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 syndro...
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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.
Aims: To characterize contemporary Eisenmenger patients at a large centre for adult congenital heart disease, assess survival prospects, and identify predictors of death in this population. Methods and results: All Ei...
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Aims: To characterize contemporary Eisenmenger patients at a large centre for adult congenital heart disease, assess survival prospects, and identify predictors of death in this population. Methods and results: All Eisenmenger patients under follow-up at our centre since 2000(n=171, mean age 37±12 years) were included. To identify predictors of mortality, a case-control study was performed. Data including symptoms, functional class, medication, laboratory, and electrocardiographic and echocardiographic parameters are presented. Iron deficiency was common and strongly related to phlebotomy(relative risk 4.1, P< 0.0001). Haemoglobin concentration was inversely related to arterial oxygen saturations in iron-replete patients(P< 0.001) but not in iron-deficient patients. During a median follow-up of 67 months,20 patients died. Survival at 40, 50, and 60 years of age was 94, 74, and 52%, respectively. When compared with healthy individuals,median survival was reduced by ～20 years in Eisenmenger patients and was worst in those with complex lesions. Predictors of mortality included functional class, signs of heart failure, history of clinical arrhythmia, QRS duration and QTc interval, and low serum albumin and potassium levels. Conclusion: Despite good short-term prognosis, life expectancy is markedly reduced in Eisenmenger patients. Markers of heart failure and parameters associated with arrhythmia are of prognostic value in terms of mortality and may guide clinicians caring for Eisenmenger patients.
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