National Audit of Pulmonary Hypertension 7th Annual Report, April 2015 to March 2016

Transcription

1 National Audit of Pulmonary Hypertension 7th Annual Report, April 2015 to March 2016 Published 23 March 2017 The Audit sets out to measure the quality of care provided to people referred to pulmonary hypertension services in England and Scotland. Key findings The Pulmonary Hypertension (PH) service managed 9,068 patients. 86 per cent of patients referred to a specialist centre attended a consultation or were discharged within 90 days and 45 per cent were seen or discharged within 30 days. 92 per cent of patients referred to a specialist centre were seen, assessed and diagnosed within six months. 80 per cent of patients had a WHO functional class and six-minute walk or shuttle exercise test recorded before being given drugs to treat PH. 88 per cent of patients received a right heart catheterization before being given drugs to treat Pulmonary Hypertension. 74 per cent of patients, who have had at least one consultation in the last year, have had a quality of life questionnaire recorded during that time. For 88 per cent of patients, their first line drug therapy was a phosphodiesterase 5 inhibitor. 92 per cent of patients taking PH specific drugs had at least one consultation at a specialist PH centre within the last 13 months. 99 per cent of patients receiving drug treatment for PH had an official diagnosis recorded. Author: Clinical Audits and Registries Management Service, NHS Digital Copyright 2017 Health and Social Care Information Centre. The Health and Social Care Information Centre is a non-departmental body created by statute, also known as NHS Digital.

2 Contents Key findings 1 Introduction 3 1. Service Provision 7 2. Treatment and timeliness Referral, Treatment and Outcomes 24 Appendix 1 Reference Tables 32 Appendix 2 Standards not used in the report 35 Appendix 3 World Health Organization (WHO) Functional Class 37 Appendix 4 Dana Point Classification of Pulmonary Hypertension 38 Appendix 5 The cohort for longitudinal analysis 40 Appendix 6 Members of staff involved from the pulmonary hypertension centres 41 Appendix 7 Members of the National Audit of Pulmonary Hypertension Project Board 43 ISBN This report is of particular interest to commissioners and healthcare providers to monitor the quality and effectiveness of services. It may also be of interest to researchers and charities working on this subject and to members of the public. Copyright 2017, Health and Social Care Information Centre. 2 Copyright 2017 Health and Social Care Information Centre. NHS Digital is the trading name of the Health and Social Care Information Centre.

3 Introduction Pulmonary hypertension is raised blood pressure within the pulmonary arteries, which are the blood vessels that supply the lungs. It is a serious medical condition that can damage the right side of the heart, making the heart less efficient at pumping blood around the body and getting oxygen to the muscles 1. The National Audit of Pulmonary Hypertension measures the quality of care provided to people referred to pulmonary hypertension services in England (including patients referred from Wales) and Scotland, by answering a number of questions: Are pulmonary hypertension services appropriate? Are patients receiving the right treatment in a timely manner? What are the outcomes for patients with pulmonary hypertension? The Audit answers these questions through the measurement of a number of professionally agreed standards reported at a national level. It is important to note that the results shown in this report are based on the data submitted into the Audit database by the pulmonary hypertension centres. If the data entered does not accurately represent clinical practice the results of the Audit may be misleading. The use of standards in the Audit This is the first time that the Audit has used standards against which to measure clinical practice. Clinical audit is a recognised and established approach to quality improvement in clinical practice. The key principle of clinical audit is the measurement of clinical practice against professionally developed clinical standards. Where practice does not meet the standard, the clinical team should investigate and reflect on why, before developing local action plans to document the proposed action for change if this is identified. The standards used in the Audit were compiled and agreed for the seventh Annual Report by: Dr Simon Gibbs, consultant cardiologist and clinical lead for the Audit Ms Kathy Blacker, lead commissioner for pulmonary hypertension services Dr Iain Armstrong, chair of PHA-UK 1 Definition from NHS Choices Copyright 2017, Health and Social Care Information Centre. 3

