Disease targeting therapies in patients with Eisenmenger syndrome: response to treatment and long-term efficiency

Int J Cardiol. 2013 Aug 10;167(3):840-7. doi: 10.1016/j.ijcard.2012.02.007. Epub 2012 Mar 3.


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.

Publication types

  • Multicenter Study
  • Randomized Controlled Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Angiotensin-Converting Enzyme Inhibitors / administration & dosage
  • Antihypertensive Agents / administration & dosage
  • Double-Blind Method
  • Drug Delivery Systems / methods*
  • Eisenmenger Complex / drug therapy*
  • Eisenmenger Complex / physiopathology*
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Retrospective Studies
  • Time Factors
  • Treatment Outcome
  • Young Adult


  • Angiotensin-Converting Enzyme Inhibitors
  • Antihypertensive Agents