Long-term Survival and Quality of Life

BackgroundChronic thromboembolic pulmonary hypertension (CTEPH) causes significant morbidity and mortality, but long-term outcomes from contemporary multicenter studies are lacking.Research QuestionHow are survival and health-related quality of life characterized in patients with CTEPH who are classified as inoperable, who are operable but have not undergone surgery, and who have undergone surgery including pulmonary thromboendarterectomy?Study Design and MethodsPatients with CTEPH recruited from 30 US sites from 2015 through 2018 completed the 36-item Short Form Health Survey (SF-36) and EmPHasis-10 survey at baseline and 6-month intervals. Mixed model repeated measures analysis was used to compare between-group differences in score up to 2 years vs baseline. Multivariable Cox proportional hazards models were used to analyze survival by CTEPH group.ResultsSeven hundred fifty patients with a median age of 59 years were enrolled; 566 patients, 88 patients, and 96 patients were in the operated, operable but no surgery, and inoperable groups, respectively. Survival at 1, 2, and 3 years was 93%, 91%, and 87%, respectively. Patients in the inoperable and the operable but no surgery groups showed higher mortality rates relative to the operated group (hazard ratios, 2.10 95% CI, 1.17-3.77 and 2.19 95% CI, 1.20-3.99, respectively). The EmPHasis-10 and both SF-36 scores improved during follow-up, with larger increases for the operated group (P 15 mm Hg if justified by the investigator) and showed evidence of CTEPH on imaging that was reviewed for eligibility by an adjudication team. Patients were classified by the enrolling center as having operable or inoperable CTEPH, and if inoperable, the reasons for this classification. Details have been published previously.8Kerr K.M. Elliott C.G. Chin K. et al.Results from the United States Chronic Thromboembolic Pulmonary Hypertension Registry: enrollment characteristics and 1-year follow-up.Chest. 2021; 160: 1822-1831Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar Patients were subdivided further into three groups: operated (patients undergoing PTE surgery during the study period), operable but no surgery (patients classified as operable who did not undergo surgery), and inoperable. Patients completed the Rand version of the 36-item Short-Form Health Survey (SF-36) and EmPHasis-10 at baseline and at 6-month intervals after enrolment. The SF-36 is a 36-item patient-reported survey of general health that has had good reliability and validity across many patient populations and settings.10Frendl D.M. Ware Jr., J.E. Patient-reported functional health and well-being outcomes with drug therapy: a systematic review of randomized trials using the SF-36 health survey.Med Care. 2014; 52: 439-445Crossref PubMed Scopus (78) Google Scholar, 11Mathai S.C. Suber T. Khair R.M. Kolb T.M. Damico R.L. Hassoun P.M. Health-related quality of life and survival in pulmonary arterial hypertension.Ann Am Thorac Soc. 2016; 13: 31-39Crossref PubMed Scopus (53) Google Scholar, 12Sanders J. Bowden T. Woolfe-Loftus N. Sekhon M. Aitken L.M. Predictors of health-related quality of life after cardiac surgery: a systematic review.Health Qual Life Outcomes. 2022; 20: 79Crossref Scopus (5) Google Scholar The EmPHasis-10 is a 10-item disease-specific questionnaire developed to assess symptoms of PH; it has good internal and test-test reliability and was developed and validated in patients with pulmonary arterial hypertension and CTEPH.13Yorke J. Corris P. Gaine S. et al.emPHasis-10: development of a health-related quality of life measure in pulmonary hypertension.Eur Respir J. 2013; Google Scholar Scores for the SF-36 physical component score (PCS) and mental component score (MCS) were calculated using standard methodology with a potential range from to the general is for with higher scores to health-related quality of Scores for the EmPHasis-10 range from to with scores to analysis was for and were for and a was for the three groups, a was for and a for the group were (P 30 right cardiac and pulmonary vascular unadjusted Cox proportional hazards model with group in the model (operated group as the was completed with three model adjusted for and model adjusted for of right and cardiac on in the with survival in with and arterial of with and inclusion of all that were significant (P 30 inoperable vs operated inoperable vs operated < was inoperable vs operated < was operable no surgery vs operated < was of or of M. Lang I. Pepke-Zaba J. et al.Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry.Circulation. 