4 Use of the standards was then approved by the representatives on the Pulmonary Hypertension Outcomes Group. The standards used are applicable to all centres, even though the centres do not provide identical services. As the standards were agreed after the audit data was collected it was decided to publish the results at a national level this first year. In line with previous annual reports, specialist centre level results will be published in the next Annual Report. Most of the standards have a threshold, the percentage value that a centre is expected to be over or under. These thresholds are not set to 0 or 100 per cent as the standards do not attempt to take every factor into account. This is because sometimes the care provided might happen in a way that does not meet the standard, but it is not an indication that what happened was incorrect or bad practice. The purpose of the standard is to set a target level of compliance and for centres to reflect locally if changes in practice need to occur. The standards will be reviewed by the Pulmonary Hypertension Outcomes Group and developed over time, to make them more challenging and thus raise the quality of care being delivered. New standards will be introduced and those no longer of value will be retired. It is expected when developing standards that sometimes the analysis does not show what was intended to be measured and therefore that standard cannot be reported on. Such standards will be included, with commentary, in the appendices and then removed from further measurement. Recommendations Recommendations form an important part of the clinical audit cycle. Based on the findings of the analysis, the clinical lead for the Audit, Dr Simon Gibbs, in collaboration with the clinical leads at each centre, has made recommendations to give direction and aid improvements in care practice. Data quality An important part of achieving the standards is correct and complete data entry into the Audit. Through the process of producing this report, a number of centres have identified that the quality of data they have submitted is not sufficient to enable an accurate picture of the performance of their services. This should be borne in mind when interpreting the results, particularly where the recommendation states the need to improve the quality of data. However, there has been a commitment from centres to review how they collect the data and enter it into the Audit database so that improvements in data quality are seen year on year. Copyright 2017, Health and Social Care Information Centre. 4

5 Abbreviations used in the report PH pulmonary hypertension PAH pulmonary arterial hypertension CTEPH chronic thromboembolic pulmonary hypertension WHO World Health Organization PHA-UK Pulmonary Hypertension Association UK PEA pulmonary endarterectomy Pulmonary Hypertension Centres Table 1: Specialist pulmonary hypertension centres participating in the National Audit of Pulmonary Hypertension 7th Annual Report Centre Golden Jubilee National Hospital Imperial College Healthcare NHS Trust Papworth Hospital NHS Foundation Trust Royal Brompton and Harefield NHS Foundation Trust Royal Free London NHS Foundation Trust Sheffield Teaching Hospitals NHS Foundation Trust The Newcastle Upon Tyne Hospitals NHS Foundation Trust Short Name used in report Golden Jubilee Imperial College Papworth Hospital Royal Brompton and Harefield Royal Free Sheffield Newcastle Upon Tyne Hospitals Copyright 2017, Health and Social Care Information Centre. 5

6 Overview of the service The service continues to grow at a steady rate with double the number of patients active in the latest year than when the Audit started in Whilst the number of patients receiving pulmonary hypertension therapies has grown, the proportion of these patients in the service has remained broadly the same (57 per cent in 2009 and 56 per cent in 2016). Notes: 1. The data for was collected before the Audit began. It was collected by the National Pulmonary Hypertension Centres of the UK and Ireland Physicians Committee. 2. From 2010 onwards these figures do not include Northern Ireland or children (from Great Ormond Street Hospital). As such they differ from previously published figures. 3. Active patients have a referral active on the 31 March of the appropriate year. 'Active Therapy' represents patients with at least one pulmonary hypertension drug therapy being prescribed on the 31 March of the appropriate year. Copyright 2017, Health and Social Care Information Centre. 6

7 1. Service Provision 1.1 Are pulmonary hypertension centres seeing sufficient numbers of patients, performing sufficient numbers of vasoreactivity studies and participating in the Audit? Why is this important? Participation in national audit can be considered a quality measure of a hospital since it indicates a willingness to share data and change practice to improve clinical performance. Evidence from other disease areas suggests that best outcomes for patients are obtained in high volume centres. High volume centres also enable provision of a full range of investigation modalities, specialist nursing support and involvement of a wider multi-professional team with expertise in patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH). The recommended number of patients seen is based on European guidelines which are used by the UK specialist centres. The number of vasoreactivity studies in patients with suspected idiopathic, heritable or drug associated PAH at cardiac catheterization is also recommended in European guidelines as an additional measurement of clinical experience. The need to perform such a study depends on clinical judgement. Standard to be measured against All specialist pulmonary hypertension centres are expected to participate in the National Audit of Pulmonary Hypertension. Result All adult centres participated in the Audit. Great Ormond Street Hospital were unable to participate due to local data collection issues. Copyright 2017, Health and Social Care Information Centre. 7

