{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,12,18]],"date-time":"2024-12-18T05:31:03Z","timestamp":1734499863145,"version":"3.30.2"},"reference-count":70,"publisher":"Georg Thieme Verlag KG","issue":"08","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pneumologie"],"published-print":{"date-parts":[[2024,8]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>Die Zahl der Erwachsenen mit angeborenen Herzfehlern (AHF) steigt st\u00e4ndig und liegt in Deutschland bei etwa 360\u200a000. AHF sind h\u00e4ufig mit einer pulmonalen Hypertonie (PH) assoziiert, die sich bei unbehandelten AHF teils schon fr\u00fchzeitig entwickeln. Trotz einer zeitgerechten Behandlung des AHF persistiert eine PH h\u00e4ufig oder entwickelt sich im h\u00f6heren Lebensalter neu und ist mit erheblicher Morbidit\u00e4t und Letalit\u00e4t behaftet.<\/jats:p><jats:p>Die \u00fcberarbeiteten Leitlinien (LL) der European Society of Cardiology\/European Respiratory Society 2022 f\u00fcr die Diagnostik und Behandlung der PH stellen einen wesentlichen Beitrag zur optimierten Versorgung der Betroffenen dar. Der Themenbereich \u201eErwachsene mit angeborenen Herzfehlern\u201c wird in diesem Zusammenhang allerdings nur relativ oberfl\u00e4chlich behandelt. In dem vorliegenden Artikel wird diese Thematik daher detailliert aus Sicht der kongenitalen Kardiologie kommentiert.<\/jats:p>","DOI":"10.1055\/a-2274-1025","type":"journal-article","created":{"date-parts":[[2024,5,24]],"date-time":"2024-05-24T22:59:18Z","timestamp":1716591558000},"page":"566-577","source":"Crossref","is-referenced-by-count":0,"title":["Pulmonale Hypertonie bei Erwachsenen mit angeborenen Herzfehlern im Lichte der 2022-ESC-PAH-Leitlinien"],"prefix":"10.1055","volume":"78","author":[{"given":"Harald","family":"Kaemmerer","sequence":"additional","affiliation":[{"name":"Internationales Zentrum f\u00fcr Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik f\u00fcr angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum M\u00fcnchen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gerhard Paul","family":"Diller","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Kardiologie III: angeborene Herzfehler (EMAH) und Klappenerkrankungen, Universit\u00e4tsklinikum M\u00fcnster, M\u00fcnster, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephan","family":"Achenbach","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsklinik Erlangen, Medizinische Klinik 2 \u2013 Kardiologie und Angiologie, Erlangen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ingo","family":"D\u00e4hnert","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsklinik f\u00fcr Kinderkardiologie, Herzzentrum Leipzig, Leipzig, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christina A.","family":"Eichstaedt","sequence":"additional","affiliation":[{"name":"Zentrum f\u00fcr Pulmonale Hypertonie, Thoraxklinik Heidelberg am Universit\u00e4tsklinikum Heidelberg, Heidelberg, Deutschland; Institut f\u00fcr Humangenetik, Universit\u00e4t Heidelberg, INF 366, TLRC am DZL Heidelberg, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Eicken","sequence":"additional","affiliation":[{"name":"Internationales Zentrum f\u00fcr Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik f\u00fcr angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum M\u00fcnchen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annika","family":"Freiberger","sequence":"additional","affiliation":[{"name":"Internationales Zentrum f\u00fcr Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik f\u00fcr angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum M\u00fcnchen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sebastian","family":"Freilinger","sequence":"additional","affiliation":[{"name":"Internationales Zentrum f\u00fcr Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik f\u00fcr angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum M\u00fcnchen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ralf","family":"Geiger","sequence":"additional","affiliation":[{"name":"Univ.