Abstract 10084: Significant Improvements in Patient Reported Outcomes in Eisenmenger Syndrome with Advanced Pulmonary Hypertension Therapies and Association with Objective Improvements in Exercise Tolerance
Introduction Eisenmenger syndrome patients have a high symptomatic burden with decreased functional ability, right sided heart failure and early death. Treatment is aimed at improving symptoms and quality of life (QOL). There is limited data to advocate the prognostic benefit of treatment. CAMPHOR (Cambridge Pulmonary Hypertension Outcome Review) is a patient questionnaire specific to pulmonary artery hypertension (PAH). It assesses the impact of PAH on patients' symptoms, activity and QOL and is a validated outcome measure.
Hypotheses We hypothesised that Eisenmenger syndrome patients' 6 minute walk test (6MWT) was associated with their CAMPHOR scores and that initiation of advanced therapy with bosentan resulted in improvement in their CAMPHOR scores and 6MWT.
Methods All patients in a tertiary centre diagnosed with Eisenmenger syndrome on treatment with bosentan completed the CAMPHOR questionnaire provided they had capacity. This was performed prior to initiation of bosentan and repeated at each routine follow up assessment from 2008-2011. This generated a score for symptoms (0-25), activity (0-30) and QOL (0-25) at each appointment. A score of 0 indicated the lowest morbidity. A 6MWT was performed at each assessment. Initial and follow up assessment results were compared using paired t-test. Association between first 6MWT and symptoms, activity and QOL was assessed using linear regression.
Results 24 of 35 patients studied (69%) were female. The median (IQR) age was 41 (30-59). 15 patients completed the CAMPHOR questionnaire pre and post treatment with bosentan. There were significant improvements in symptoms, activity, QOL scores and 6MWT distances between pre treatment and 3-6 months after treatment with bosentan, the respective values are as follows: 12±4, 8±5 (p=0.005); 11±5, 8±4 (p=0.0135); 11±5, 7±5 (p=0.0183); 302±120, 333 ±107m (p=0.0199). Higher 6MWT distance was significantly associated with better activity scores (R2=0.2 (p=0.009)) at first assessment.
Conclusion In our study of patients with Eisenmenger syndrome, treatment with bosentan resulted in significant improvements in the patient reported parameters. There was a significant association between exercise tolerance distance and patient reported activity scores.
- Adult congenital heart disease
- Congenital heart disease
- Physical activity
- Pulmonary hypertension
- Quality of life
- © 2011 by American Heart Association, Inc.