8 Standard to be measured against The specialist pulmonary hypertension service as a whole is expected to manage at least 2100 patients per annum (with a minimum number in each centre of 300 patients) patients per annum should be patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Result Table 2: Number of managed patients, and managed patients with a latest diagnosis of pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) during Percentage of managed patients with PAH or CTEPH Managed Patients Managed Patients with PAH or CTEPH National totals 9,068 5,337 59% Key Findings Source: NHS Digital The specialist pulmonary hypertension service is seeing the recommended number of patients with PAH and CTEPH. Standard to be measured against The specialist pulmonary hypertension service is expected to perform at least 140 vasoreactivity studies per annum (with a minimum number in each centre of 20) in patients with idiopathic, heritable or anorexigen-induced pulmonary arterial hypertension. Result A total of 40 vasoreactivity studies were performed by the service. Vasoreactivity studies are not expected to be carried out as part of all catheterizations - a vasoreactivity study would be expected to be carried out somewhere between 1 in 10 and 4 in 10 catheterizations. 274 patients had a catheterization with a latest diagnosis of idiopathic, heritable or anorexigen-induced PAH. Table 3: Number of vasoreactivity studies and catheterizations performed on patients with a latest diagnosis of idiopathic, heritable or anorexigen-induced PAH in Catheterizations for patients with a latest diagnosis of Vasoreactivity studies idiopathic, heritable or anorexigen-induced PAH National totals Source: NHS Digital Copyright 2017, Health and Social Care Information Centre. 8

9 Key Findings The standard for the number of vasoreactivity studies has not been met. Recommendations Centres should perform vasoreactivity studies in appropriate patients. It is recognised that vasoreactivity studies may not be appropriate in certain clinical situations. Data concerning vasoreactivity studies needs to be entered into the Audit since preliminary enquiries have noted that this field is not being completed systematically. Copyright 2017, Health and Social Care Information Centre. 9

10 2. Treatment and timeliness 2.1 Are the World Health Organization functional class and exercise test results recorded in patients with PAH or CTEPH? Why is this important? The World Health Organization (WHO) functional class is a measure of the extent of functional limitation due to pulmonary hypertension and is a prognostic factor. Patients in different WHO functional classes require different PAH drugs and combinations of these drugs. The extent of exercise limitation due to PAH/CTEPH can be assessed quantitatively by a formal exercise test such as the six minute walk test or incremental shuttle walking test. Both WHO functional class and exercise performance may improve with treatment and by recording this information a response to treatment can be documented later. Standard to be measured against 95 per cent of patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension should have a WHO functional class and exercise test (six minute walk test or shuttle test) recorded before they begin any pulmonary hypertension therapy. Results Key findings 80 per cent of patients are having their WHO functional class assessed and have completed an exercise test. Whilst high, this does not meet the agreed standard. Copyright 2017, Health and Social Care Information Centre. 10

11 Recommendations Centres should aim to record the WHO functional class and an exercise test result for all patients prior to commencing therapy. Allowance needs to be made for patients who are unable to perform an exercise test for reasons unrelated to pulmonary hypertension or who refuse. Copyright 2017, Health and Social Care Information Centre. 11

12 2.2 Do patients receiving a pulmonary hypertension drug therapy have a cardiac catheterization recorded before treatment begins? Why is this important? Cardiac catheterization is considered the gold standard method for confirming the presence, nature and severity of pulmonary hypertension. It is reasonable to be certain about the diagnosis before starting treatment. Standard to be measured against 95 per cent of patients receiving a pulmonary hypertension drug therapy (excluding patients diagnosed as pulmonary arterial hypertension associated with congenital heart disease) should have a cardiac catheterization recorded before treatment begins. Results Key findings Almost 90 per cent of patients are being assessed to ensure pulmonary hypertension is present before treatment is commenced. Whilst high, this does not meet the agreed standard. Recommendations Centres should aim to perform and record the results of right heart catheterizations in all patients prior to commencing therapy. Note that it is recognised that commencing treatment prior to right heart catheterization may be appropriate in certain uncommon clinical situations. Copyright 2017, Health and Social Care Information Centre. 12

13 2.3 Do patients receiving a pulmonary hypertension drug have a pulmonary hypertension diagnosis recorded? Why is this important? Specialist pulmonary hypertension drugs should only be given to patients with pulmonary hypertension. It is important to confirm that a patient to whom a drug is prescribed, has an appropriate diagnosis. Standard to be measured against 99 per cent of patients receiving a pulmonary hypertension drug therapy should have a pulmonary hypertension diagnosis recorded. Results Key findings The specialist pulmonary hypertension service is ensuring that a diagnosis has been made before pulmonary hypertension treatment is started. Copyright 2017, Health and Social Care Information Centre. 13

