Advanced therapy in patients with unstable Eisenmenger syndrome did not improve mean survival time (8.5 vs 8.5 years; P=0.31) but significantly delayed time to death or transplant listing (P=0.006).
Cohort (n=43)
Does advanced therapy (prostacyclin analogues, endothelin receptor antagonists) reduce death or delay the need for heart-lung transplantation in patients with unstable Eisenmenger syndrome?
In patients with unstable Eisenmenger syndrome, advanced therapies (prostacyclin analogues, endothelin receptor antagonists) significantly delayed the composite of death or need for heart-lung transplantation, though overall survival was not significantly improved.
p-value: p=0.31
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.
Adriaenssens et al. (Wed,) conducted a cohort in Eisenmenger syndrome (n=43). Advanced therapies (prostacyclin analogues, endothelin receptor antagonists) vs. Without advanced therapy was evaluated on Death from any cause (p=0.31). Advanced therapy in patients with unstable Eisenmenger syndrome did not improve mean survival time (8.5 vs 8.5 years; P=0.31) but significantly delayed time to death or transplant listing (P=0.006).
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