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Observational Study
. 2024 Jan 4;14(1):e080068.
doi: 10.1136/bmjopen-2023-080068.

Risk assessment and real-world outcomes in chronic thromboembolic pulmonary hypertension: insights from a UK pulmonary hypertension referral service

David G Kiely  1   2   3 , Neil Hamilton  4 , Steven Wood  3   5 , Charlotte Durrington  4   2 , Fernando Exposto  6 , Ruvimbo Muzwidzwa  6 , Louise Raiteri  6 , Amélie Beaudet  7 , Audrey Muller  7 , Rafael Sauter  7 , Nadia Pillai  7 , Allan Lawrie  2   8 ; ASPIRE consortium
Collaborators, Affiliations
Observational Study

Risk assessment and real-world outcomes in chronic thromboembolic pulmonary hypertension: insights from a UK pulmonary hypertension referral service

David G Kiely et al. BMJ Open. .

Abstract

Objectives: This study was conducted to evaluate the ability of risk assessment to predict healthcare resource utilisation (HCRU), costs, treatments, health-related quality of life (HRQoL) and survival in patients diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH).

Design: Retrospective observational study.

Setting: Pulmonary hypertension referral centre in the UK.

Participants: Adults diagnosed with CTEPH between 1 January 2012 and 30 June 2019 were included. Cohorts were retrospectively defined for operated patients (received pulmonary endarterectomy (PEA)) and not operated; further subgroups were defined based on risk score (low, intermediate or high risk for 1-year mortality) at diagnosis.

Primary and secondary outcome measures: Demographics, clinical characteristics, comorbidities, treatment patterns, HRQoL, HCRU, costs and survival outcomes were analysed.

Results: Overall, 683 patients were analysed (268 (39%) operated; 415 (61%) not operated). Most patients in the operated and not-operated cohorts were intermediate risk (63%; 53%) or high risk (23%; 31%) at diagnosis. Intermediate-risk and high-risk patients had higher HCRU and costs than low-risk patients. Outpatient and accident and emergency visits were lower postdiagnosis for both cohorts and all risk groups versus prediagnosis. HRQoL scores noticeably improved in the operated cohort post-PEA, and less so in the not-operated cohort at 6-18 months postdiagnosis. Survival at 5 years was 83% (operated) and 49% (not operated) and was lower for intermediate-risk and high-risk patients compared with low-risk patients.

Conclusions: Findings from this study support that risk assessment at diagnosis is prognostic for mortality in patients with CTEPH. Low-risk patients have better survival and HRQoL and lower HCRU and costs compared with intermediate-risk and high-risk patients.

Keywords: Chronic airways disease; Mortality; Quality of Life.

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Conflict of interest statement

Competing interests: DGK has received grants from Janssen Pharmaceuticals and Ferrer; consulting fees from Janssen Pharmaceuticals, MSD, Ferrer, Altavant and United Therapeutics; honoraria from Janssen Pharmaceuticals, MSD, Ferrer and United Therapeutics; funding from Janssen Pharmaceuticals, MSD and Ferrer to attend scientific meetings; has participated in a data safety monitoring board or Advisory Board for Janssen Pharmaceuticals and MSD; serves on the Specialist Respiratory Clinical Reference Group (unpaid) and as the UK National Audit Chair. NH has received honoraria payments from Janssen Pharmaceuticals, Vifor Pharmaceuticals and MSD. SW has received grants from Janssen Pharmaceuticals in support of the current study. CD has received a grant from Janssen, UK for investigator-led research unrelated to the present research, and a speaker’s honorarium from Janssen for an educational lecture. FE, LR and RM have no conflicts to disclose. AB, AM, RS and NP are employees of Actelion Pharmaceuticals. AB, AM and NP own stock in Johnson & Johnson. AL is supported by a British Heart Foundation Senior Basic Science Research Fellowship (FS/18/52/33808).

Figures

Figure 1
Figure 1
Overview of observation times and database coverage in the study. Purple boxes represent HES database; green boxes represent Sheffield PVDU database. EMR, electronic medical record; HCRU, healthcare resource utilisation; HES, Hospital Episode Statistics; HRQoL, health-related quality of life; PEA, pulmonary endarterectomy; PVDU, Pulmonary Vascular Disease Unit.
Figure 2
Figure 2
Comorbidities recorded as present in the 5 years prior to CTEPH diagnosis. Data were collected from the HES database. CKD, chronic kidney disease; COPD, chronic obstructive pulmonary disease; CTEPH, chronic thromboembolic pulmonary hypertension; HES, Hospital Episode Statistics.
Figure 3
Figure 3
Median HCRU per patient per year, 1 year before and 1 and 3 years after CTEPH diagnosis in (A) operated patients and (B) not-operated patients. *HCRU at 3 years postdiagnosis represents median per year over 3 years, not during the third year. Same-day visits defined as inpatient visit where admission and discharge date are the same. A&E, accident and emergency; HCRU, healthcare resource utilisation.
Figure 4
Figure 4
Median HCRU per patient in the year before and after CTEPH diagnosis, stratified by risk category at diagnosis in (A) operated and (B) not-operated patients. Same-day visits defined as inpatient visit where admission and discharge date are the same. A&E, accident and emergency; HCRU, healthcare resource utilisation.
Figure 5
Figure 5
Median cost (GBP) per patient in the year after CTEPH diagnosis. Costs were obtained from HES, which is for administrative use; therefore, practices might vary across hospitals/clinical coders. Costs associated with PEA surgery in patients with CTEPH are reimbursed separately and the values quoted for cost of care do not include those associated with PEA surgery. Same-day visits defined as inpatient visit where admission and discharge date are the same. CTEPH, chronic thromboembolic pulmonary hypertension; GBP, British Pound Sterling; HES, Hospital Episode Statistics; PEA, pulmonary endarterectomy.
Figure 6
Figure 6
Median EmPHasis-10 scores at diagnosis and follow-up in the (A) operated cohort and (B) not-operated cohort by risk score at diagnosis. Due to suppression rules, paired analysis of low-risk patients could not be conducted. The EmPHasis-10 is a quality-of-life instrument developed and validated specifically for assessment of HRQoL in patients with PH. Higher scores are indicative of worse HRQoL. HRQoL, health-related quality of life; PEA, pulmonary endarterectomy; PH, pulmonary hypertension; Supp, suppressed.
Figure 7
Figure 7
Survival from diagnosis in the operated and not-operated cohorts by risk score at diagnosis. Solid lines represent the not-operated cohort and dashed lines represent the operated cohort.
See this image and copyright information in PMC

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