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Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects.

Abstract：

AIMS: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. METHODS AND RESULTS: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). CONCLUSION: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.

journal_name

Eur Heart J

journal_title

European heart journal

authors

Diller GP,Körten MA,Bauer UM,Miera O,Tutarel O,Kaemmerer H,Berger F,Baumgartner H,German Competence Network for Congenital Heart Defects Investigators.

doi

10.1093/eurheartj/ehv743

subject

Has Abstract

pub_date

2016-05-07 00:00:00

pages

1449-55

issue

18

eissn

0195-668X

issn

1522-9645

pii

ehv743

journal_volume

37

pub_type

杂志文章

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