-Klinik f\u00fcr P\u00e4diatrie III, Kardiologie, Pneumologie, Allergologie, Cystische Fibrose, Innsbruck, \u00d6sterreich"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthias","family":"Gorenflo","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Kinderkardiologie und angeborene Herzfehler, Universit\u00e4tsklinikum Heidelberg, Heidelberg, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ekkehard","family":"Gr\u00fcnig","sequence":"additional","affiliation":[{"name":"Zentrum f\u00fcr Pulmonale Hypertonie, Thoraxklinik Heidelberg am Universit\u00e4tsklinikum Heidelberg, Heidelberg, Deutschland; Institut f\u00fcr Humangenetik, Universit\u00e4t Heidelberg, INF 366, TLRC am DZL Heidelberg, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alfred","family":"Hager","sequence":"additional","affiliation":[{"name":"Internationales Zentrum f\u00fcr Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik f\u00fcr angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum M\u00fcnchen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Huntgeburth","sequence":"additional","affiliation":[{"name":"Internationales Zentrum f\u00fcr Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik f\u00fcr angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum M\u00fcnchen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ann-Sophie","family":"Kaemmerer-Suleiman","sequence":"additional","affiliation":[{"name":"Herzchirurgische Klinik, Universit\u00e4tsklinikum Erlangen, Erlangen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rainer","family":"Kozlik-Feldmann","sequence":"additional","affiliation":[{"name":"Klinik und Poliklinik f\u00fcr Kinderkardiologie, Universit\u00e4res Herz- und Gef\u00e4\u00dfzentrum Hamburg, Klinik und Poliklinik f\u00fcr Kinderherzmedizin und Erwachsene mit angeborenen Herzfehlern, Hamburg, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Astrid E.","family":"Lammers","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr P\u00e4diatrische Kardiologie, Universit\u00e4tsklinikum M\u00fcnster, Albert-Schweitzer-Campus 1, M\u00fcnster, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicole","family":"Nagdyman","sequence":"additional","affiliation":[{"name":"Internationales Zentrum f\u00fcr Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik f\u00fcr angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum M\u00fcnchen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sebastian","family":"Michel","sequence":"additional","affiliation":[{"name":"LMU Klinikum, Herzchirurgische Klinik und Poliklinik, Sektion f\u00fcr Chirurgie angeborener Herzfehler und Kinderherzchirurgie, Campus Gro\u00dfhadern, M\u00fcnchen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kai Helge","family":"Schmidt","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsmedizin Mainz, Zentrum f\u00fcr Kardiologie \u2013 Kardiologie I, Johannes Gutenberg-Universit\u00e4t Mainz, Mainz, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anselm","family":"Uebing","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsklinikum Schleswig-Holstein, Klinik f\u00fcr angeborene Herzfehler und Kinderkardiologie, Kiel, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fabian","family":"von Scheidt","sequence":"additional","affiliation":[{"name":"Internationales Zentrum f\u00fcr Erwachsene mit angeborenen Herzfehlern (EMAH), Klinik f\u00fcr angeborene Herzfehler und Kinderkardiologie, Deutsches Herzzentrum M\u00fcnchen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian","family":"Apitz","sequence":"additional","affiliation":[{"name":"Sektion P\u00e4diatrische Kardiologie, Universit\u00e4tsklinik f\u00fcr Kinder- und Jugendmedizin Ulm, Ulm, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2024,5,24]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1093\/eurheartj\/ehaa554","article-title":"2020 ESC Guidelines for the management of adult congenital heart disease","volume":"42","author":"H Baumgartner","year":"2021","journal-title":"Eur Heart J"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"888","DOI":"10.1183\/09031936.00145608","article-title":"Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review","volume":"34","author":"G Kovacs","year":"2009","journal-title":"Eur Respir J"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.jjcc.2014.09.002","article-title":"Current