovo, and United Therapeutics; and research support from Altavant and Gossamer Bio. All disclosed fees and support are outside the submitted work. M.M.M. reports consultancy fees and royalties from Wexler Surgical and consultancy fees from Actelion/Janssen and Johnson & Johnson. H.M. reports research grants from Nippon Shinyaku; speaker and consultant fees from Bayer, Janssen, and MSD; speaker fees from Kaneka Medix, Mochida, Nippon Shinyaku, and Nipro, all outside of the submitted work. J.S.O.A. reports personal fees from Bayer and MSD. R.S.K. reports having received speaker fees from AOP Health, Actelion/Janssen, and MSD, all outside of the submitted work. GS reports receiving advisory board and speaker fees from Acceleron, Bayer, Janssen, MSD, and Merck. C.B.W. reports speaker fees or consultant honoraria from Actelion/Janssen, AOP Orphan Pharmaceuticals AG, Bayer AG, BTG, MSD, OrphaCare, and Pfizer. E.M. reports receiving consultancy or speaker fees from Actelion/Janssen, Bayer, and MSD. I.M.L. has relationships with drug companies including AOP Health, Actelion/Janssen, MSD, United Therapeutics, Pulnovo, Medtronic, Neutrolis, and Sanofi; in addition to being investigator in trials involving these companies, relationships include consultancy services, research grants, and membership of scientific advisory boards. The other authors report no conflicts.

Figures

Figure 1.
Figure 1.
Kaplan–Meier survival curve by intervention. Pulmonary endarterectomy (PEA) vs balloon pulmonary angioplasty (BPA), P=0.074; PEA and BPA vs no PEA/BPA, P<0.05.
Figure 2.
Figure 2.
Kaplan–Meier survival curve for patients not undergoing PEA, by reason for no PEA. Comorbidity and considered operable patients who were not operated on had worse survival rates than technically inoperable patients (P<0.001 and P=0.003, respectively). Comorbidity patients also had worse survival rates than patient refusal patients (P=0.014). PEA indicates pulmonary endarterectomy.
Figure 3.
Figure 3.
Predictors of all-cause death for the whole cohort. Multivariable regression analysis with adjusted hazard ratios. For a full list of candidate variables, please refer to the statistical section; any variables not included in the figure did not emerge as associated. BPA indicates balloon pulmonary angioplasty; NYHA-FC, New York Heart Association functional class; PEA, pulmonary endarterectomy; PVR, pulmonary vascular resistance; RAP, right atrial pressure; ref, reference for comparison; trt, treatment; and VTE, venous thromboembolism.
Figure 4.
Figure 4.
Predictors of all-cause death. A, Patients receiving pulmonary endarterectomy (PEA). B, Patients receiving balloon pulmonary angioplasty (BPA). C, Patients receiving neither PEA nor BPA. Multivariable regression analysis with adjusted hazard ratios. ECMO indicates extracorporeal membrane oxygenation; ICU, intensive care unit; NYHA-FC, New York Heart Association functional class; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; RAP, right atrial pressure; RHF, right heart failure; and VTE, venous thromboembolism.
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