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Publication, Part of

National Audit of Pulmonary Hypertension, 13th Annual Report

Audit, Open data

Publication Date:
19 Jan 2023
Geographic Coverage:
England, Scotland
Geographical Granularity:
NHS Trusts, Country, Hospital Trusts
Date Range:
01 Apr 2021 to 31 Mar 2022

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Future publication format

Please note that future reports will be web-based only. This is to improve the accessibility of our publications.

19 January 2023 09:30 AM

Page contents

Reference tables

Summary

Chart R 1 and Chart R 2 shows that the number of new referrals and patients who started therapy increased steadily between 2009-10 and 2019-20, but dropped sharply in 2020-21. These numbers increased in 2021-22 to levels similar to those seen in 2019-20.

Chart R 3 shows that the number of patients newly diagnosed with CTEPH or PAH have risen since 2009-10 but have shown a sharp drop in 2020-21. These numbers increased in 2021-22 but not to 2019-20 levels.

Chart R 4 shows that the number of active patients has consistently risen since 2004. This is also the case for number of active drug therapies until 2021 where there is a slight decrease for the first time.

Table R 1 shows that the growth in the number of active patients is evident across most PH centres since 2010.

The number of managed patients with and without PAH / CTEPH has consistently risen since 2009 as shown in Table R 2.

Variations in diagnosis types between PH centres are shown in Table R 3 and Table R 4.

Table R 5 shows that Sildenafil is prescribed most frequently. The most marked percentage increase in prescription numbers between 31 March 2021 and 2022 was for Ambrisentan, Iloprost, Tadalafil, and Selexipag, and there was a marked decrease in Bosentan. Similar patterns are evident for CTEPH patients (Table R 6).

Table R 7 shows that the number of patients living with CTEPH is increasing, as is the number of CTEPH patients referred to PH centres. The proportion of CTEPH patients discharged for reasons other than death is around 18%. A similar pattern for patients living with PAH is shown in Table R 8. Around 5% of PAH patients were discharged for reasons other than death.

Chart R 5 suggests a shortening of the length between initial diagnosis of CTEPH and PEA performed between 2012-13 and 2019-20. However, this greatly increased in 2020-21 and although the range of waiting times improved in 2021-22, the overall average waiting time did not.

Table R 9 shows that there has been an increase in active drug therapies that are combination therapies since 2010, though this trend is less pronounced for CTEPH patients.

Analysis of patients with IPAH with comorbidities and IPAH without comorbidities is not included in the 13th Annual Report. This is because the NAPH team are in the process of incorporating the new NAPH comorbidities fields into the definition of IPAH with comorbidities and IPAH without comorbidities.

In previous annual reports IPAH with comorbidities was defined as a IPAH diagnosis with a secondary diagnosis relating to a comorbidity. The number of patients with IPAH with comorbidities recorded in the Audit using this definition is believed to be an underestimate of the true prevalence and therefore the results from analysis of this cohort may not be a true representation.

Referrals and therapy

Chart R 1: Number of new referrals to PH centres during audit year, Great Britain, 2009-22

Chart R 1: Number of new referrals to PH centres during audit year, Great Britain, 2009-22

 

Chart R 2: Number of patients who started therapy during audit year, Great Britain, 2009-22

Chart R 2: Number of patients who started therapy during audit year, Great Britain, 2009-22

Key findings
  • The number of new referrals and patients who started therapy have steadily increased between 2009-10 and 2019-20. However these numbers dropped sharply in 2020-21 to the extent that the number of patients who started therapy in 2020-21 is lower than the number in 2009-10. In 2021-22 the number of new referrals and patients who started therapy increased to levels similar to those in 2019-20.

New CTEPH or PAH diagnoses

Chart R 3: Number of patients newly diagnosed with CTEPH or PAH during audit year, Great Britain, 2009-22

Chart R 3: Number of patients newly diagnosed with CTEPH or PAH during audit year, Great Britain, 2009-22

Key findings
  • The number of patients newly diagnosed with CTEPH or PAH increased slightly over the audit years but then made a sharp drop in 2020-21 and has increased again in 2021-22.

Active patients and therapies

Chart R 4: Number of active patients and active drug therapies on 31 March, Great Britain, 2004-22

Chart R 4: Number of active patients and active drug therapies on 31 March, Great Britain, 2004-22

Notes:

1. Active patients have a referral active on the 31 March of the stated year (see Glossary: Patient cohorts).

2. Active drug therapies represent patients with at least 1 pulmonary hypertension drug therapy being prescribed on the 31 March of the appropriate year (see Glossary: Patient cohorts).