concept of transcatheter closure of atrial septal defect in adults","volume":"65","author":"T Akagi","year":"2015","journal-title":"J Cardiol"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"475","DOI":"10.21037\/cdt-21-798","article-title":"A multi-center trial on efficacy and safety of the LifeTech CeraFlex(TM) ASD occluder for transcatheter closure in patients with secundum atrial septal defects","volume":"12","author":"C Fritz","year":"2022","journal-title":"Cardiovasc Diagn Ther"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1002\/ccd.27899","article-title":"A randomized, controlled, multi-center trial of the efficacy and safety of the Occlutech Figulla Flex-II Occluder compared to the Amplatzer Septal Occluder for transcatheter closure of secundum atrial septal defects","volume":"93","author":"D Kenny","year":"2019","journal-title":"Catheter Cardiovasc Interv"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"1266","DOI":"10.1016\/j.jacc.2019.12.070","article-title":"Transcatheter Correction of Superior Sinus Venosus Atrial Septal Defects as an Alternative to Surgical Treatment","volume":"75","author":"J H Hansen","year":"2020","journal-title":"J Am Coll Cardiol"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.1002\/ccd.30650","article-title":"Transcatheter versus surgical treatment for isolated superior sinus venosus atrial septal defect","volume":"101","author":"F Brancato","year":"2023","journal-title":"Catheter Cardiovasc Interv"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"1069","DOI":"10.1002\/ccd.28150","article-title":"Temporary balloon occlusion of atrial septal defects in suspected or documented left ventricular diastolic dysfunction: Hemodynamic and clinical findings","volume":"93","author":"W R Miranda","year":"2019","journal-title":"Catheter Cardiovasc Interv"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1002\/ccd.20292","article-title":"Left ventricular conditioning in the elderly patient to prevent congestive heart failure after transcatheter closure of atrial septal defect","volume":"64","author":"S Schubert","year":"2005","journal-title":"Catheter Cardiovasc Interv"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1111\/chd.12367","article-title":"Fenestrated Transcatheter ASD Closure in Adults with Diastolic Dysfunction and\/or Pulmonary Hypertension: Case Series and Review of the Literature","volume":"11","author":"A Abdelkarim","year":"2016","journal-title":"Congenit Heart Dis"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"403","DOI":"10.4244\/EIJ-D-19-00342","article-title":"The AFR-PRELIEVE trial: a prospective, non-randomised, pilot study to assess the Atrial Flow Regulator (AFR) in heart failure patients with either preserved or reduced ejection fraction","volume":"15","author":"C Paitazoglou","year":"2019","journal-title":"EuroIntervention"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1007\/s00246-020-02456-2","article-title":"Short- and Long-term Outcome After Interventional VSD Closure: A Single-Center Experience in Pediatric and Adult Patients","volume":"42","author":"M Bergmann","year":"2021","journal-title":"Pediatr Cardiol"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"2361","DOI":"10.1093\/eurheartj\/ehm314","article-title":"Transcatheter closure of congenital ventricular septal defects: results of the European Registry","volume":"28","author":"M Carminati","year":"2007","journal-title":"Eur Heart J"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1017\/S1047951121002985","article-title":"Percutaneous closure of ventricular septal defect using LifeTech(TM) Konar-MF VSD Occluder: initial and short-term multi-institutional results","volume":"32","author":"M Sadiq","year":"2022","journal-title":"Cardiol Young"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"382","DOI":"10.1007\/s00392-020-01750-6","article-title":"Long-term outcome of perimembranous VSD closure using the Nit-Occlud\u00ae L\u00ea VSD coil system","volume":"110","author":"R Kozlik-Feldmann","year":"2021","journal-title":"Clin Res Cardiol"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"895","DOI":"10.1002\/ccd.23074","article-title":"Closure of perimembranous ventricular