- 作者：Gerhard-Paul Diller ; Rafael Alonso-Gonzalez ; Konstantinos Dimopoulos ; Maria Alvarez-Barredo ; Chiehyang Koo ; Aleks ; er Kempny ; Carl Harries ; Lisa Parfitt ; Anselm S. Uebing ; Lorna Swan ; Philip S. Marino ; Stephen J. Wort ; Michael A. Gatzoulis
- 关键词：Pulmonary arterial hypertension ; Eisenmenger syndrome ; Therapy ; 6-minute walk test distance ; Functional class
- 刊名：International Journal of Cardiology
- 出版年：2013
- 出版时间：10 August, 2013
- 年：2013
- 卷：167
- 期：3
- 页码：840-847
- 全文大小：835 K
Objectives
To examine long-term efficacy of disease targeting therapies (DTT) in patients with Eisenmenger syndrome.Methods
All adult patients with Eisenmenger syndrome treated with DTT at our institution were included. Functional class (FC), oxygen saturation and 6-minute walk test distance (6MWTd) were analysed retrospectively.
Results
Between 2002 and 2010, 79 Eisenmenger patients (21 males, 16 with Down syndrome) aged 34 ¡À 10 years (range 17-68 years) were included. Median follow-up was 3.3 years (range 0.2 to 8.9 years). 6MWTd increased early after initiation of DTT, with a plateau after approximately 3 years and no obvious trend towards a deterioration on average during longer-term follow-up. Two patients died during follow-up and escalation of treatment was required in 18 patients after a median period of 2.5 years. Escalation of therapy was also associated with an increase in 6MWTd. In addition, FC improved on DTT and oxygen saturation, increased, both at rest and peak exercise. This effect was more pronounced in the patients with the lowest baseline oxygen saturation at rest.
Conclusions
Long-term DTT is safe and improves objective exercise capacity and subjective symptoms. Response to DTT was typically observed early after initiation of DTT and was, on average, maintained longer-term. However, 1 in 5 patients required escalation of DTT, with time, due to symptomatic deterioration and this was associated with an afresh improvement in 6MWTd.