Exercise CMR in Eisenmenger-syndrome
Research type
Research Study
Full title
Effect of pulmonary vasodilator therapy and exercise training on right ventricular function and pulmonary haemodynamics during exercise as assessed by magnetic resonance imaging in patients with Eisenmenger syndrome
IRAS ID
181525
Contact name
Mark C K Hamilton
Contact email
Sponsor organisation
Research and lnnovation, University Hospitals Bristol NHS Foundation Trust
Duration of Study in the UK
1 years, 1 months, 28 days
Research summary
Patients with adult congenital heart disease (CHD) frequently have pulmonary arterial hypertension (PAH). As a result of a congenital cardiac defect causing a left to right shunt in childhood, there is eventually an elevated pulmonary vascular resistance that leads to an irreversible right-to-left shunt and Eisenmenger Syndrome (ES).
Current therapy is aimed at palliation of such PAH, which depends on accurate diagnosis and assessment of severity. Investigations such as echocardiography and cardiac catheterisation have improved the diagnosis but suffer from limitations. Most guidelines provide only resting reference values because of the technical difficulties with obtaining exercise-related data.
The pathophysiological mechanisms of such altered pulmonary haemodynamics are not fully understood, particularly the changes that occur during exercise. The exercise-induced response of cardio-pulmonary haemodynamics in patients with ACHD-PAH has been investigated in few studies and has never been assessed in ES with real-time volume and flow quantification sequences. Following our work with this technique on healthy controls, we propose to investigate cardiac and pulmonary haemodynamic function in ES at rest and on exercise.
We also wish to determine the response to short-term (oxygen) and long-term (sildenafil or endothelin receptor antagonist) pulmonary vasodilator therapy by performing real time image acquisition using cardiac magnetic resonance imaging at rest and with use of a supine ergometer. Some patients will be embarking on an exercise rehabilitation programme and we will study them after such a programme to determine the responses of improved cardio-pulmonary efficiency.
We hypothesise that there will be exercise-related increase in pulmonary blood flow with acute oxygen, chronic disease modifying therapy or exercise training. This may be due to a combination of factors: reduced pulmonary vascular resistance, increased cardiac output, cardiac remodelling and improved cardio-pulmonary interactions. We also expect that such interventions will allow increased exercise activity.REC name
Wales REC 3
REC reference
16/WA/0013
Date of REC Opinion
10 Feb 2016
REC opinion
Further Information Favourable Opinion