septal defects with aneurysmal tissue using the Amplazter Duct Occluder I: lessons learned and medium term follow up","volume":"80","author":"H G El Said","year":"2012","journal-title":"Catheter Cardiovasc Interv"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"e40","DOI":"10.1016\/j.ijcard.2006.10.023","article-title":"Transcatheter closure of a non-restrictive patent ductus arteriosus with an Amplatzer muscular ventricular septal defect occluder","volume":"117","author":"A Eicken","year":"2007","journal-title":"Int J Cardiol"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"2045894018776518","DOI":"10.1177\/2045894018776518","article-title":"Creation of a restrictive atrial communication in pulmonary arterial hypertension (PAH): effective palliation of syncope and end-stage heart failure","volume":"8","author":"A Bauer","year":"2018","journal-title":"Pulm Circ"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"1188","DOI":"10.1016\/j.cjca.2017.06.004","article-title":"Safety and Feasibility of the Transcatheter Approach to Create a Reverse Potts Shunt in Children With Idiopathic Pulmonary Arterial Hypertension","volume":"33","author":"Y Boudjemline","year":"2017","journal-title":"Can J Cardiol"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"101678","DOI":"10.1016\/j.jaccas.2022.08.050","article-title":"Transcatheter Valve Implantation in Reversed Potts Shunt in Pulmonary Arterial Hypertension: Keeping the Shunt Reversed","volume":"4","author":"G Goldar","year":"2022","journal-title":"JACC Case reports"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1053\/j.jvca.2019.06.015","article-title":"Anesthesia in Pediatric Patients With Congenital Heart Disease Undergoing Noncardiac Surgery: Defining the Risk","volume":"34","author":"M L Brown","year":"2020","journal-title":"J Cardiothorac Vasc Anesth"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1213\/01.ane.0000255732.16057.1c","article-title":"Perioperative complications in children with pulmonary hypertension undergoing noncardiac surgery or cardiac catheterization","volume":"104","author":"M J Carmosino","year":"2007","journal-title":"Anesth Analg"},{"volume-title":"Congenital Heart Disease in Adults","year":"2008","author":"J Perloff","key":"ref23"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"1302","DOI":"10.1183\/09031936.00089212","article-title":"Outcomes of noncardiac, nonobstetric surgery in patients with PAH: an international prospective survey","volume":"41","author":"S Meyer","year":"2013","journal-title":"Eur Respir J"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1097\/ALN.0b013e3181ae51a6","article-title":"Anesthesia for noncardiac surgery in adults with congenital heart disease","volume":"111","author":"M Cannesson","year":"2009","journal-title":"Anesthesiology"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"3826","DOI":"10.1093\/eurheartj\/ehac270","article-title":"2022 ESC Guidelines on cardiovascular assessment and management of patients undergoing non-cardiac surgery","volume":"43","author":"S Halvorsen","year":"2022","journal-title":"Eur Heart J"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"334","DOI":"10.1055\/a-0668-4653","article-title":"Pulmonale Hypertonie: an\u00e4sthesiologisches Management","volume":"54","author":"A H\u00f6tzel","year":"2019","journal-title":"An\u00e4sthesiol Intensivmed Notfallmed Schmerzther"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/j.ijcard.2018.08.078","article-title":"Pulmonary hypertension in adults with congenital heart disease: Updated recommendations from the Cologne Consensus Conference 2018","volume":"272","author":"H Kaemmerer","year":"2018","journal-title":"Int J Cardiol"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1016\/j.ijcard.2012.09.036","article-title":"Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease","volume":"168","author":"T Becker-Gr\u00fcnig","year":"2013","journal-title":"Int J Cardiol"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1186\/s12890-015-0031-1","article-title":"Exercise training improves physical fitness in patients with pulmonary arterial hypertension: a systematic review and meta-analysis of controlled