European Heart Journal Advance Access originally published online on May 17, 2006
European Heart Journal 2006 27(12):1472-1477; doi:10.1093/eurheartj/ehl017
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Advanced therapy may delay the need for transplantation in patients with the Eisenmenger syndrome
1 Department of Cardiology, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium
2 Department of Pneumology, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium
Received 2 December 2005; revised 10 March 2006; accepted 13 April 2006; online publish-ahead-of-print 17 May 2006.
* Corresponding author. Tel: +32 16 344235; fax: +32 16 344240. E-mail address: werner.budts{at}uz.kuleuven.ac.be
Aims Advanced therapies (prostacyclin analogues, endothelin receptor antagonists) are successfully used in the treatment of idiopathic pulmonary arterial hypertension. In addition, patients with the Eisenmenger syndrome (ES) seem to benefit from these news drugs regarding symptoms, but there is still no evidence for changes in outcome.
Methods and results The clinical course of 43 patients (M/F 13/30, age 34.0±12.7 years), registered with unstable ES in our database, was retrospectively analysed. These patients were divided into two groups: those treated with and those treated without advanced therapy. The primary endpoint was defined as death from any cause. Death or inscription on the active waiting list of heart–lung transplantation was considered as secondary endpoint. Kaplan–Meier survival and log rank testing were performed to determine differences in outcome between the two groups. The total cohort was followed for a median period of 4.9 (range 0.2–14.9) years. Mean survival time for patients treated with (n=26) and without (n=17) advanced therapy therapies were 8.5±1.5 and 8.5±0.9 years, respectively (log rank testing, P=0.31). However, the mean time to death or inscription on the active waiting list was significantly longer for patients treated with advanced therapy when compared with those without (7.8±1.0 vs. 3.4±0.9 years, P=0.006).
Conclusion For the given follow-up period, no improvement in survival time could be documented in adult patients with unstable ES treated with advanced therapy. However, we might suggest with these data that the need for heart–lung transplantation can be substantially delayed with new drugs.
Key Words: Eisenmenger syndrome • Pulmonary arterial hypertension • Prostacyclin analogues • Endothelin receptor antagonists
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