14 2.4 What is the proportion of patients who have a diagnosis of no pulmonary hypertension who have an average pulmonary artery pressure greater than or equal to 25mm Hg? Why is this important? This standard checks the consistency of the measurement of the pulmonary artery pressure at cardiac catheterization with the final diagnosis for each patient. Since pulmonary hypertension is defined as a mean pulmonary artery pressure greater than or equal to 25 mm Hg, patients with this finding should not be given a diagnosis of no pulmonary hypertension. Standard to be measured against The proportion of patients who have a diagnosis of no pulmonary hypertension and a mean pulmonary artery pressure greater than or equal to 25 mm Hg should be less than five per cent. Results Key findings Less than three per cent of patients are given a final diagnosis of no pulmonary hypertension with a mean pulmonary artery pressure greater than or equal to 25 mm Hg. Recommendations Centres need to ensure that the final diagnosis for each patient is correctly recorded and consistent with the right heart catheterization results. Copyright 2017, Health and Social Care Information Centre. 14

15 2.5 How long are patients waiting for a diagnosis once a referral is made? Why is this important? It is understandable that most people who have symptoms want to know what is causing them and find a suitable treatment as soon as possible. Symptoms may get worse while waiting for a diagnosis of pulmonary hypertension. Specialist pulmonary hypertension centres need to ensure that they are seeing their new patients in a timely fashion. Standard to be measured against 95 per cent of patients whose referral letter was received over six months ago should have a diagnosis recorded. Results Key findings Over 90 per cent of patients in the specialist pulmonary hypertension service are receiving a timely diagnosis. Whilst high, this does not meet the agreed standard. Recommendations Centres need to ensure that the diagnosis is being recorded in all patients in a timely manner. Copyright 2017, Health and Social Care Information Centre. 15

16 2.6 Is the first line drug therapy for pulmonary arterial hypertension a phosphodiesterase 5 inhibitor? Why is this important? Pulmonary arterial hypertension can be treated with three different groups of medicines. The NHS England policy requires that a phosphodiesterase 5 inhibitor (sildenafil or tadalafil) is used first on economic grounds. Nevertheless, this drug is contraindicated in some patients. By the policy, the patients who manifest the most severe symptoms (in WHO functional class IV) may be recommended a prostacyclin instead. Standard to be measured against First line drug therapy for pulmonary arterial hypertension should be a phosphodiesterase 5 inhibitor in more than 80 per cent of patients. Results Key findings Almost 90 per cent of patients are receiving the drug therapy required by NHS England. Recommendations Centres need to ensure correct recording of the drug therapy and the date on which patients commence it. Copyright 2017, Health and Social Care Information Centre. 16

17 2.7 How many patients in WHO functional class I (and have never had a higher value) are on pulmonary hypertension drug therapy? Why is this important? NHS England does not permit patients without symptoms to be treated with drugs for pulmonary arterial hypertension. A patient with WHO class II, III or IV is showing symptoms while a patient with a WHO class of I is not showing any symptoms. This standard measures what proportion of patients have never shown any symptoms and yet are on some form of drug therapy. Standard to be measured against Fewer than five per cent of patients with a highest recorded WHO functional class of I or a missing WHO functional class should be on pulmonary hypertension drug therapy. Results Key findings Fewer than five per cent of patients with a highest recorded WHO functional class of I, or a missing WHO functional class, are on pulmonary hypertension drug therapy. Recommendations Centres should ensure the correct recording of WHO functional class in each patient commencing PAH drug therapies. This data should also be recorded during follow-up. Copyright 2017, Health and Social Care Information Centre. 17

18 2.8 What proportion of patients receiving a pulmonary hypertension drug have had a consultation within the last year? Why is this important? Specialist pulmonary hypertension centres provide follow-up for patients in whom they are prescribing specialist drug therapies. It is crucial that the effects of these drug therapies are monitored for adequacy of response to treatment and side effects. This monitoring should be carried out at least annually by prescribers. Note that 13 months is chosen to represent one year so as to allow some variation in the timing of patients who only have an annual appointment with their pulmonary hypertension centre. Standard to be measured against 95 per cent of patients receiving a pulmonary hypertension drug therapy should have had a consultation (inpatient, day case or outpatient) within the last 13 months. Results Key findings Over 90 per cent of patients on drug therapy in the pulmonary hypertension service, are being reviewed within an appropriate timeframe. Whilst high, it is not meeting the agreed standard. Recommendations All centres should aim to review all patients receiving PAH therapy at least annually. Some patients with severe disease and/or frailty in whom no escalation of PAH therapy is deemed appropriate may find it physically challenging to travel to their PAH centre. Copyright 2017, Health and Social Care Information Centre. 18