trials","volume":"15","author":"R Buys","year":"2015","journal-title":"BMC Pulm Med"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1093\/eurheartj\/ehv337","article-title":"Exercise training improves peak oxygen consumption and haemodynamics in patients with severe pulmonary arterial hypertension and inoperable chronic thrombo-embolic pulmonary hypertension: a prospective, randomized, controlled trial","volume":"37","author":"N Ehlken","year":"2016","journal-title":"Eur Heart J"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"R148","DOI":"10.1186\/ar3883","article-title":"Exercise training in pulmonary arterial hypertension associated with connective tissue diseases","volume":"14","author":"E Gr\u00fcnig","year":"2012","journal-title":"Arthritis Res Ther"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"1032","DOI":"10.1161\/CIRCHEARTFAILURE.115.002130","article-title":"Efficacy and Safety of Exercise Training in Chronic Pulmonary Hypertension: Systematic Review and Meta-Analysis","volume":"8","author":"A Pandey","year":"2015","journal-title":"Circ Heart Fail"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"778","DOI":"10.1016\/j.rmed.2013.02.006","article-title":"Effect of aerobic exercise training on fatigue and physical activity in patients with pulmonary arterial hypertension","volume":"107","author":"A A Weinstein","year":"2013","journal-title":"Respir Med"},{"volume-title":"Pulmonary Arterial Hypertension in Congenital Heart Disease: Eisenmenger\u2019s Syndrome \u2013 A Global Perspective","year":"2013","author":"H Kaemmerer","key":"ref35"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1093\/eurheartj\/ehn597","article-title":"Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension?","volume":"30","author":"E B\u00e9dard","year":"2009","journal-title":"Eur Heart J"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/j.healun.2020.12.002","article-title":"Pregnancy in pulmonary arterial hypertension: Midterm outcomes of mothers and offspring","volume":"40","author":"J C Kamp","year":"2021","journal-title":"J Heart Lung Transplant"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1159\/000453062","article-title":"Sildenafil in Pregnancy: A Systematic Review of Maternal Tolerance and Obstetric and Perinatal Outcomes","volume":"41","author":"L Dunn","year":"2017","journal-title":"Fetal Diagn Ther"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1086\/682230","article-title":"Statement on pregnancy in pulmonary hypertension from the Pulmonary Vascular Research Institute","volume":"5","author":"A R Hemnes","year":"2015","journal-title":"Pulm Circ"},{"key":"ref40","doi-asserted-by":"crossref","first-page":"1546","DOI":"10.1093\/eurheartj\/ehx720","article-title":"Clinical recommendations for high altitude exposure of individuals with pre-existing cardiovascular conditions: A joint statement by the European Society of Cardiology, the Council on Hypertension of the European Society of Cardiology, the European Society of Hypertension, the International Society of Mountain Medicine, the Italian Society of Hypertension and the Italian Society of Mountain Medicine","volume":"39","author":"G Parati","year":"2018","journal-title":"Eur Heart J"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"3618","DOI":"10.1093\/eurheartj\/ehac237","article-title":"2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"43","author":"M Humbert","year":"2022","journal-title":"Eur Heart J"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"1122","DOI":"10.21037\/cdt-20-494","article-title":"Altitude exposure in pediatric pulmonary hypertension \u2013 are we ready for (flight) recommendations?","volume":"11","author":"U Herberg","year":"2021","journal-title":"Cardiovasc Diagn Ther"},{"key":"ref43","doi-asserted-by":"crossref","first-page":"1682","DOI":"10.1164\/ajrccm.164.9.2106076","article-title":"Nocturnal oxygen therapy in patients with the Eisenmenger syndrome","volume":"164","author":"J Sandoval","year":"2001","journal-title":"Am J Respir Crit Care Med"},{"key":"ref44","first-page":"e74","article-title":"Iron deficiency in a patient with extreme erythrocytosis due to cyanotic congenital heart disease","volume":"117","author":"S