3. The data for 2004-2009 was collected before the Audit began. It was collected by the National Pulmonary Hypertension Centres of the UK and Ireland Physicians Committee.

Active patients

Table R 1: Number of active patients on 31 March by PH centre, Great Britain, 2010-22

PH centre 2010 2011 2012 2013 2014 2015 2016 2017 2018 2019 2020 2021 2022
Total 4,348 4,833 5,247 5,590 5,968 6,299 6,523 6,754 7,014 7,205 7,473 7,721 8,267
Golden Jubilee 350 369 401 441 498 531 575 589 637 649 652 638 645
Great Ormond Street 278 265 301 297 293 288 293 294 299 282 282 308 311
Imperial College 653 716 797 849 882 941 927 906 952 935 948 955 972
Newcastle 280 290 300 313 316 331 335 326 333 341 365 363 380
Royal Brompton & Harefield 508 607 639 698 698 696 657 755 768 804 868 874 963
Royal Free 629 753 854 902 899 978 1,044 1,109 1,218 1,320 1,360 1,425 1,533
Royal Papworth 702 745 808 884 926 999 1,028 1,045 976 911 1,002 1,061 1,177
Sheffield 948 1,088 1,147 1,206 1,456 1,535 1,664 1,730 1,831 1,963 1,996 2,097 2,286

Notes:

1. Active patients have a referral active on the 31 March of the stated year (see Glossary: Patient cohorts).

Key findings
  • The number of active patients has consistently increased across most PH centres from 2010 to 2022.

Managed patients

Table R 2: Number of managed patients during the audit year, Great Britain, 2009-22

Audit year Managed patients Managed patients with PAH or CTEPH
n %
2009-10 5,710 3,311 58
2010-11 6,418 3,781 59
2011-12 7,001 4,133 59
2012-13 7,500 4,454 59
2013-14 8,112 4,861 60
2014-15 8,598 5,201 60
2015-16 8,944 5,441 61
2016-17 9,470 5,712 60
2017-18 9,713 5,899 61
2018-19 10,135 6,065 60
2019-20 10,327 6,285 61
2020-21 9,875 6,290 64
2021-22 10,922 6,310 58

Notes:

1. Managed patients have a referral active at any point during the stated year (see Glossary: Patient cohorts).

Key findings
  • The number of managed patients with PH has consistently increased from 2009-10 to 2019-20, but has decreased slightly in 2020-21. This increased again in 2021-22.

Diagnosis type of active patients

Table R 3: Percentage of active patients on 31 March 2022 by PH centre and diagnosis type, Great Britain, 2022

Diagnosis type PH centre
National total Golden Jubilee Great Ormond Street Imperial College Newcastle Royal Brompton and Harefield Royal Free Royal Papworth Sheffield
% % % % % % % % %
PAH 44 50 46 44 51 55 29 26 56
Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis 0 2 1 0 0 0 0 0 0
Left heart disease 3 1 3 2 1 1 8 2 2
Lung disease 5 9 27 3 0 7 2 1 6
CTEPH 25 24 1 23 38 19 18 45 23
...operated 12 11 1 13 18 6 9 26 11
...not operated 12 13 0 11 20 13 9 18 12
Miscellaneous/uncertain causes 3 3 3 1 2 8 1 2 3
Not pulmonary hypertension 14 8 15 23 2 2 29 17 5
No final diagnosis possible 1 0 0 0 0 4 2 1 0
No diagnosis 6 4 4 2 6 2 10 8 5
Total patients (n) 8,267 645 311 972 380 963 1,533 1,177 2,286

Notes:

1. Active patients have a referral active on the 31 March of the stated year (see Glossary: Patient cohorts).

PAH type of active patients

Table R 4: Percentage of active patients on 31 March 2022 by PH centre and PAH type, Great Britain, 2022

PAH type PH centre
National total Golden Jubilee Great Ormond Street Imperial College Newcastle Royal Brompton and Harefield Royal Free Royal Papworth Sheffield
% % % % % % % % %
IPAH 33 47 18 43 46 22 24 36 32
Connective Tissue Disease APAH 24 21 1 12 14 20 48 25 24
…scleroderma 17 11 1 5 9 13 37 25 18
…excluding scleroderma 7 10 1 7 5 7 11 0 6
Portal Hypertension APAH 4 8 1 5 5 2 5 1 4
Congenital Heart Disease APAH 33 24 71 30 27 54 11 17 35
PAH - Other 4 0 1 4 4 1 10 2 4
PAH - No sub diagnosis 3 0 8 5 4 0 2 19 1
Total patients (n) 3,649 321 144 430 194 534 451 303 1,272