19 All centres should ensure that when patients are discharged from the service that they close the medication in the database to maintain the quality of the data in the analysis. 2.9 What proportion of patients have had a consultation or been discharged within 30 days of referral? Why is this important? This is a measurement of how quickly more urgent patients are seen by specialist pulmonary hypertension centres following a new referral. Unlike the standard in section 2.5 ( How long are patients waiting for a diagnosis once a referral is made? ) this explores the proportion of patients who have had at least a consultation within a fixed time period. Standard to be measured against 40 per cent of patients should have attended a consultation (inpatient, day case or outpatient) or have been discharged within 30 days of receipt of their first referral. Results Key findings Over 40 per cent of the patients within the specialist pulmonary hypertension service are being seen by a specialist or discharged in a timely manner. Recommendations To improve the timeliness of patients being seen, the threshold for this standard should be raised. Copyright 2017, Health and Social Care Information Centre. 19

20 2.10 What proportion of patients receive a consultation or are discharged within 90 days of their first referral? Why is this important? This measurement complements the standard in section 2.9. It is a measurement of how quickly patients are seen by specialist pulmonary hypertension centres following a new referral. Unlike the standard in section 2.5 ( How long are patients waiting for a diagnosis once a referral is made? ), this explores the proportion of patients who have had at least a consultation within a fixed time period. Standard to be measured against 75 per cent of patients should receive a consultation (inpatient, day case or outpatient) or have been discharged within 90 days of receipt of their first referral. Results Key findings Over 85 per cent of patients are being seen by the specialist pulmonary hypertension service following a new referral. Recommendations To improve the timeliness of patients being seen, the threshold for this standard should be raised. Copyright 2017, Health and Social Care Information Centre. 20

21 2.11 Are patients starting pulmonary hypertension drug therapy in a timely manner? Why is this important? For patients with some types of pulmonary arterial hypertension it is important to start drug treatment in a timely manner to try to avoid deterioration as a result of progressive disease. This measurement of the time it takes from referral to treatment provides an overview of the timeliness of clinical processes. Standard to be measured against 75 per cent of new referrals who have a diagnosis of idiopathic, heritable, anorexigen-induced or connective tissue disease associated PAH and are in WHO functional class II, III or IV, should commence PAH drug therapy within 12 weeks of receipt of their first referral. Results Key findings Over 70 per cent of patients are starting drug therapy in a timely manner. Whilst high, the standard has not been met. Recommendations Centres should review their patient pathway to ensure that patients are managed in a timely manner. Copyright 2017, Health and Social Care Information Centre. 21

22 2.12 How long are patients waiting for a pulmonary endarterectomy once they have been diagnosed? Why is this important? A pulmonary endarterectomy (PEA) is an operation which is performed for the treatment of selected patients with CTEPH. This operation may result in marked clinical improvement for patients. This measurement looks at the time it takes from diagnosis at a specialist pulmonary hypertension centre to surgery at Papworth, the single surgical centre in the UK. Standard to be measured against 90 per cent of patients who undergo a pulmonary endarterectomy should wait less than four months from their diagnosis of chronic thromboembolic pulmonary hypertension. Results Additional information in Appendix 1 shows the number of operations and the median wait time. Key findings The waiting time standard for PEA has not been met. Recommendations Possible reasons for the failure to meet this standard need exploring and should be reviewed by specialised commissioners and the national service. Copyright 2017, Health and Social Care Information Centre. 22

23 2.13 Are patients completing a quality of life questionnaire at least annually? Why is this important? Assessment of the quality of life of patients with pulmonary hypertension is best achieved by asking patients to complete a questionnaire. Changes in quality of life can then be monitored in response to treatment. PHA-UK have championed the introduction of this standard. Standard to be measured against 90 per cent of patients who have at least one consultation (inpatient, day case, or outpatient) in the last year should have at least one quality of life questionnaire recorded in the last year. Results Key findings The proportion of patients completing a quality of life questionnaire falls below the standard. Recommendations This standard needs to be amended in future audits to require questionnaire completion only in patients with PAH or CTEPH, which are the only forms of pulmonary hypertension for which quality of life assessments have been validated. Centres should review their processes to ensure that patients are given the opportunity to complete a questionnaire. Allowance needs to be made for patients who are unable to complete a questionnaire for reasons of comprehension or who refuse. Copyright 2017, Health and Social Care Information Centre. 23

