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2016 ; 37
(18
): 1449-55
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Current therapy and outcome of Eisenmenger syndrome: data of the German National
Register for congenital heart defects
#MMPMID26843280
Diller GP
; Körten MA
; Bauer UM
; Miera O
; Tutarel O
; Kaemmerer H
; Berger F
; Baumgartner H
Eur Heart J
2016[May]; 37
(18
): 1449-55
PMID26843280
show ga
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% ?-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.