Christian Opitz1, Ekkehard Grünig2, Stephan Rosenkranz3, Ann-Sophie Kaemmerer4, Matthias Gorenflo5, Dörte Huscher6, David Pittrow7, Peter Ewert4, Christine Pausch8, Marion Delcroix9, Hossein A Ghofrani10, Marius M Hoeper11, Rainer Kozlik-Feldmann12, Andris Skride13, Gerd Stähler14, Carmine Dario Vizza15, Elena Jureviciene16, Dovile Jancauskaite16, Lina Gumbiene16, Ralf Ewert17, Ingo Dähnert18, Matthias Held19, Michael Halank20, Dirk Skowasch21, Hans Klose22, Heinrike Wilkens23, Katrin Milger24, Christian Jux25, Martin Koestenberger26, Laura Scelsi27, Eva Brunnemer28, Michael Hofbeck29, Silvia Ulrich30, Anton Vonk Noordegraaf31, Tobias J Lange32, Leonhard Bruch33, Stavros Konstantinides34, Martin Claussen35, Judith Löffler-Ragg36, Hubert Wirtz37, Christian Apitz38, Rhoia Neidenbach4, Sebastian Freilinger4, Attila Nemes39. 1. DRK Kliniken Berlin Westend, Klinik für Innere Medizin, Schwerpunkt Kardiologie, Berlin, Germany. 2. Thoraxklinik Heidelberg gGmbH, Zentrum für Pulmonale Hypertonie, Heidelberg, and German Centre for Lung Research, Heidelberg, Germany. 3. Universitätsklinik Köln - Herzzentrum, Klinik III für Innere Medizin, Köln, Germany. 4. Deutsches Herzzentrum München, Klinik für angeborene Herzfehler und Kinderkardiologie, München, Technische Universität München, and Deutsches Zentrum für Herz-Kreislauf-Forschung (DZHK), Munich Heart Alliance, Munich, Germany. 5. Universitätsklinikum Heidelberg, Zentrum für Kinder- und Jugendmedizin, Angelika-Lautenschläger-Klinik, Heidelberg, Germany. 6. Institute of Biometry and Clinical Epidemiology, and Berlin Institute of Health, Charité-Universitätsmedizin, Berlin, Germany. 7. Medical Faculty, Institute for Clinical Pharmacology, Technical University, Dresden, Germany. 8. Epidemiological Centre, GWT-TUD GmbH, Dresden, Germany. 9. UZ Leuven, Campus Gasthuisberg, Department of Pneumology, Leuven, Belgium. 10. Universitätsklinikum Gießen und Marburg GmbH, Medizinische Klinik II/V, Gießen, Germany. 11. Medizinische Hochschule Hannover, Abt. Pneumologie, Hannover, Germany. 12. Universitäres Herzzentrum Hamburg, Klinik und Poliklinik für Kinderherzmedizin und Erwachsene mit angeborenen Herzfehlern, Hamburg, Germany. 13. Riga Stradins University, Clinical University Hospital, Riga, Lativa. 14. Klinik Löwenstein, Medizinische Klinik I, Löwenstein, Germany. 15. Pulmonary Hypertension Center, Department of Clinical Anestesiologic and Cardiovascular Sciences, University of Rome La Sapienza, Rome, Italy. 16. Faculty of Medicine of Vilnius University, Referal Centre of Pulmonary Hypertension, Vilnius University Hospital Santaros Klinikos, Vilnius, Lithuania. 17. Universitätsmedizin Greifswald, Zentrum für Innere Medizin, Klinik und Poliklinik für Innere Medizin B, Greifswald, Germany. 18. Herzzentrum Leipzig GmbH, Klinik für Kinderkardiologie, Leipzig, Germany. 19. Med. Klinik mit Schwerpunkt Pneumologie und Beatmungsmedizin, Klinikum Würzburg Mitte, Standort Missioklinik, Würzburg, Germany. 20. Universitätsklinikum Carl Gustav Carus der Technischen Universität Dresden, Medizinische Klinik und Poliklinik I, Dresden, Germany. 21. Universitätsklinikum Bonn, Medizinische Klinik und Poliklinik II, Innere Medizin - Kardiologie/Pneumologie, Bonn. 22. Abteilung für Pneumologie, Zentrum für Onkologie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany. 23. Universitätsklinikum des Saarlandes, Innere Medizin V, Homburg, Germany. 24. LMU Klinikum, Medizinische Klinik und Poliklinik V, München, Germany. 25. Kinderherzzentrum und Zentrum für angeborene Herzfehler, Justus-Liebig-Universität, Zentrum für Kinderheilkunde, Abteilung Kinderkardiologie, Gießen, Germany. 26. LKH - Univ. Klinikum Graz, Universitätsklinik für Kinder- und Jugendheilkunde, Abteilung für Pädiatrische Kardiologie, Graz, Austria. 