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2. Highlights from the International Chronic Thromboembolic Pulmonary Hypertension Congress 2021

Author

Simonneau, Gérald, Fadel, Elie, Noordegraaf, Anton Vonk, Toshner, Mark, Lang, Irene M, Klok, Frederikus A, McInnis, Micheal C, Screaton, Nicholas, Madani, Michael M, Martinez, Guillermo, Salaunkey, Kiran, Jenkins, David P, Matsubara, Hiromi, Brénot, Philippe, Hoeper, Marius M, Ghofrani, Hossein A, Jaïs, Xavier, Wiedenroth, Christoph B, Guth, Stefan, Kim, Nick H, Pepke-Zaba, Joanna, Delcroix, Marion, and Mayer, Eckhard

Subjects
Lung, Aetiology, 2.1 Biological and endogenous factors, Cardiovascular, Humans, Hypertension, Pulmonary, Chronic Disease, Pulmonary Embolism, Pulmonary Artery, Angioplasty, Balloon, Endarterectomy, Anticoagulants, Medical Physiology, Respiratory System
Abstract

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.

Published
2023

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

Author

Delcroix, Marion, Pepke-Zaba, Joanna, D’Armini, Andrea M., Fadel, Elie, Guth, Stefan, Hoole, Stephen P., Jenkins, David P., Kiely, David G., Kim, Nick H., Madani, Michael M., Matsubara, Hiromi, Nakayama, Kazuhiko, Ogawa, Aiko, Ota-Arakaki, Jaquelina S., Quarck, Rozenn, Sadushi-Kolici, Roela, Simonneau, Gérald, Wiedenroth, Christoph B., Yildizeli, Bedrettin, Mayer, Eckhard, and Lang, Irene M.

Published
2024
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6. Current strategies for managing chronic thromboembolic pulmonary hypertension: results of the worldwide prospective CTEPH Registry

Author

Guth, Stefan, D'Armini, Andrea M, Delcroix, Marion, Nakayama, Kazuhiko, Fadel, Elie, Hoole, Stephen P, Jenkins, David P, Kiely, David G, Kim, Nick H, Lang, Irene M, Madani, Michael M, Matsubara, Hiromi, Ogawa, Aiko, Ota-Arakaki, Jaquelina S, Quarck, Rozenn, Sadushi-Kolici, Roela, Simonneau, Gérald, Wiedenroth, Christoph B, Yildizeli, Bedrettin, Mayer, Eckhard, and Pepke-Zaba, Joanna

Subjects
Rare Diseases, Lung, Cardiovascular
Abstract

Pulmonary endarterectomy (PEA), pulmonary arterial hypertension (PAH) therapy and balloon pulmonary angioplasty (BPA) are currently accepted therapies for chronic thromboembolic pulmonary hypertension (CTEPH). This international CTEPH Registry identifies clinical characteristics of patients, diagnostic algorithms and treatment decisions in a global context. 1010 newly diagnosed consecutive patients were included in the registry between February 2015 and September 2016. Diagnosis was confirmed by right heart catheterisation, ventilation-perfusion lung scan, computerised pulmonary angiography and/or invasive pulmonary angiography after at least 3 months on anticoagulation. Overall, 649 patients (64.3%) were considered for PEA, 193 (19.1%) for BPA, 20 (2.0%) for both PEA and BPA, and 148 (14.7%) for PAH therapy only. Reasons for PEA inoperability were technical inaccessibility (n=235), comorbidities (n=63) and patient refusal (n=44). In Europe and America and other countries (AAO), 72% of patients were deemed suitable for PEA, whereas in Japan, 70% of patients were offered BPA as first choice. Sex was evenly balanced, except in Japan where 75% of patients were female. A history of acute pulmonary embolism was reported for 65.6% of patients. At least one PAH therapy was initiated in 35.8% of patients (26.2% of PEA candidates, 54.5% of BPA candidates and 54.1% of those not eligible for either PEA or BPA). At the time of analysis, 39 patients (3.9%) had died of pulmonary hypertension-related causes (3.5% after PEA and 1.8% after BPA). The registry revealed noticeable differences in patient characteristics (rates of pulmonary embolism and sex) and therapeutic approaches in Japan compared with Europe and AAO.

Published
2021

8. Shared and Distinct Genomics of Chronic Thromboembolic Pulmonary Hypertension and Pulmonary Embolism

Author

Liley, James, primary, Newnham, Michael, additional, Bleda, Marta, additional, Bunclark, Katherine, additional, Auger, William, additional, Barbera, Joan Albert, additional, Bogaard, Harm, additional, Delcroix, Marion, additional, Fernandes, Timothy M., additional, Howard, Luke, additional, Jenkins, David, additional, Lang, Irene, additional, Mayer, Eckhard, additional, Rhodes, Chris, additional, Simpson, Michael, additional, Southgate, Laura, additional, Trembath, Richard, additional, Wharton, John, additional, Wilkins, Martin R, additional, Gräf, Stefan, additional, Morrell, Nicholas, additional, Pepke Zaba, Joanna, additional, and Toshner, Mark, additional

Published
2024
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9. Pulmonary endarterectomy in the management of chronic thromboembolic pulmonary hypertension

Author

Jenkins, David, Madani, Michael, Fadel, Elie, D'Armini, Andrea Maria, and Mayer, Eckhard

