title: Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects.
abstract: We aimed to assess the contemporary outcome of Eisenmenger syndrome (ES), delineate the use of disease targeting therapies (DTT) in these patients and to investigate the effect of treatment on outcome in the community.
  
  
  
  Patients with ES were systematically identified from the German National Register for Congenital Heart Defects. Data on underlying diagnosis, medical therapy, and survival were collected. The impact of DTT on survival was assessed using time-dependant Cox analysis. Overall, 153 ES patients were included (mean age 34.0 ± 13.3 years, 46% females). Of these, 88 (57.5%) were treated with at least one DTT (76.1% Bosentan, 20.5% Sildenafil) while 17.6% were on dual DTT. In addition, 24.8% of patients received digoxin, 10.5% angiotensin-converting enzyme-inhibitors/angiotensin receptor blockers, and 17.6% β-blockers. Moreover, 17.6% of patients were treated with oral anticoagulants, while 23.5% of patients received Aspirin. The survival rate at 1, 5, and 10 years of follow-up was only 92, 75, and 57% in the entire cohort, and was even worse in treatment naive ES patients (survival rate 86, 60, and 34% at 1, 5, and 10 years). Use of DTT was independently associated with a better survival (hazard ratio 0.42, P= 0.015).
  
  
  
  This study illustrates the alarmingly poor survival prospects of Eisenmenger patients by community-based data even in the current era with advanced DTT and in a country with a wealthy health system. Treatment naive ES patients had especially high mortality rates approaching 60-70% at 10 years of follow-up. Treatment with DTT was associated with better survival.
authors: Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Albert-Schweitzer-Str. 33, Muenster 48149, Germany Competence Network for Congenital Heart Defects, Germany gerhard.diller@ukmuenster.de.
authors: Gerhard-Paul
authors: GP
authors: Diller
authors: National Register for Congenital Heart Defects, Berlin, Germany.
authors: Marc-André
authors: MA
authors: Körten
authors: Competence Network for Congenital Heart Defects, Germany National Register for Congenital Heart Defects, Berlin, Germany.
authors: Ulrike M M
authors: UM
authors: Bauer
authors: Department of Congenital Heart Disease and Pediatric Cardiology, Deutsches Herzzentrum Berlin, Berlin, Germany.
authors: Oliver
authors: O
authors: Miera
authors: Competence Network for Congenital Heart Defects, Germany Department of Cardiology & Angiology, Hannover Medical School, Hannover, Germany.
authors: Oktay
authors: O
authors: Tutarel
authors: Competence Network for Congenital Heart Defects, Germany Department of Pediatric Cardiology and Congenital Heart Disease, The German Heart Centre, Technical University Munich, Munich, Germany.
authors: Harald
authors: H
authors: Kaemmerer
authors: Competence Network for Congenital Heart Defects, Germany Department of Congenital Heart Disease and Pediatric Cardiology, Deutsches Herzzentrum Berlin, Berlin, Germany.
authors: Felix
authors: F
authors: Berger
authors: Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Albert-Schweitzer-Str. 33, Muenster 48149, Germany Competence Network for Congenital Heart Defects, Germany.
authors: Helmut
authors: H
authors: Baumgartner
mesh_terms: D000328:Adult; D000806:Angiotensin-Converting Enzyme Inhibitors; D000925:Anticoagulants; D004541:Eisenmenger Complex; D005260:Female; D006801:Humans; D008297:Male; D000068677:Sildenafil Citrate
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D000925:Anticoagulants; D000068677:Sildenafil Citrate
keywords: Adult congenital heart disease; Advanced therapy; Eisenmenger syndrome; Registry