24 3. Referral, Treatment and Outcomes While the focus of this report is upon the agreed standards, this section of the report contains reporting on particular areas of the audit considered of interest, but not incorporated in a standard. This sort of investigation can be the first step towards establishing a standard. 3.1 Referral and treatment rates by country Why is this important? Previous audits have published information showing differences between England, Scotland and Wales. This analysis investigates if those differences are due to differences in the population of the countries or for other reasons. Results Table 4: Referral and Treatment active on the 31 March 2016 by diagnosis approved treatment group by country of residence Active Referral (rate per million) Country Treatment approved Treatment not approved Diagnosis Missing Great Britain England Scotland Wales Active Treatment (rate per million) Country Treatment approved Treatment not approved Diagnosis Missing Great Britain England Scotland Wales Source: NHS Digital Notes: 1. For adults over 20 years of age on 31 March Standardised for age and sex. 3. Rate per million rounded to nearest whole number. 4. Patients are included in 'Treatment approved' if any of their diagnosis codes (main, additional 1, additional 2) are listed in Appendix NHS England Drug Policy approves certain drugs to be used to treat specific diagnoses (Pulmonary Arterial Hypertension, Chronic Thromboembolic Pulmonary Hypertension, Pulmonary Veno Occlusive Disease and/or Pulmonary Capillary Haemangiomatosis, Systemic disorders Sarcoidosis, Other - Chronic renal failure on dialysis). The Treatment approved group is patients whose latest diagnosis contains one or more of these diagnoses. The Treatment not approved group is patients whose latest diagnosis does not contain one or more of these diagnoses. The Diagnosis Missing group is those patients for who no diagnosis has ever been recorded within the audit. Copyright 2017, Health and Social Care Information Centre. 24

25 Key Findings England and Scotland are similar with respect to their active referrals. However Wales seems to have fewer referrals active - in particular where treatment by pulmonary hypertension therapy is not appropriate. Wales has a low rate of active treatment, but given its lower rate of referrals, this is not unexpected. Scotland has a higher rate of therapy usage where the diagnosis indicates therapy is not approved. 3.2 Use of Prostanoid Why is this important? Current guidelines recommend the use of intravenous prostanoids for patients with severe pulmonary arterial hypertension, in particular in WHO functional class IV. However the use of intravenous prostanoids is suitable only in specific clinical conditions and places significant difficulties on patients. This analysis looks at the use of prostanoids by patients who have died. In many circumstances inhaled prostanoids are used before or instead of intravenous prostanoids and as such we have included them in this analysis. Results Table 5: Proportion of patients with use of a Prostanoid, both within one month of death and any use before death, for idiopathic, heritable, or anorexigen-induced Pulmonary Arterial Hypertension between 2009 and 2016, by WHO functional class Class Number of Deaths Use of Prostanoid (any type) within One Month of Death Use of Prostanoid (any type) ever Total 1,291 6% 17% Class IV % 28% Class III 713 2% 10% Class I-II 41 0% 2% Source: NHS Digital Notes: 1. Only for patients within the cohort for longitudinal analysis. 2. This table covers the entire audit period Differences in prescribing patterns between years cannot be excluded. 3. The use of a prostanoid in WHO functional class I or II may indicate that the WHO functional class has not been updated. 4. Full details of the cohort for longitudinal analysis can be found in Appendix 5. Copyright 2017, Health and Social Care Information Centre. 25

26 Key Findings That as according to the guidelines, as the symptoms worsen the rate of prostanoid use increases. The rate of prostanoid use in the month before death is low. It is more common to use prostanoids and then cease using them than to be using them in the month before death. Recommendations Multidisciplinary teams should discuss the use of prostanoids with appropriate patients. The guidelines consider appropriate patients to be patients with pulmonary arterial hypertension in WHO functional class IV or patients with pulmonary arterial hypertension in WHO functional class III who have an inadequate treatment response. Copyright 2017, Health and Social Care Information Centre. 26

27 3.3 Survival Analysis Why is this important? Pulmonary hypertension may be a life shortening condition. One of the aims of treatment is to prolong life as well as to improve its quality. Measurement of survival is used as a clinical outcome measure to assess the effect of treatment in many life threatening diseases. Results Figure 15: Kaplan Meier curves showing the time from first diagnosis to death by latest pulmonary hypertension diagnosis for specific diagnostic groups Copyright 2017, Health and Social Care Information Centre. 27