Braun","year":"2006","journal-title":"Int J Cardiol"},{"volume-title":"Das Eisenmenger-Syndrom \u2013 vom Symptom zur Diagnose, 1. Aufl","year":"2011","author":"H Kaemmerer","key":"ref45"},{"volume-title":"Pulmonary Arterial Hypertension in Congenital Heart Disease: Eisenmenger\u02bcs Syndrome \u2013 A Global Perspective","year":"2013","author":"H Kaemmerer","key":"ref46"},{"key":"ref47","first-page":"292","article-title":"Klinische Aspekte und Therapieoptionen bei Angeborenen Herzfehlern mit pulmonal-arterieller Hypertonie","volume":"64","author":"H Kaemmerer","year":"2013","journal-title":"Med Welt"},{"key":"ref48","doi-asserted-by":"crossref","first-page":"343","DOI":"10.2174\/157340310793566154","article-title":"The adult patient with eisenmenger syndrome: a medical update after dana point part I: epidemiology, clinical aspects and diagnostic options","volume":"6","author":"H Kaemmerer","year":"2010","journal-title":"Curr Cardiol Rev"},{"key":"ref49","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1016\/j.amjcard.2006.01.083","article-title":"Pathogenesis of thrombocytopenia in cyanotic congenital heart disease","volume":"98","author":"M C Lill","year":"2006","journal-title":"Am J Cardiol"},{"key":"ref50","doi-asserted-by":"crossref","first-page":"363","DOI":"10.2174\/157340310793566127","article-title":"The Adult Patient with Eisenmenger Syndrome: A Medical Update after Dana Point Part III: Specific Management and Surgical Aspects","volume":"6","author":"E Oechslin","year":"2010","journal-title":"Curr Cardiol Rev"},{"key":"ref51","doi-asserted-by":"crossref","first-page":"406","DOI":"10.7326\/0003-4819-109-5-406","article-title":"Adults with cyanotic congenital heart disease: hematologic management","volume":"109","author":"J K Perloff","year":"1988","journal-title":"Annals of internal medicine"},{"key":"ref52","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/S0022-3476(75)80073-4","article-title":"Shortened platelet survival in cyanotic heart disease","volume":"87","author":"J D Waldman","year":"1975","journal-title":"The Journal of pediatrics"},{"key":"ref53","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-319-46028-4","volume-title":"Pulmonary Hypertension in Adult Congenital Heart Disease","author":"K Dimopoulos","year":"2017"},{"key":"ref54","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.ijcard.2018.08.080","article-title":"Pulmonary hypertension associated with left heart disease: Updated Recommendations of the Cologne Consensus Conference 2018","volume":"272","author":"S Rosenkranz","year":"2018","journal-title":"International Journal of Cardiology"},{"key":"ref55","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1161\/CIRCULATIONAHA.106.630715","article-title":"Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study","volume":"114","author":"N Gali\u00e8","year":"2006","journal-title":"Circulation"},{"key":"ref56","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1161\/CIRCULATIONAHA.118.033575","article-title":"Evaluation of Macitentan in Patients With Eisenmenger Syndrome","volume":"139","author":"M A Gatzoulis","year":"2019","journal-title":"Circulation"},{"key":"ref57","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/j.ijcard.2019.07.004","article-title":"A single-centre, placebo-controlled, double-blind randomised cross-over study of nebulised iloprost in patients with Eisenmenger syndrome: A pilot study","volume":"299","author":"H Nashat","year":"2020","journal-title":"Int J Cardiol"},{"key":"ref58","doi-asserted-by":"crossref","first-page":"1124","DOI":"10.1093\/eurheartj\/ehq011","article-title":"Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial","volume":"31","author":"K Iversen","year":"2010","journal-title":"Eur Heart J"},{"key":"ref59","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1016\/j.ijcard.2010.10.051","article-title":"Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology","volume":"155","author":"M D'Alto","year":"2012","journal-title":"Int J Cardiol"},{"key":"ref60","doi-asserted-by":"crossref","first-page":"2201347","DOI":"10.1183\/13993003.01347-2022","article-title":"Sotatercept for the treatment of pulmonary arterial hypertension: PULSAR open-label extension","volume":"61","author":"M Humbert","year":"2023","journal-title":"Eur Respir J"},{"key":"ref61","doi-asserted-by":"crossref","first-page":"1478","DOI":"10.1056\/NEJMoa2213558","article-title":"Phase 3 Trial of Sotatercept for Treatment of Pulmonary Arterial Hypertension","volume":"388","author":"M M Hoeper","year":"2023","journal-title":"N Engl J Med"},{"key":"ref63","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1056\/NEJMoa1213917","article-title":"Macitentan and morbidity and mortality in pulmonary arterial hypertension","volume":"369","author":"T Pulido","year":"2013","journal-title":"N Engl J Med"},{"key":"ref64","doi-asserted-by":"crossref","first-page":"2522","DOI":"10.1056\/NEJMoa1503184","article-title":"Selexipag for the Treatment of Pulmonary Arterial Hypertension","volume":"373","author":"O Sitbon","year":"2015","journal-title":"N Engl J Med"},{"key":"ref65","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1002\/ejhf.2706","article-title":"Exercise-induced changes in pulmonary artery wedge pressure in adults post-Fontan versus heart failure with preserved ejection fraction and non-cardiac dyspnoea","volume":"25","author":"W R Miranda","year":"2023","journal-title":"Eur J Heart Fail"},{"key":"ref66","doi-asserted-by":"crossref","first-page":"1015","DOI":"10.1016\/j.healun.2019.08.003","article-title":"The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Twenty-second pediatric lung and heart-lung transplantation report-2019; Focus theme: Donor and recipient size match","volume":"38","author":"D Hayes Jr","year":"2019","journal-title":"J Heart Lung Transplant"},{"key":"ref67","first-page":"596","article-title":"Heart or heart-lung transplantation for patients with congenital heart disease in England","volume":"105","author":"K Dimopoulos","year":"2019","journal-title":"Heart (British Cardiac Society)"},{"key":"ref68","doi-asserted-by":"crossref","first-page":"e090522204486","DOI":"10.2174\/1573403X18666220509203335","article-title":"Reversed Potts Shunt Outcome in Suprasystemic Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis","volume":"18","author":"B Mendel","year":"2022","journal-title":"Curr Cardiol Rev"},{"key":"ref69","doi-asserted-by":"crossref","first-page":"e14289","DOI":"10.1111\/petr.14289","article-title":"Extracorporeal membrane oxygenation and paracorporeal lung assist devices as a bridge to pediatric lung transplantation","volume":"26","author":"L E Chiel","year":"2022","journal-title":"Pediatr Transplant"},{"key":"ref70","doi-asserted-by":"crossref","first-page":"2102311","DOI":"10.1183\/13993003.02311-2021","article-title":"COMPERA 2.0: a refined four-stratum risk assessment model for pulmonary arterial hypertension","volume":"60","author":"M M Hoeper","year":"2022","journal-title":"Eur Respir J"},{"key":"ref71","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1016\/j.ijcard.2015.10.081","article-title":"Determinants of outpatient clinic attendance amongst adults with congenital heart disease and outcome","volume":"203","author":"A Kempny","year":"2016","journal-title":"Int J Cardiol"}],"container-title":["Pneumologie"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2274-1025.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,12,17]],"date-time":"2024-12-17T13:12:52Z","timestamp":1734441172000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2274-1025"}},"subtitle":["Part II: Supportive Therapie, Sondersituationen (Schwangerschaft, Kontrazeption, nicht-kardiale Operationen), zielgerichtete Pharmakotherapie, Organtransplantation, spezielles Management (Shuntvitien, Linksherzerkrankungen, univentrikul\u00e4re Herzen), Interventionen, Intensivmedizin, Nachsorge, Zukunftsperspektive"],"short-title":[],"issued":{"date-parts":[[2024,5,24]]},"references-count":70,"journal-issue":{"issue":"08","published-online":{"date-parts":[[2024,8,19]]},"published-print":{"date-parts":[[2024,8]]}},"URL":"https:\/\/doi.org\/10.1055\/a-2274-1025","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0934-8387","1438-8790"],"issn-type":[{"type":"print","value":"0934-8387"},{"type":"electronic","value":"1438-8790"}],"subject":[],"published":{"date-parts":[[2024,5,24]]}}}