Subjects
Biomedical and Clinical Sciences, Cardiovascular Medicine and Haematology, Clinical Sciences, Lung, Cardiovascular, Chronic Disease, Endarterectomy, Hemodynamics, Hospital Mortality, Humans, Hypertension, Pulmonary, Patient Selection, Postoperative Complications, Pulmonary Artery, Pulmonary Embolism, Risk Assessment, Risk Factors, Time Factors, Treatment Outcome, Medical Physiology, Respiratory System, Cardiovascular medicine and haematology
Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension, resulting from fibrotic transformation of pulmonary artery clots causing chronic obstruction in macroscopic pulmonary arteries and associated vascular remodelling in the microvasculature.Pulmonary endarterectomy (PEA) offers the best chance of symptomatic and prognostic improvement in eligible patients; in expert centres, it has excellent results. Current in-hospital mortality rates are 90% at 1 year and >70% at 10 years. However, PEA, is a complex procedure and relies on a multidisciplinary CTEPH team led by an experienced surgeon to decide on an individual's operability, which is determined primarily by lesion location and the haemodynamic parameters. Therefore, treatment of patients with CTEPH depends largely on subjective judgements of eligibility for surgery by the CTEPH team.Other controversies discussed in this article include eligibility for PEA versus balloon pulmonary angioplasty, the new treatment algorithm in the European Society of Cardiology/European Respiratory Society guidelines and the definition of an "expert centre" for the management of this condition.

Published
2017

12. Pulmonary Hypertension

Author

Wiedenroth, Christoph B., Mayer, Eckhard, Willerson, James T., Series Editor, Feldman, David S., editor, and Mohacsi, Paul, editor

Published
2019
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15. Long-term effect of thymectomy plus prednisone versus prednisone alone in patients with non-thymomatous myasthenia gravis: 2-year extension of the MGTX randomised trial