27. Fondazione IRCCS Policlinico San Matteo University of Pavia, Pavia, Italy. 28. Medizinische Universitätsklinik (Krehl-Klinik), Klinik für Kardiologie, Angiologie und Pneumologie (Innere Medizin III), Heidelberg, Germany. 29. Universitätsklinik für Kinder- und Jugendmedizin Tübingen, Kinderkardiologie, Pulmologie, Intensivmedizin, Tübingen, Germany. 30. Universitätsspital Zürich, Klinik für Pneumologie, Zürich, Switzerland. 31. Amsterdam UMC, Vrije Universiteit Amsterdam, dept of Pulmonary Medicine, Amsterdam, Netherlands. 32. Universitätsklinikum Regensburg, Medizinische Klinik und Poliklinik II, Regensburg, Germany. 33. Unfallkrankenhaus Berlin, Klinik für Innere Medizin, Berlin, Germany. 34. Universitätsmedizin Mainz, Center for Thrombosis and Hemostasis, Mainz, Germany. 35. LungenClinic Grosshansdorf, Fachabteilung Pneumologie, Großhansdorf, Germany. 36. Universitätsklinik für Innere Medizin II, Innsbruck, Austria. 37. Universitätsklinikum Leipzig, Medizinische Klinik und Poliklinik I, Abteilung für Pneumologie, Leipzig, Germany. 38. Universitätsklinik für Kinder- und Jugendmedizin, Sektion Pädiatrische Kardiologie, Ulm, Germany. 39. University of Szeged, Department of Medicine, Szeged, Hungary.
Abstract
BACKGROUND: Pulmonary arterial hypertension (PAH) is common in congenital heart disease (CHD). Because clinical-trial data on PAH associated with CHD (PAH-CHD) remain limited, registry data on the long-term course are essential. This analysis aimed to update information from the COMPERA-CHD registry on management strategies based on real-world data. METHODS: The prospective international pulmonary hypertension registry COMPERA has since 2007 enrolled more than 10,000 patients. COMPERA-CHD is a sub-registry for patients with PAH-CHD. RESULTS: A total of 769 patients with PAH-CHD from 62 specialized centers in 12 countries were included into COMPERA-CHD from January 2007 through September 2020. At the last follow-up in 09/2020, patients [mean age 45.3±16.8 years; 512 (66%) female] had either post-tricuspid shunts (n=359; 46.7%), pre-tricuspid shunts (n=249; 32.4%), complex CHD (n=132; 17.2%), congenital left heart or aortic valve or aortic disease (n=9; 1.3%), or miscellaneous CHD (n=20; 2.6%). The mean 6-minute walking distance was 369±121 m, and 28.2%, 56.0%, and 3.8% were in WHO functional class I/II, III or IV, respectively (12.0% unknown). Compared with the previously published COMPERA-CHD data, after 21 months of follow-up, the number of included PAH-CHD patients increased by 91 (13.4%). Within this group the number of Eisenmenger patients rose by 39 (16.3%), the number of "Non-Eisenmenger PAH" patients by 45 (26.9%). Currently, among the 674 patients from the PAH-CHD group with at least one follow-up, 450 (66.8%) received endothelin receptor antagonists (ERA), 416 (61.7%) PDE-5 inhibitors, 85 (12.6%) prostacyclin analogues, and 36 (5.3%) the sGC stimulator riociguat. While at first inclusion in the COMPERA-CHD registry, treatment was predominantly monotherapy (69.3%), this has shifted to favoring combination therapy in the current group (53%). For the first time, the nature, frequency, and treatment of significant comorbidities requiring supportive care and medication are described. CONCLUSIONS: Analyzing "real life data" from the international COMPERA-CHD registry, we present a comprehensive overview about current management modalities and treatment concepts in PAH-CHD. There was an trend towards more aggressive treatment strategies and combination therapies. In the future, particular attention must be directed to the "Non-Eisenmenger PAH" group and to patients with complex CHD, including Fontan patients. TRIAL REGISTRATION: www.clinicaltrials.gov, study identifier: Clinicaltrials.gov NCT01347216. 2021 Cardiovascular Diagnosis and Therapy. All rights reserved.