28 Notes: 1. Only for patients within the cohort for longitudinal analysis. 2. A Kaplan Meier curve is a way of graphically displaying an estimate of what proportion of patients will still be in a cohort a number of days after your chosen start date. For example, it provides an estimate of the percentage of patients that will still be alive a year after they received a specific diagnosis. 3. The results differ from previous years as survival time is measured from diagnosis rather than initial referral, which has been used in previous reports. 4. Portal Hypertension is not calculated beyond three years due to insufficient data. 5. Full details on the patients within the cohort for longitudinal analysis can be found in appendix 5. Key findings The survival curves show variation in survival related to the underlying cause of pulmonary hypertension. Patients with congenital heart disease have often had pulmonary hypertension since childhood and survive longer with pulmonary hypertension than some of the forms acquired in later life. Recommendations Survival should continue to be monitored annually and the duration of the curves should be extended as statistical analysis allows. A comparison of survival between centres was reported to show no difference in the last annual report. Copyright 2017, Health and Social Care Information Centre. 28

29 3.4 Deprivation and pulmonary hypertension Why is this important? We would like to understand what factors determine patient access to the National Pulmonary Hypertension Service. An analysis of data in the Audit s 2014 Annual Report suggested that the distance a patient lived from a specialist centre might play a role in determining whether they were referred or not. A common factor in many diseases is deprivation which in England can be derived from where people live in the country. An index of deprivation has been derived for England and we used this to find out if deprivation plays a role in patient referrals to specialist centres. Details of the deprivation index used can be found at: Results Notes: 1. Directly standardised for age and sex. 2. The dots are the standardised rates. 3. The vertical lines are the 95 per cent confidence intervals, which means we are 95 per cent confident that the true results lie somewhere on that line. 4. The black line is the linear line of best fit. The R squared value is a measure of how much the variation in rate is associated with the level of deprivation. The value of 0.71 indicates that there is a relatively strong association. Copyright 2017, Health and Social Care Information Centre. 29

30 5. Deciles of deprivation are taken from The Indices of Deprivation 2010 created by the Department for Communities and Local Government (DCLG). It is based around the lower super output area and is designed to allow comparison of relative deprivation of an area. Each decile contains 10 per cent of the lower super output areas. Decile 1 is the most deprived while decile 10 is the least deprived. Key Findings The above is evidence that some correlation between deprivation and PAH may exist, namely a higher prevalence of PAH in patients attending pulmonary hypertension specialist centres is found in the areas of higher deprivation. Comparison with the same analysis on CTEPH patients indicates there is a difference between the two diagnosis groups. The results show association, not causation, so caution should be taken in drawing any conclusion. In the 2014 Annual Report, information on the impact of distance from a pulmonary hypertension centre on patients diagnosed with pulmonary hypertension showed there was some impact but it was unclear. Results Notes: 1. Directly standardised for age and sex. 2. The dots are the standardised rates. Copyright 2017, Health and Social Care Information Centre. 30

31 3. The vertical lines are the 95 per cent confidence intervals, which means we are 95 per cent confident that the true results lie somewhere on that line. 4. The line indicates the linear line of best fit, and the R squared value shows how close to that line the points are (a value of 1 would show that the points were exactly on the line). The value of 0.23 indicates that there is a weak association. 5. Deciles of deprivation are taken from The Indices of Deprivation 2010 created by the Department for Communities and Local Government (DCLG). It is based around the lower super output area and is designed to allow comparison of relative deprivation of an area. Each decile contains 10 per cent of the lower super output areas. Decile 1 is the most deprived while decile 10 is the least deprived. Key findings The above is evidence that if any correlation exists between deprivation and CTEPH, it is a weak one. Comparison with the same analysis on PAH patients indicates there is a difference between the two diagnosis groups. Recommendations Investigation into the joint impact of deprivation and distance on PAH should be undertaken. This is an area the Audit could consider in the future. Copyright 2017, Health and Social Care Information Centre. 31

32 Appendix 1 Reference Tables Table 6: Number of patients active on 31 March, by specialist centre Hospital Name Total 4,153 4,688 5,097 5,510 6,000 6,557 7,035 Golden Jubilee Imperial College Papworth Hospital Royal Brompton and Harefield Royal Free ,000 1,092 1,143 1,345 1,536 Sheffield 949 1,090 1,146 1,204 1,458 1,548 1,675 Newcastle Upon Tyne Hospitals Source: NHS Digital Notes: 1. Active patients have a referral active on 31 March of the appropriate year. 2. Years contain data from Northern Ireland. 3. Every centre has seen an increase in the number of patients they are seeing. There has been no major shift in the distribution of patients between 2010 and Table 7: Number of days (median, lower quartile, and upper quartile) between initial diagnosis of chronic thromboembolic pulmonary hypertension and pulmonary endarterectomy operation performed in Number of Operations Lower Quartile Median Upper Quartile Total Notes: 1. Wait times are rounded to the nearest day. Source: NHS Digital Copyright 2017, Health and Social Care Information Centre. 32