Author

Cutter, Gary, Aban, Inmaculada, Minisman, Greg, Feese, Michelle, Kuo, Hui-Chien, Newsom-Davis, John, Wolfe, Gil, Kaminski, Henry, Jaretzki, Alfred, Sonett, Joshua, Mazia, Claudio, Saluto, Valeria, Rosenberg, Moises, Alvarez, Valeria, Rey, Lisa, King, John, Butzkueven, Helmut, Goldblatt, John, Carey, John, Pollard, John, Reddel, Stephen, Handel, Nicholas, McCaughan, Brian, Pallot, Linda, Waddington-Cruz, Márcia, Novis, Ricardo, Boasquevisque, Carlos, Dias-Tosta, Elza, Morato-Fernandez, Rubens, Ximenes, Manoel, Werneck, Lineu, Scola, Rosana, Soltoski, Paulo, Chalk, Colin, Moore, Fraser, Mulder, David, Wadup, Lisa, Oger, Joel, Mezei, Michele, Evans, Kenneth, Jiwa, Theresa, Schaffar, Anne, White, Chris, Toth, Cory, Gelfand, Gary, Wood, Susan, Pringle, Elizabeth, Zwicker, Jocelyn, Maziak, Donna, Shamji, Farid, Sundaresan, Sudhir, Seely, Andrew, Cea, Gabriel, Verduga, Renato, Aguayo, Alberto, Jander, Sebastian, Zickler, Philipp, Klein, Michael, Marx, Alexander, Ströbel, Philipp, Weis, Cleo-Aron, Melms, Arthur, Bischof, Felix, Aebert, Hermann, Ziemer, Gerhard, Nix, Wilfred, Thümler, Björn, Wilhem-Schwenkmezger, Thomas, Mayer, Eckhard, Schalke, Berthold, Pöschel, Peter, Hieber, Gisela, Wiebe, Karsten, Antonini, Giovanni, Clemenzi, Alessandro, Ceschin, Vanessa, Rendina, Erino, Venuta, Federico, Morino, Stefania, Bucci, Elisabetta, Durelli, Luca, Tavella, Alessia, Clerico, Marinella, Contessa, Giulia, Borasio, Piero, Evoli, Amelia, Servidei, Serenella, Granone, Pierluigi, Mantegazza, Renato, Berta, Emilia, Novellino, Lorenzo, Spinelli, Luisa, Motomura, Masakatsu, Matsuo, Hidenori, Nagayasu, Takeshi, Yoshikawa, Hiroaki, Takamori, Masaharu, Oda, Makoto, Matsumoto, Isao, Furukawa, Yutaka, Noto, Daisuke, Motozaki, Yuko, Iwasa, Kazuo, Yanase, Daisuke, Garcia Ramos, Guillermo, Cacho, Bernardo, de la Garza, Lorenzo, Kostera-Pruszczyk, Anne, Lipowska, Marta, Kwiecinski, Hubert, Potulska-Chromik, Anna, Orlowski, Tadeusz, Silva, Ana, Feijo, Marta, Freitas, António, Heckmann, Jeannine, Frost, Andrew, Pan, Edward, Tucker, Lawrence, Rossouw, Johan, Drummond, Fiona, Illa, Isabel, Diaz, Jorge, Leon, Carlos, Yeh, Jiann-Horng, Chiu, Hou-Chang, Hsieh, Yei-San, Witoonpanich, Rawiphan, Tunlayadechanont, Supoch, Attanavanich, Sukasom, Verschuuren, Jan, Straathof, Chiara, Titulaer, Maarten, Versteegh, Michel, Pels, Arda, Krum, Yvonne, Buckley, Camilla, Leite, M. Isabel, Vincent, Angela, Hilton-Jones, David, Ratnatunga, Chandi, Farrugia, Maria, Petty, Richard, Overell, James, Kirk, Alan, Gibson, Andrew, McDermott, Chris, Hopkinson, David, Lecky, Bryan, Watling, David, Marshall, Dot, Saminaden, Sam, Davies, Deborah, Dougan, Charlotte, Sathasivam, Siva, Page, Richard, Sussman, Jon, Ealing, John, Krysiak, Peter, Amato, Anthony, Salajegheh, Mohammad, Jaklitsch, Michael, Roe, Kristen, Ashizawa, Tetsuo, Smith, Robert Glenn, Zwischenberg, Joseph, Stanton, Penny, Barboi, Alexandru, Jaradeh, Safwan, Tisol, William, Gasparri, Mario, Haasler, George, Yellick, Mary, Dennis, Cedric, Barohn, Richard, Pasnoor, Mamatha, Dimachkie, Mazen, McVey, April, Gronseth, Gary, Dick, Arthur, Kramer, Jeffrey, Currence, Melissa, Herbelin, Laura, Belsh, Jerry, Li, George, Langenfeld, John, Mertz, Mary Ann, Benatar, Michael, Harrison, Taylor, Force, Seth, Usher, Sharon, Beydoun, Said, Lin, Frank, DeMeester, Steve, Akhter, Salem, Malekniazi, Ali, Avenido, Gina, Crum, Brian, Milone, Margherita, Cassivi, Stephen, Fisher, Janet, Ciafaloni, Emma, Heatwole, Chad, Watson, Thomas, Hilbert, James, Smirnow, Alexis, Distad, B. Jane, Weiss, Michael, Wood, Douglas, Haug, Joanna, Ernstoff, Raina, Cao, Jingyang, Chmielewski, Gary, Welsh, Robert, Duris, Robin, Gutmann, Laurie, Pawar, Gauri, Graeber, Geoffrey Marc, Altemus, Patricia, Nance, Christopher, Gutmann, Ludwig, Jackson, Carlayne, Grogan, Patrick, Calhoon, John, Kittrell, Pamela, Myers, Deborah, Hayat, Ghazala, Naunheim, Keith, Eller, Susan, Holzemer, Eve, Katirji, Bashar, Alshekhlee, Amer, Robke, Jason, Karlinchak, Brenda, Katz, Jonathan, Miller, Robert, Roan, Ralph, Forshew, Dallas, Kissel, John, Elsheikh, Bakri, Ross, Patrick, Chelnick, Sharon, Lewis, Richard, Acsadi, Agnes, Baciewicz, Frank, Masse, Stacey, Massey, Janice, Juel, Vern, Onaitis, Mark, Lowe, James, Lipscomb, Bernadette, Mozaffar, Tahseen, Thai, Gaby, Milliken, Jeffrey, Martin, Veronica, Karayan, Ronnie, Muley, Suraj, Parry, Gareth, Shumway, Sara, Oh, Shin, Claussen, Gwen, Lu, Liang, Cerfolio, Robert, Young, Angela, Morgan, Marla, Pascuzzi, Robert, Kincaid, John, Kesler, Kenneth, Guingrich, Sandy, Michaels, Angi, Phillips, Lawrence, Burns, Ted, Jones, David, Fischer, Cindy, Pulley, Michael, Berger, Alan, D'Agostino, Harry, Smith, Lisa, Rivner, Michael, Pruitt, Jerry, Landolfo, Kevin, Hillman, Demetric, Shaibani, Aziz, Sermas, Angelo, Ruel, Ross, Ismail, Farah, Sivak, Mark, Goldstein, Martin, Camunas, Jorge, Bratton, Joan, Tandan, Rup, Panitch, Hill, Leavitt, Bruce, Jones, Marilee, Muppidi, Srikanth, Vernino, Steven, Nations, Sharon, Meyer, Dan, Gorham, Nina, Wolfe, Gil I, Kaminski, Henry J, Aban, Inmaculada B, Cea, J Gabriel, Heckmann, Jeannine M, King, John O, Beydoun, Said R, Chalk, Colin H, Barboi, Alexandru C, Amato, Anthony A, Shaibani, Aziz I, Lecky, Bryan R F, Pulley, Michael T, Rivner, Michael H, Kostera-Pruszczyk, Anna, Pascuzzi, Robert M, Jackson, Carlayne E, Verschuuren, Jan J G M, Massey, Janice M, Kissel, John T, Werneck, Lineu C, Barohn, Richard J, Silvestri, Nicholas J, Conwit, Robin, Sonett, Joshua R, Jaretzki, Alfred, III, and Cutter, Gary R

Published
2019
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16. Chronic thromboembolic pulmonary hypertension.