2016; 133: 859-871Crossref PubMed Scopus (424) Google mm inoperable vs operated < was mm used for cardiac and cardiac operable no surgery vs operated < was are as or median functional pulmonary pulmonary wedge pulmonary vascular right Health inoperable vs operated < was operable no surgery vs operated < was used for cardiac and cardiac in a are as or median functional pulmonary pulmonary wedge pulmonary vascular right Health a median of 25 months of follow-up, patients to PTE surgery or reasons for PH as symptoms or right heart or The was in the PTE group at CI, vs CI, and CI, in the inoperable and operable but no surgery groups, respectively. In the cohort and survival at 1, 2, and 3 years was 93%, 91%, and 87%, this includes in the operated group with survival Health functional and and including arterial and more including higher right and pulmonary vascular of Time to functional for functional is and arterial pulmonary arterial pulmonary vascular right Health for functional is and in a arterial pulmonary arterial pulmonary vascular right Health Survival by CTEPH group is in 2 and the survival and characteristics of this cohort vs multicenter with CTEPH. and survival was 93%, and (operated and (inoperable and 91%, and (operable but no surgery group), respectively. Patients in the inoperable and the operable but no surgery groups showed higher mortality relative to the operable group on Cox proportional hazards models (hazard ratios, 95% CI, 1.17-3.77 and 2.19 [95% CI, respectively). were in with that or of Time to but no for and but no for and but no for significant in the but no of right and cardiac significant in the analysis were functional right pulmonary arterial and pulmonary vascular in a of right and cardiac significant in the analysis were functional right pulmonary arterial and pulmonary vascular Survival by median age and are in analyses were not for these subgroups the but outcomes generally for vs patients, whereas mortality higher for patients than the median age with those than the median and follow-up scores for the EmPHasis-10 and SF-36 are in In the cohort the SF-36 was than the SF-36 at with a of on the SF-36 and on the SF-36 The baseline EmPHasis-10 score was 6 improved across all three groups vs baseline inoperable, and operable but no vs baseline was larger in the operated with results after by median age 2, In a analysis the of patients meeting or the for at 6 and a larger of patients this in the operated group vs the inoperable and operable but no surgery groups In the models including both unadjusted results and results adjusted for and the score from baseline score also was for the operated group relative to both the operable but no surgery and inoperable groups at time points for scores by from baseline scores by and unadjusted and adjusted model results The US CTEPH Registry was to provide and outcomes of a cohort of patients with CTEPH and patients with CTEPH from 30 in the United characteristics were K.M. Elliott C.G. Chin K. et al.Results from the United States Chronic Thromboembolic Pulmonary Hypertension Registry: enrollment characteristics and 1-year follow-up.Chest. 2021; 160: 1822-1831Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar; the long-term survival results as as and quality-of-life in the current study higher survival for patients with CTEPH who underwent PTE vs the inoperable group and vs the operable but no surgery group and in health-related quality-of-life patients with CTEPH who underwent PTE vs changes in patients in the inoperable and operable but no surgery The a disease-specific and the a health-related quality-of-life were used in this study to assess symptoms and health-related quality of have to in some by symptoms and that are whereas provide a for both and of that patients with CTEPH in the US CTEPH Registry a baseline on EmPHasis-10 scores and significant in baseline health on SF-36 and SF-36 longitudinal follow-up, scores on both improved for all three CTEPH but the improvements were in patients who underwent of the current study is the inclusion of patients treated both and CTEPH studies that have on a single and after V. health-related quality of life after surgery in patients with chronic thromboembolic pulmonary Life Scopus Google Scholar, M. K. N. et patient-reported outcomes are improved by pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension.Eur Respir J. Scopus Google Scholar, J. S. L. et of the and SF-36 in pulmonary Med. 2013; 13: PubMed Scopus Google Scholar The further into inoperable and operable but no surgery groups vs inclusion of a single no surgery group is an potential when patients who underwent surgery with those who did patients who undergo surgery to with of the from this study, is that in symptoms and quality of life to for patients with CTEPH undergoing PTE surgery vs both the inoperable and operable but no surgery The in SF-36 and EmPHasis-10 results also to for this with patients showed that or the differences for S.C. The in the for patients with pulmonary arterial hypertension.Am J Respir Crit Care Med. 2012; PubMed Scopus Google M. T. et as a measure of health-related quality of life in pulmonary arterial hypertension: data from Respir J. 2021; Scopus Google Scholar Patients in the operated group showed a of mortality with those in the inoperable and operable but no surgery groups, and this significant in of the with the quality-of-life results that patients to for PTE surgery as the cohort studies also have long-term survival for patients with CTEPH undergoing PTE surgery vs S.R. Swift A.J. Billings C.G. et al.The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension.Eur Respir J. 2018; 52Crossref PubMed Scopus (60) Google M. Lang I. Pepke-Zaba J. et al.Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry.Circulation. 2016; 133: 859-871Crossref PubMed Scopus (424) Google R. Kiely D.G. Gibbs J.S. et al.Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension.Am J Respir Crit Care Med. 2008; 177: 1122-1127Crossref PubMed Scopus (339) Google Scholar and vs R.L. J. of for pulmonary arterial hypertension: Full Text Full Text PDF PubMed Scopus Google Scholar the current study, these were and were to potential or a mortality also was after for potential in the by et M. Lang I. Pepke-Zaba J. et al.Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry.Circulation. 2016; 133: 859-871Crossref PubMed Scopus (424) Google Scholar in a study by et S.R. Swift A.J. Billings C.G. et al.The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension.Eur Respir J. 2018; 52Crossref PubMed Scopus (60) Google Scholar from the United patients surgery choice than no surgery of also showed survival rates vs operated In the current study, also that survival was with Health functional of and and of right and pulmonary vascular Although of these are these results the of mortality in this patient including both CTEPH as as age and with a higher of patients in the current study underwent PTE surgery, with in the operated group vs to in S.R. Swift A.J. Billings C.G. et al.The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension.Eur Respir J. 2018; 52Crossref PubMed Scopus (60) Google M. Lang I. Pepke-Zaba J. et al.Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry.Circulation. 2016; 133: 859-871Crossref PubMed Scopus (424) Google R. Kiely D.G. Gibbs J.S. et al.Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension.Am J Respir Crit Care Med. 2008; 177: 1122-1127Crossref PubMed Scopus (339) Google Scholar a of patients classified as inoperable and also more patients classified as operable in surgery was not this in patient or is Although these the of patients in the current study also underwent at rates and at rates with a recent S. D’Armini A.M. M. et for chronic thromboembolic pulmonary hypertension: results of the prospective CTEPH 2021; PubMed Scopus Google Scholar In that study, significant was with at as has been characteristics vs patients in the operated group to to have and to more patients vs inoperable and operable but no surgery also that patients in the operated group were more to vs patients in the operable but no surgery group. survival at 3 years in the current study years was vs that in this in was larger in the inoperable and operable no surgery groups, outcomes across studies have been S.R. Swift A.J. Billings C.G. et al.The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension.Eur Respir J. 2018; 52Crossref PubMed Scopus (60) Google M. Lang I. Pepke-Zaba J. et al.Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry.Circulation. 2016; 133: 859-871Crossref PubMed Scopus (424) Google R. Kiely D.G. Gibbs J.S. et al.Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension.Am J Respir Crit Care Med. 2008; 177: 1122-1127Crossref PubMed Scopus (339) Google Scholar in the current study, survival at 3 years was in the inoperable group and in the operable no surgery group vs in the inoperable group and in the operable but no surgery group in the cohort by et S.R. Swift A.J. Billings C.G. et al.The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension.Eur Respir J. 2018; 52Crossref PubMed Scopus (60) Google Scholar in Although this to differences in the patient in the treatment of CTEPH also potentially is approved as medical for patients with CTEPH with inoperable or PH after PTE surgery, and is used for patients with CTEPH with on in both a the clot surgically as a patient is a good for surgery more surgical a to review and to and in the is CTEPH is In the current study, the of to adjudication were as CTEPH on review of with the reasons were imaging more with and no evidence of CTEPH on imaging patients also significant in with a median of months from to diagnosis in this the for a of in patients with dyspnea as as the for improved of the differences in imaging characteristics for vs chronic S. in chronic thromboembolic pulmonary Thorac PubMed Scopus Google Scholar patients with a diagnosis of surgically CTEPH but who did not undergo surgery, the reasons were that the patient surgery and the of surgery the reasons are to those in the study by et S.R. 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