Article Text
Abstract
Objective To determine long-term objective and Patient Reported Outcomes at a single centre in patients with CTEPH (distal or residual post Pulmonary endarterectomy).
Method A retrospective study of all incident patients with CTEPH (2000–2011). Data obtained from the service database—6-minute walk test (6MWT), CAMbridge Pulmonary Hypertension Outcome Review (CAMPHOR) symptom, activity and Quality of Life (QoL) scores, and NTproBNP. Results obtained at baseline (at the time of diagnosis, pre-commencement of first targeted therapy), 3 months, 1, 2, 3, and 4 years follow-up. Over this period were managed according to clinical need and NCG guidelines. N=124. Targeted therapy defined as Prostacyclin, endothelin receptor antagonists, and phosphodiesterase 5 inhibitors.
Results Changes at 1st and 2nd time points (median 3 months and 1 year) show statistically significant improvements in patient reported outcomes and objective measures. The NTproBNP improvement peaks at 3 years and begins to tail off at 4 years. The 6MWD improvement plateaus out between 2 and 3 years. The CAMPHOR symptom score shows significant improvement including year 3. The 4-year 6MWD does not achieve statistical significance compared to baseline. Further investigation revealed that between the third and fourth year 22 subject's follow-up was not performed; 3 died, 1 transferred to another centre, 18 are awaiting their 4-year follow-up.
Conclusion Objective and patient reported measures of change over time show improvements for CTEPH patients up to 3 years. After 3 years it becomes more difficult to determine a categorical change. More 6MW and CAMPHOR data for the 4 year time point is needed to determine if initial improvements are sustained after 3 years in this patient group, or if the improvement seen really has begun to tail off as the results here may suggest. The 4-year follow-up data for the remaining 18 patients will be available later this year.