Author

Kim, Nick H, Delcroix, Marion, Jenkins, David P, Channick, Richard, Dartevelle, Philippe, Jansa, Pavel, Lang, Irene, Madani, Michael M, Ogino, Hitoshi, Pengo, Vittorio, and Mayer, Eckhard

Subjects
Lung, Cardiovascular, Heart Disease, Clinical Research, Respiratory, Angiography, Angioplasty, Balloon, Anticoagulants, Cardiac Catheterization, Chronic Disease, Endarterectomy, Heart Failure, Humans, Hypertension, Pulmonary, Pulmonary Embolism, Pyrazoles, Pyrimidines, Tomography, X-Ray Computed, chronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, ventilation/perfusion scan, pulmonary angiogram, Cardiovascular System & Hematology
Abstract

Since the last World Symposium on Pulmonary Hypertension in 2008, we have witnessed numerous and exciting developments in chronic thromboembolic pulmonary hypertension (CTEPH). Emerging clinical data and advances in technology have led to reinforcing and updated guidance on diagnostic approaches to pulmonary hypertension, guidelines that we hope will lead to better recognition and more timely diagnosis of CTEPH. We have new data on treatment practices across international boundaries as well as long-term outcomes for CTEPH patients treated with or without pulmonary endarterectomy. Furthermore, we have expanded data on alternative treatment options for select CTEPH patients, including data from multiple clinical trials of medical therapy, including 1 recent pivotal trial, and compelling case series of percutaneous pulmonary angioplasty. Lastly, we have garnered more experience, and on a larger international scale, with pulmonary endarterectomy, which is the treatment of choice for operable CTEPH. This report overviews and highlights these important interval developments as deliberated among our task force of CTEPH experts and presented at the 2013 World Symposium on Pulmonary Hypertension in Nice, France. (J Am Coil Cardiol 2013;62:D92-9) ©2013 by the American College of Cardiology Foundation.

Published
2014

17. Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension (MERIT-1): results from the multicentre, phase 2, randomised, double-blind, placebo-controlled study.

Author

Ghofrani, Hossein-Ardeschir, Simonneau, Gérald, D'Armini, Andrea M, Fedullo, Peter, Howard, Luke S, Jaïs, Xavier, Jenkins, David P, Jing, Zhi-Cheng, Madani, Michael M, Martin, Nicolas, Mayer, Eckhard, Papadakis, Kelly, Richard, Dominik, and Kim, Nick H

Subjects
PULMONARY hypertension, PHOSPHODIESTERASE inhibitors, PULMONARY arterial hypertension, THROMBOEMBOLISM, HOSPITAL patients
Abstract

Macitentan is beneficial for long-term treatment of pulmonary arterial hypertension. The microvasculopathy of chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension are similar. The phase 2, double-blind, randomised, placebo-controlled MERIT-1 trial assessed macitentan in 80 patients with CTEPH adjudicated as inoperable. Patients identified as WHO functional class II–IV with a pulmonary vascular resistance (PVR) of at least 400 dyn·s/cm5 and a walk distance of 150–450 m in 6 min were randomly assigned (1:1), via an interactive voice/web response system, to receive oral macitentan (10 mg once a day) or placebo. Treatment with phosphodiesterase type-5 inhibitors and oral or inhaled prostanoids was permitted for WHO functional class III/IV patients. The primary endpoint was resting PVR at week 16, expressed as percentage of PVR measured at baseline. Analyses were done in all patients who were randomly assigned to treatment; safety analyses were done in all patients who received at least one dose of the study drug. This study is registered with ClinicalTrials.gov , number NCT02021292. Between April 3, 2014, and March 17, 2016, we screened 186 patients for eligibility at 48 hospitals across 20 countries. Of these, 80 patients in 36 hospitals were randomly assigned to treatment (40 patients to macitentan, 40 patients to placebo). At week 16, geometric mean PVR decreased to 71·5% of baseline in the macitentan group and to 87·6% in the placebo group (geometric means ratio 0·81, 95% CI 0·70–0·95, p=0·0098). The most common adverse events in the macitentan group were peripheral oedema (9 [23%] of 40 patients) and decreased haemoglobin (6 [15%]). In MERIT-1, macitentan significantly improved PVR in patients with inoperable CTEPH and was well tolerated. Actelion Pharmaceuticals Ltd. [ABSTRACT FROM AUTHOR]

Published
2024
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18. Chronisch thromboembolische pulmonale Hypertonie (Gruppe 4)

Author

Guth, Stefan, additional, Wilkens, Heinrike, additional, Halank, Michael, additional, Held, Matthias, additional, Hobohm, Lukas, additional, Konstantinides, Stavros, additional, Omlor, Albert, additional, Seyfarth, Hans-Jürgen, additional, Schäfers, Hans-Joachim, additional, Mayer, Eckhard, additional, and Wiedenroth, Christoph B., additional

Published
2023
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19. Endothelial Overexpression of TGF-β-Induced Protein Impairs Venous Thrombus Resolution

Author

Bochenek, Magdalena L., primary, Saar, Kathrin, additional, Nazari-Jahantigh, Maliheh, additional, Gogiraju, Rajinikanth, additional, Wiedenroth, Christoph B., additional, Münzel, Thomas, additional, Mayer, Eckhard, additional, Fink, Ludger, additional, Schober, Andreas, additional, Hübner, Norbert, additional, Guth, Stefan, additional, Konstantinides, Stavros, additional, and Schäfer, Katrin, additional

Published
2023
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22. Patient-Reported Long-Term Outcome of Balloon Pulmonary Angioplasty for Inoperable CTEPH

