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 (6 MWTd) were analysed retrospectively.
Results: Between 2002 and 2010, 79 Eisenmenger patients (21 males, 16 with Down syndrome) aged 34 ± 10 years (range 17-68 years) were included. Median follow-up was 3.3 years (range 0.2 to 8.9 years). 6 MWTd 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 6 MWTd. 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 6 MWTd.
Keywords: 6-minute walk test distance; Eisenmenger syndrome; Functional class; Pulmonary arterial hypertension; Therapy.
Copyright © 2012. Published by Elsevier Ireland Ltd.