BACKGROUND: Pulmonary arterial hypertension (PAH) is common in congenital heart disease (CHD). Because clinical-trial data on PAH associated with CHD (PAH-CHD) remain limited, registry data on the long-term course are essential. This analysis aimed to update information from the COMPERA-CHD registry on management strategies based on real-world data. METHODS: The prospective international pulmonary hypertension registry COMPERA has since 2007 enrolled more than 10,000 patients. COMPERA-CHD is a sub-registry for patients with PAH-CHD. RESULTS: A total of 769 patients with PAH-CHD from 62 specialized centers in 12 countries were included into COMPERA-CHD from January 2007 through September 2020. At the last follow-up in 09/2020, patients [mean age 45.3±16.8 years; 512 (66%) female] had either post-tricuspid shunts (n=359; 46.7%), pre-tricuspid shunts (n=249; 32.4%), complex CHD (n=132; 17.2%), congenital left heart or aortic valve or aortic disease (n=9; 1.3%), or miscellaneous CHD (n=20; 2.6%). The mean 6-minute walking distance was 369±121 m, and 28.2%, 56.0%, and 3.8% were in WHO functional class I/II, III or IV, respectively (12.0% unknown). Compared with the previously published COMPERA-CHD data, after 21 months of follow-up, the number of included PAH-CHD patients increased by 91 (13.4%). Within this group the number of Eisenmenger patients rose by 39 (16.3%), the number of "Non-Eisenmenger PAH" patients by 45 (26.9%). Currently, among the 674 patients from the PAH-CHD group with at least one follow-up, 450 (66.8%) received endothelin receptor antagonists (ERA), 416 (61.7%) PDE-5 inhibitors, 85 (12.6%) prostacyclin analogues, and 36 (5.3%) the sGC stimulator riociguat. While at first inclusion in the COMPERA-CHD registry, treatment was predominantly monotherapy (69.3%), this has shifted to favoring combination therapy in the current group (53%). For the first time, the nature, frequency, and treatment of significant comorbidities requiring supportive care and medication are described. CONCLUSIONS: Analyzing "real life data" from the international COMPERA-CHD registry, we present a comprehensive overview about current management modalities and treatment concepts in PAH-CHD. There was an trend towards more aggressive treatment strategies and combination therapies. In the future, particular attention must be directed to the "Non-Eisenmenger PAH" group and to patients with complex CHD, including Fontan patients. TRIAL REGISTRATION: www.clinicaltrials.gov, study identifier: Clinicaltrials.gov NCT01347216. 2021 Cardiovascular Diagnosis and Therapy. All rights reserved.
Authors: Annelieke C M J van Riel; Mark J Schuuring; Irene D van Hessen; Aielko H Zwinderman; Luc Cozijnsen; Constant L A Reichert; Jan C A Hoorntje; Lodewijk J Wagenaar; Marco C Post; Arie P J van Dijk; Elke S Hoendermis; Barbara J M Mulder; Berto J Bouma Journal: Int J Cardiol Date: 2014-04-16 Impact factor: 4.164
Authors: H Ardeschir Ghofrani; Oliver Distler; Felix Gerhardt; Matthias Gorenflo; Ekkehard Grünig; Walter E Haefeli; Matthias Held; Marius M Hoeper; Christian M Kähler; Harald Kaemmerer; Hans Klose; Volker Köllner; Bruno Kopp; Siegrun Mebus; Andreas Meyer; Oliver Miera; David Pittrow; Gabriela Riemekasten; Stephan Rosenkranz; Dietmar Schranz; Robert Voswinckel; Horst Olschewski Journal: Int J Cardiol Date: 2011-12 Impact factor: 4.164
Authors: Karen M Olsson; Marion Delcroix; H Ardeschir Ghofrani; Henning Tiede; Doerte Huscher; Rudolf Speich; Ekkehard Grünig; Gerd Staehler; Stephan Rosenkranz; Michael Halank; Matthias Held; Tobias J Lange; Juergen Behr; Hans Klose; Martin Claussen; Ralf Ewert; Christian F Opitz; C Dario Vizza; Laura Scelsi; Anton Vonk-Noordegraaf; Harald Kaemmerer; J Simon R Gibbs; Gerry Coghlan; Joanna Pepke-Zaba; Uwe Schulz; Matthias Gorenflo; David Pittrow; Marius M Hoeper Journal: Circulation Date: 2013-09-30 Impact factor: 29.690
Authors: Mm Idrees; K Al-Najashi; A Khan; S Al-Dammas; H Al-Awwad; E Batubara; A Al Otai; J Abdulhameed; A Fayed; T Kashour Journal: Ann Thorac Med Date: 2014-10 Impact factor: 2.219
Authors: Pavel Jansa; Jiri Jarkovsky; Hikmet Al-Hiti; Jana Popelova; David Ambroz; Tomas Zatocil; Regina Votavova; Pavel Polacek; Jana Maresova; Michael Aschermann; Petr Brabec; Ladislav Dusek; Ales Linhart Journal: BMC Pulm Med Date: 2014-03-15 Impact factor: 3.317