Author

Wiedenroth, Christoph B., additional, Steinhaus, Kristin, additional, Rolf, Andreas, additional, Breithecker, Andreas, additional, Adameit, Miriam S. D., additional, Kriechbaum, Steffen D., additional, Haas, Moritz, additional, Roller, Fritz, additional, Hamm, Christian W., additional, Ghofrani, H.-Ardeschir, additional, Mayer, Eckhard, additional, Guth, Stefan, additional, and Liebetrau, Christoph, additional

Published
2023
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25. CTEPH has shared and distinct genetic associations with pulmonary embolism in a genome-wide association study

Author

Liley, James, primary, Newnham, Michael, additional, Bleda, Marta, additional, Bunclark, Katherine, additional, Auger, William, additional, Barbera, Joan Albert, additional, Bogaard, Harm, additional, Delcroix, Marion, additional, Fernandes, Timothy M., additional, Howard, Luke, additional, Jenkins, David, additional, Lang, Irene, additional, Mayer, Eckhard, additional, Rhodes, Chris, additional, Simpson, Michael, additional, Southgate, Laura, additional, Trembath, Richard, additional, Wharton, John, additional, Wilkins, Martin R, additional, Gräf, Stefan, additional, Morrell, Nicholas, additional, Pepke Zaba, Joanna, additional, and Toshner, Mark, additional

Published
2023
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29. Value of Right and Left Ventricular T1 and T2 Blood Pool Mapping in Patients with Chronic Thromboembolic Hypertension before and after Balloon Pulmonary Angioplasty

Author

Roller, Fritz C., primary, Schüßler, Armin, additional, Kremer, Nils, additional, Harth, Sebastian, additional, Kriechbaum, Steffen D., additional, Wiedenroth, Christoph B., additional, Guth, Stefan, additional, Breithecker, Andreas, additional, Richter, Manuel, additional, Tello, Khodr, additional, Seeger, Werner, additional, Mayer, Eckhard, additional, and Krombach, Gabriele A., additional

Published
2023
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30. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

Author

Humbert, Marc, Kovacs, Gabor, Hoeper, Marius M., Badagliacca, Roberto, Berger, Rolf M.F., Brida, Margarita, Carlsen, Jørn, Coats, Andrew J.S., Escribano-Subias, Pilar, Ferrari, Pisana, Ferreira, Diogenes S., Ghofrani, Hossein Ardeschir, Giannakoulas, George, Kiely, David G., Mayer, Eckhard, Meszaros, Gergely, Nagavci, Blin, Olsson, Karen M., Pepke-Zaba, Joanna, Quint, Jennifer K., Rådegran, Göran, Simonneau, Gerald, Sitbon, Olivier, Tonia, Thomy, Toshner, Mark, Vachiery, Jean Luc, Vonk Noordegraaf, Anton, Delcroix, Marion, Rosenkranz, Stephan, Humbert, Marc, Kovacs, Gabor, Hoeper, Marius M., Badagliacca, Roberto, Berger, Rolf M.F., Brida, Margarita, Carlsen, Jørn, Coats, Andrew J.S., Escribano-Subias, Pilar, Ferrari, Pisana, Ferreira, Diogenes S., Ghofrani, Hossein Ardeschir, Giannakoulas, George, Kiely, David G., Mayer, Eckhard, Meszaros, Gergely, Nagavci, Blin, Olsson, Karen M., Pepke-Zaba, Joanna, Quint, Jennifer K., Rådegran, Göran, Simonneau, Gerald, Sitbon, Olivier, Tonia, Thomy, Toshner, Mark, Vachiery, Jean Luc, Vonk Noordegraaf, Anton, Delcroix, Marion, and Rosenkranz, Stephan

Published
2023

34. Right Ventricular Strain by Magnetic Resonance Feature Tracking Is Largely Afterload-Dependent and Does Not Reflect Contractility: Validation by Combined Volumetry and Invasive Pressure Tracings

Author

Rolf, Andreas, primary, Keller, Till, additional, Wolter, Jan Sebastian, additional, Kriechbaum, Steffen, additional, Weferling, Maren, additional, Guth, Stefan, additional, Wiedenroth, Christoph, additional, Mayer, Eckhard, additional, Hamm, Christian W., additional, Fischer-Rasokat, Ulrich, additional, and Treiber, Julia, additional

Published
2022
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35. Value of Right and Left Ventricular T1 and T2 Blood Pool Mapping in Patients with Chronic Thromboembolic Hypertension before and after Balloon Pulmonary Angioplasty

Author

Roller, Fritz C., Schüßler, Armin, Kremer, Nils, Harth, Sebastian, Kriechbaum, Steffen D., Wiedenroth, Christoph B., Guth, Stefan, Breithecker, Andreas, Richter, Manuel, Tello, Khodr, Seeger, Werner, Mayer, Eckhard, Krombach, Gabriele A., and Justus Liebig University Giessen

Subjects
T2 mapping, ddc:610, CTEPH, parametric imaging, CMR, T1 mapping
Abstract