Notes:

1. Active patients have a referral active on the 31 March of the stated year (see Glossary: Patient cohorts). 

Drug prescriptions

Table R 5: Number of drug prescriptions on 31 March, Great Britain, 2010-22

Drug 2010 2011 2012 2013 2014 2015 2016 2017 2018 2019 2020 2021 2022
Sildenafil 1,521 1,756 2,098 2,384 2,665 2,973 3,128 3,263 3,272 3,312 3,303 3,131 3,156
Tadalafil 3 4 31 107 146 192 260 345 509 719 947 1,078 1,313
Bosentan 1,032 1,113 1,141 1,133 1,177 1,000 831 707 613 580 549 515 451
Ambrisentan 125 274 324 431 516 542 598 615 661 721 754 807 1,016
Sitaxsentan 100 2 -    -    -    -    -    -    -    -    -                -    -
Macitentan -    -    -    -    -    297 537 726 984 1,144 1,309 1,338 1,371
Iloprost 109 107 112 126 154 157 151 154 158 192 175 104 129
Treprostinil 88 69 60 55 44 40 32 27 26 27 28 26 26
Epoprostenol 47 58 68 85 88 97 114 119 139 153 154 200 222
CCB* for vaso-reactive PAH 39 36 43 52 58 58 60 63 74 80 79 88 84
Riociguat -    -    -    -    -    14 74 110 154 199 213 223 253
Selexipag -    -    -    -    -    -    -    0 3 53 204 290 346
Unknown – Drug trial 108 126 125 123 246 241 245 240 228 222 199 182 174
Unknown – Not known 12 10 8 8 7 7 7 7 7 7 6 7 7

Notes:

* Calcium channel blocker.

- = Drug not available for use throughout year.

1. Drug prescriptions include monotherapy and combination therapy.

2. Sitaxsentan was withdrawn from use in late 2010.

Drug prescriptions for CTEPH patients

Table R 6: Number of drug prescriptions for CTEPH patients on 31 March, Great Britain, 2010-22

Drug 2010 2011 2012 2013 2014 2015 2016 2017 2018 2019 2020 2021 2022
Sildenafil 317 381 430 488 529 609 638 647 668 695 683 649 659
Tadalafil 0 0 1 11 18 21 26 35 55 71 126 173 226
Bosentan 180 174 162 154 147 127 91 71 46 45 38 30 26
Ambrisentan 10 20 32 53 66 76 71 65 60 53 48 62 106
Macitentan -    -    -    -    -    32 55 76 140 178 223 233 242
Iloprost 11 14 10 12 12 15 9 7 6 8 7 4 5
Treprostinil 4 4 3 2 2 0 0 0 0 0 1 1 1
Epoprostenol 1 2 3 4 3 3 4 2 1 2 3 5 4
Riociguat -    -    -    -    -    8 66 99 121 154 163 162 184
Selexipag -    -    -    -    -    -    -    0 0 3 2 4 3
Unknown – Drug trial 0 1 2 1 1 1 1 1 1 1 1 1 1
Unknown – Not known 3 3 4 5 20 15 8 9 9 8 13 11 10

Notes:

- = Drug not available for use throughout year.

1. Drug prescriptions include monotherapy and combination therapy.

CTEPH

Table R 7: Patients with CTEPH and new CTEPH diagnoses, Great Britain, 2009-22

Patients with CTEPH… Audit year (patients)
2009-10 2010-11 2011-12 2012-13 2013-14 2014-15 2015-16 2016-17 2017-18 2018-19 2019-20 2020-21 2021-22
alive on 31 March at the end of audit year 803 992 1,179 1,364 1,523 1,716 1,906 2,047 2,261 2,487 2,666 2,721 2,849
with an active referral (1) at the end of audit year 752 901 1,040 1,166 1,268 1,381 1,485 1,537 1,660 1,777 1,899 1,922 2,041
newly-diagnosed with CTEPH during audit year 236 263 269 280 279 324 344 327 380 401 401 277 327
Patients with CTEPH… End of audit year (%)
2010 2011 2012 2013 2014 2015 2016 2017 2018 2019 2020 2021 2022
in the cohort for longitudinal analysis (2) and having active drug therapy on 31 March (2) 49 46 42 41 42 45 47 47 48 50 50 48 52
Patients with CTEPH… Diagnosed before end of audit period (%)
2009-10 2009-11 2009-12 2009-13 2009-14 2009-15 2009-16 2009-17 2009-18 2009-19 2009-20 2009-21 2009-22
in the cohort for longitudinal analysis (2) and discharged from PH service within 5 years (excluding where reason for PH discharge is death) - - - - - 21 21 21 21 21 20 19 18
As above (CTEPH operated) - - - - - 21 21 23 22 23 21 20 19
As above (CTEPH not operated) - - - - - 20 20 19 19 19 18 19 17