Background: Parametric imaging has taken a steep rise in recent years and non-cardiac applications are of increasing interest. Therefore, the aim of our study was to assess right (RV) and left ventricular (LV) blood pool T1 and T2 values in patients with chronic thromboembolic pulmonary hypertension (CTEPH) compared to control subjects and their correlation to pulmonary hemodynamic. Methods: 26 patients with CTEPH (mean age 64.8 years ± 12.8 SD; 15 female), who underwent CMR and right heart catheterization (RHC) before and 6-months after balloon pulmonary angioplasty (BPA), were retrospectively included. Ventricular blood pool values were measured, compared to control subjects (mean age 40.5 years ± 12.8 SD; 16 female) and correlated to invasive measures (CI, mPAP, PVR). Results: In both, control subjects and CTEPH patients, RVT1 and RVT2 were significantly reduced compared to LVT1 and LVT2. Compared to control subjects, RVT2 was significantly reduced in CTEPH patients (p = 0.0065) and increased significantly after BPA (p = 0.0048). Moreover, RVT2 was positively correlated with CI and negatively correlated with mPAP and PVR before (r = 0.5155, r = −0.2541, r = −0.4571) and after BPA (r = 0.4769, r = −0.2585, r = −0.4396). Conclusion: Ventricular blood pool T2 mapping might be novel non-invasive CMR imaging marker for assessment of disease severity, prognosis, follow-up and even therapy monitoring in PH.

Published
2023
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39. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension Developed by the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by the International Society for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG)

Author

Humbert, Marc, Kovacs, Gabor, Hoeper, Marius M., Badagliacca, Roberto, Berger, Rolf M. F., Brida, Margarita, Carlsen, Jorn, Coats, Andrew J. S., Escribano-Subias, Pilar, Ferrari, Pisana, Ferreira, Diogenes S., Ghofrani, Hossein Ardeschir, Giannakoulas, George, Kiely, David G., Mayer, Eckhard, Meszaros, Gergely, Nagavci, Blin, Olsson, Karen M., Pepke-Zaba, Joanna, Quint, Jennifer K., Radegran, Goran, Simonneau, Gerald, Sitbon, Olivier, Tonia, Thomy, Toshner, Mark, Vachiery, Jean-Luc, Noordegraaf, Anton Vonk, Delcroix, Marion, Rosenkranz, Stephan, Schwerzmann, Markus, Anh-Tuan Dinh-Xuan, Bush, Andy, Abdelhamid, Magdy, Aboyans, Victor, Arbustini, Eloisa, Asteggiano, Riccardo, Barbera, Joan-Albert, Beghetti, Maurice, Cikes, Maja, Condliffe, Robin, de Man, Frances, Falk, Volkmar, Fauchier, Laurent, Gaine, Sean, Galie, Nazzareno, Gin-Sing, Wendy, Granton, John, Grunig, Ekkehard, Hassoun, Paul M., Hellemons, Merel, and Cardiovascular Centre (CVC)

Subjects
ENDOTHELIN-RECEPTOR ANTAGONIST, Guidelines, Pulmonary arterial hypertension, Pulmonary hypertension, Chronic thrombo-embolic pulmonary hypertension, LONG-TERM OUTCOMES, QUALITY-OF-LIFE, Balloon pulmonary angioplasty, Soluble guanylate cyclase stimulators, Connective tissue disease, CALCIUM-CHANNEL BLOCKERS, Congenital heart disease, RIGHT-VENTRICULAR DYSFUNCTION, IMPROVES EXERCISE CAPACITY, Endothelin receptor antagonists, Lung transplantation, PRESERVED EJECTION FRACTION, EOSINOPHILIA-MYALGIA-SYNDROME, Pulmonary endarterectomy, Left heart disease, Lung disease, Prostacyclin receptor agonists, Prostacyclin analogues, AFFAIRS CLINICAL-ASSESSMENT, Phosphodiesterase type 5 inhibitors, CONTINUOUS INTRAVENOUS EPOPROSTENOL
Published
2022

40. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension:Developed by the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by the International Society for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG)

Author

Humbert, Marc, Kovacs, Gabor, Hoeper, Marius M, Badagliacca, Roberto, Berger, Rolf M F, Brida, Margarita, Carlsen, Jørn, Coats, Andrew J S, Escribano-Subias, Pilar, Ferrari, Pisana, Ferreira, Diogenes S, Ghofrani, Hossein Ardeschir, Giannakoulas, George, Kiely, David G, Mayer, Eckhard, Meszaros, Gergely, Nagavci, Blin, Olsson, Karen M, Pepke-Zaba, Joanna, Quint, Jennifer K, Rådegran, Göran, Simonneau, Gerald, Sitbon, Olivier, Tonia, Thomy, Toshner, Mark, Vachiery, Jean Luc, Vonk Noordegraaf, Anton, Delcroix, Marion, Rosenkranz, Stephan, and Nielsen, Jens Cosedis

Subjects
Endothelin Receptor Antagonists, Hypertension, Pulmonary/diagnosis, Humans
Published
2022
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44. Late outcomes after acute pulmonary embolism: rationale and design of FOCUS, a prospective observational multicenter cohort study

Author

Konstantinides, Stavros V., Barco, Stefano, Rosenkranz, Stephan, Lankeit, Mareike, Held, Matthias, Gerhardt, Felix, Bruch, Leonard, Ewert, Ralf, Faehling, Martin, Freise, Julia, Ghofrani, Hossein-Ardeschir, Grünig, Ekkehard, Halank, Michael, Heydenreich, Nadine, Hoeper, Marius M., Leuchte, Hanno H., Mayer, Eckhard, Meyer, F. Joachim, Neurohr, Claus, Opitz, Christian, Pinto, Antonio, Seyfarth, Hans-Jürgen, Wachter, Rolf, Zäpf, Bianca, Wilkens, Heinrike, Binder, Harald, and Wild, Philipp S.

Published
2016
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45. Complications of balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension: Impact on the outcome

Author

Wiedenroth, Christoph B., primary, Deissner, Henrike, additional, Adameit, Miriam S.D., additional, Kriechbaum, Steffen D., additional, Ghofrani, H.-Ardeschir, additional, Breithecker, Andreas, additional, Haas, Moritz, additional, Roller, Fritz, additional, Rolf, Andreas, additional, Hamm, Christian W., additional, Mayer, Eckhard, additional, Guth, Stefan, additional, and Liebetrau, Christoph, additional

Published
2022
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46. Chronic thromboembolic pulmonary hypertension and impairment after pulmonary embolism: the FOCUS study

Author

Valerio, Luca, Mavromanoli, Anna C., Barco, Stefano, Abele, Christina, Becker, Dorothea, Bruch, Leonhard, Ewert, Ralf, Faehling, Martin, Fistera, David, Gerhardt, Felix, Ghofrani, Hossein-Ardeschir, Grgic, Aleksandar, Gruenig, Ekkehard, Halank, Michael, Held, Matthias, Hobohm, Lukas, Hoeper, Marius M., Klok, Frederikus A., Lankeit, Mareike, Leuchte, Hanno H., Martin, Nadine, Mayer, Eckhard, Meyer, F. Joachim, Neurohr, Claus, Opitz, Christian, Schmidt, Kai-Helge, Seyfarth, Hans-Jurgen, Wachter, Rolf, Wilkens, Heinrike, Wild, Philipp S., Konstantinides, Stavros, V, Rosenkranz, Stephan, Valerio, Luca, Mavromanoli, Anna C., Barco, Stefano, Abele, Christina, Becker, Dorothea, Bruch, Leonhard, Ewert, Ralf, Faehling, Martin, Fistera, David, Gerhardt, Felix, Ghofrani, Hossein-Ardeschir, Grgic, Aleksandar, Gruenig, Ekkehard, Halank, Michael, Held, Matthias, Hobohm, Lukas, Hoeper, Marius M., Klok, Frederikus A., Lankeit, Mareike, Leuchte, Hanno H., Martin, Nadine, Mayer, Eckhard, Meyer, F. Joachim, Neurohr, Claus, Opitz, Christian, Schmidt, Kai-Helge, Seyfarth, Hans-Jurgen, Wachter, Rolf, Wilkens, Heinrike, Wild, Philipp S., Konstantinides, Stavros, V, and Rosenkranz, Stephan

Abstract

Aims To systematically assess late outcomes of acute pulmonary embolism (PE) and to investigate the clinical implications of post-PE impairment (PPEI) fulfilling prospectively defined criteria. Methods and results A prospective multicentre observational cohort study was conducted in 17 large-volume centres across Germany. Adult consecutive patients with confirmed acute symptomatic PE were followed with a standardized assessment plan and pre-defined visits at 3, 12, and 24 months. The co-primary outcomes were (i) diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH), and (ii) PPEI, a combination of persistent or worsening clinical, functional, biochemical, and imaging parameters during follow-up. A total of 1017 patients (45% women, median age 64 years) were included in the primary analysis. They were followed for a median duration of 732 days after PE diagnosis. The CTEPH was diagnosed in 16 (1.6%) patients, after a median of 129 days; the estimated 2-year cumulative incidence was 2.3% (1.2-4.4%). Overall, 880 patients were evaluable for PPEI; the 2-year cumulative incidence was 16.0% (95% confidence interval 12.8-20.8%). The PPEI helped to identify 15 of the 16 patients diagnosed with CTEPH during follow-up (hazard ratio for CTEPH vs. no CTEPH 393; 95% confidence interval 73-2119). Patients with PPEI had a higher risk of re-hospitalization and death as well as worse quality of life compared with those without PPEI. Conclusion In this prospective study, the cumulative 2-year incidence of CTEPH was 2.3%, but PPEI diagnosed by standardized criteria was frequent. Our findings support systematic follow-up of patients after acute PE and may help to optimize guideline recommendations and algorithms for post-PE care.