Notes:

- = Number of patients in the cohort below 50.

1. Active patients have a referral active on the 31 March of the stated year (see Glossary: Patient cohorts).

2. The cohort for longitudinal analysis includes those whose 1st referral was on or after 1 April 2009 (see Glossary: Patient cohorts).

 

PAH

Table R 8: Patients with PAH and new PAH diagnoses, Great Britain, 2009-22

Patients with PAH… Audit year (patients)
2009-10 2010-11 2011-12 2012-13 2013-14 2014-15 2015-16 2016-17 2017-18 2018-19 2019-20 2020-21 2021-22
alive on 31 March at the end of audit year 2,234 2,459 2,709 2,900 3,189 3,385 3,573 3,721 3,865 4,034 4,191 4,163 4,269
with an active referral (1) at the end of audit year 1,939 2,145 2,364 2,533 2,770 2,944 3,093 3,171 3,294 3,419 3,553 3,536 3,649
newly-diagnosed with PAH during audit year 506 516 573 539 646 625 606 609 617 580 631 438 568
Patients with PAH… End of audit year (%)
2010 2011 2012 2013 2014 2015 2016 2017 2018 2019 2020 2021 2022
in the cohort for longitudinal analysis (2) and having active drug therapy on 31 March 61 68 74 78 82 84 87 87 88 90 91 90 92
Patients with PAH… Diagnosed before end of audit period (%)
2009-10 2009-11 2009-12 2009-13 2009-14 2009-15 2009-16 2009-17 2009-18 2009-19 2009-20 2009-21 2009-22
in the cohort for longitudinal analysis (2) and discharged from PH service within 5 years (excluding where reason for PH discharge is death) 1 3 3 4 4 5 5 5 5 5 5 5 5

Notes: 
1. Active patients have a referral active on the 31 March of the stated year (see Glossary: Patient cohorts). 
2. The cohort for longitudinal analysis includes those whose first referral was on or after 1 April 2009 (see Glossary: Patient cohorts).
 

Interval to PEA

Chart R 5: Number of weeks between initial diagnosis of CTEPH and PEA performed, Great Britain, 2009-22

Chart R 5: Number of weeks between initial diagnosis of CTEPH and PEA performed, Great Britain, 2009-22

Notes:

1. Intervals outside the interquartile range (IQR) by more than 1.5 times the IQR are shown as ‘out of range’ (blue cross). Not all out of range values are displayed on the plot. The maximum interval for each audit year is shown on the y axis (left).

Therapy types and transplants

Table R 9: Percentage of active drug therapies (1) that are mono-, dual or triple therapies on 31 March, Great Britain, 2010-22

Year All patients CTEPH
Mono Dual Triple+ Mono Dual Triple+
2010 72 25 3 81 18 1
2011 71 26 3 82 17 1
2012 68 30 3 82 17 1
2013 64 32 3 78 21 1
2014 64 33 3 78 21 1
2015 61 36 3 78 21 1
2016 59 38 4 80 19 1
2017 58 39 4 81 19 0
2018 54 42 4 79 21 0
2019 52 42 6 77 23 0
2020 50 42 8 74 26 0
2021 49 41 10 73 26 0
2022 47 42 11 71 29 0

Notes: 
1. Active drug therapies represent patients with at least 1 pulmonary hypertension drug therapy being prescribed on the 31 March of the appropriate year (see Glossary: Patient cohorts). 
 

Table R 10: Number of transplants (lung or heart/lung), Great Britain, 2021-22

Description Number of transplants
Transplants 1

This is a particularly low number of transplants and is believed to be an underestimate of the true number of transplants performed within the audit year. This is likely to be a data quality issue and we encourage PH centres to submit data on transplants as part of the Audit.

Last edited: 19 January 2023 9:31 am

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