Published
2022

47. Chronic thromboembolic pulmonary hypertension and impairment after pulmonary embolism: the FOCUS study

Author

Valerio, Luca; https://orcid.org/0000-0003-4466-0724, Mavromanoli, Anna C, Barco, Stefano; https://orcid.org/0000-0002-2618-347X, Abele, Christina, Becker, Dorothea, Bruch, Leonhard, Ewert, Ralf, Faehling, Martin, Fistera, David, Gerhardt, Felix, Ghofrani, Hossein-Ardeschir, Grgic, Aleksandar, Grünig, Ekkehard, Halank, Michael, Held, Matthias, Hobohm, Lukas; https://orcid.org/0000-0002-4312-0366, Hoeper, Marius M; https://orcid.org/0000-0001-9086-2293, Klok, Frederikus A; https://orcid.org/0000-0001-9961-0754, Lankeit, Mareike, Leuchte, Hanno H, Martin, Nadine, Mayer, Eckhard, Meyer, F Joachim, Neurohr, Claus, Opitz, Christian, Schmidt, Kai-Helge, Seyfarth, Hans-Jürgen, Wachter, Rolf; https://orcid.org/0000-0003-2231-2200, Wilkens, Heinrike, Wild, Philipp S, et al, Valerio, Luca; https://orcid.org/0000-0003-4466-0724, Mavromanoli, Anna C, Barco, Stefano; https://orcid.org/0000-0002-2618-347X, Abele, Christina, Becker, Dorothea, Bruch, Leonhard, Ewert, Ralf, Faehling, Martin, Fistera, David, Gerhardt, Felix, Ghofrani, Hossein-Ardeschir, Grgic, Aleksandar, Grünig, Ekkehard, Halank, Michael, Held, Matthias, Hobohm, Lukas; https://orcid.org/0000-0002-4312-0366, Hoeper, Marius M; https://orcid.org/0000-0001-9086-2293, Klok, Frederikus A; https://orcid.org/0000-0001-9961-0754, Lankeit, Mareike, Leuchte, Hanno H, Martin, Nadine, Mayer, Eckhard, Meyer, F Joachim, Neurohr, Claus, Opitz, Christian, Schmidt, Kai-Helge, Seyfarth, Hans-Jürgen, Wachter, Rolf; https://orcid.org/0000-0003-2231-2200, Wilkens, Heinrike, Wild, Philipp S, and et al

Abstract

AIMS: To systematically assess late outcomes of acute pulmonary embolism (PE) and to investigate the clinical implications of post-PE impairment (PPEI) fulfilling prospectively defined criteria. METHODS AND RESULTS: A prospective multicentre observational cohort study was conducted in 17 large-volume centres across Germany. Adult consecutive patients with confirmed acute symptomatic PE were followed with a standardized assessment plan and pre-defined visits at 3, 12, and 24 months. The co-primary outcomes were (i) diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH), and (ii) PPEI, a combination of persistent or worsening clinical, functional, biochemical, and imaging parameters during follow-up. A total of 1017 patients (45% women, median age 64 years) were included in the primary analysis. They were followed for a median duration of 732 days after PE diagnosis. The CTEPH was diagnosed in 16 (1.6%) patients, after a median of 129 days; the estimated 2-year cumulative incidence was 2.3% (1.2-4.4%). Overall, 880 patients were evaluable for PPEI; the 2-year cumulative incidence was 16.0% (95% confidence interval 12.8-20.8%). The PPEI helped to identify 15 of the 16 patients diagnosed with CTEPH during follow-up (hazard ratio for CTEPH vs. no CTEPH 393; 95% confidence interval 73-2119). Patients with PPEI had a higher risk of re-hospitalization and death as well as worse quality of life compared with those without PPEI. CONCLUSION: In this prospective study, the cumulative 2-year incidence of CTEPH was 2.3%, but PPEI diagnosed by standardized criteria was frequent. Our findings support systematic follow-up of patients after acute PE and may help to optimize guideline recommendations and algorithms for post-PE care.

Published
2022

50. Доклад ERS по хронической тромбоэмболической легочной гипертензии

Author

Delcroix, Marion, Torbicki, Adam, Gopalan, Deepa, Sitbon, Olivier, Klok, Frederikus A., Lang, Irene, Jenkins, David, Kim, Nick H., Humbert, Marc, Jais, Xavier, Vonk Noordegraaf, Anton, Pepke-Zaba, Joanna, Brénot, Philippe, Dorfmuller, Peter, Fadel, Elie, Ghofrani, Hossein-Ardeschir, Hoeper, Marius M., Jansa, Pavel, Madani, Michael, Matsubara, Hiromi, Ogo, Takeshi, Grunig, Ekkehard, D'Armini, Andrea, Galie, Nazzareno, Meyer, Bernhard, Corkery, Patrick, Meszaros, Gergely, Mayer, Eckhard, Simonneau, G. rald, Pulmonary medicine, and ACS - Pulmonary hypertension & thrombosis

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism, either symptomatic or not. The occlusion of proximal pulmonary arteries by fibrotic intravascular material, in combination with a secondary microvasculopathy of vessels < 500 μm, leads to increased pulmonary vascular resistance and progressive right heart failure. The mechanism responsible for the transformation of red clots into fibrotic material remnants has not yet been elucidated. In patients with pulmonary hypertension, the diagnosis is suspected when a ventilation/perfusion lung scan shows mismatched perfusion defects, and confirmed by right heart catheterisation and vascular imaging. Today, in addition to lifelong anticoagulation, treatment modalities include surgery, angioplasty and medical treatment according to the localisation and characteristics of the lesions. This statement outlines a review of the literature and current practice concerning diagnosis and management of CTEPH. It covers the definitions, diagnosis, epidemiology, follow-up after acute pulmonary embolism, pathophysiology, treatment by pulmonary endarterectomy, balloon pulmonary angioplasty, drugs and their combination, rehabilitation and new lines of research in CTEPH. It represents the first collaboration of the European Respiratory Society, the International CTEPH Association and the European Reference Network-Lung in the pulmonary hypertension domain. The statement summarises current knowledge, but does not make formal recommendations for clinical practice.

Published
2022
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