33 Table 8: Number of drug prescriptions on 31 March including monotherapy and combination therapy Drug Sildenafil 1,373 1,621 1,936 2,234 2,552 2,866 3,070 Tadalafil Bosentan 960 1,044 1,065 1,056 1, Ambrisentan Sitaxsentan Macitentan Iloprost Treprostinil Epoprostenol CCB for vasoreactive PAH Riociguat Unknown Notes: 1. Unknown includes drug trials. 2. Sitaxsentan was withdrawn from use in late Source: NHS Digital Table 9: Percentage of patients with at least one quality of life (QoL) questionnaire recorded in the last year who had a consultation (inpatient, day case, or outpatient) in for patients with idiopathic, heritable, anorexigen induced Pulmonary Hypertension or CTEPH Percentage QoL completeness for patients with idiopathic, heritable, anorexigen induced Pulmonary Hypertension or CTEPH 82% Source: NHS Digital Notes: 1. This is a modified version of the standard in section 2.13 following feedback from the centres. 2. No score was required in order for the quality of life questionnaire to be completed. Copyright 2017, Health and Social Care Information Centre. 33

34 Table 10a: Reference table for Kaplan Meier curves showing the time from first diagnosis to death for people by latest diagnosis Congenital heart disease Connective tissue Disease Idiopathic, heritable, or anorexigen-induced Years elapsed Patients Survival (%) Patients Survival (%) Patients Survival (%) % 1, % 1, % % % % % % % % % % % % % % % % Left heart disease Lung disease Portal hypertension Years elapsed Patients Survival (%) Patients Survival (%) Patients Survival (%) 0 2, % 1, % % 1 1,561 88% % % 2 1,117 81% % 94 76% % % 54 68% % % % % - - Source: NHS Digital Notes: 1. Only for patients within the cohort for longitudinal analysis. 2. Portal Hypertension is not calculated beyond three years due to insufficient data. 3. Full details on the patients within the cohort for longitudinal analysis can be found in appendix 5. Table 10b: Reference table for Kaplan Meier curves showing the day at which that diagnosis group has 50 per cent mortality Identifier Day on which the cohort has 50% mortality Idiopathic, heritable, or anorexigen-induced 4 years and 86 days Lung Disease 3 years and 123 days Connective Tissue Disease 4 years and 4 days Source: NHS Digital Copyright 2017, Health and Social Care Information Centre. 34

35 Appendix 2 Standards not used in the report A2.1 How many patients with pulmonary hypertension due to left heart disease are receiving pulmonary hypertension drug therapy? Why is this important? Clinical guidelines recommend that patients with left heart disease causing pulmonary hypertension should not be treated with pulmonary hypertension drug therapies. Clinical trials have shown that these patients either do not respond to pulmonary arterial hypertension drug treatment or may even deteriorate while taking it. Standard to be measured against Fewer than 10 per cent of patients with a primary diagnosis of pulmonary hypertension due to left heart disease should be receiving a pulmonary hypertension drug therapy. Results Table 11: Percentage of patients with a diagnosis of pulmonary hypertension due to left heart disease receiving a pulmonary hypertension drug therapy on 31 March 2016 Standard which centres are Percentage not expected to exceed National totals 10% 10% Source: NHS Digital This standard has been moved to the appendix because it does not measure what it was intended to measure. Some centres discharge patients with pulmonary hypertension due to left heart disease, and only retaining those that are being treated with drugs. This can produce a very high percentage against a low level of treatment. Copyright 2017, Health and Social Care Information Centre. 35

36 A2.2 How many patients with pulmonary hypertension due to lung disease are receiving pulmonary hypertension drug therapy? Why is this important? Clinical guidelines recommend that patients with parenchymal lung disease causing pulmonary hypertension should not be treated with pulmonary hypertension drug therapies. Clinical trials have shown that these patients either do not respond to pulmonary arterial hypertension drug treatment or may even deteriorate while taking it. Standards to be measured against Fewer than 10 per cent of patients with a primary diagnosis of pulmonary hypertension due to lung disease should be receiving a pulmonary hypertension drug therapy. Results Table 12: Percentage of patients with a diagnosis of pulmonary hypertension due to lung disease/hypoxia receiving a pulmonary hypertension drug therapy on 31 March 2016 Standard which centres are Percentage not expected to exceed National totals 40% 10% Source: NHS Digital This standard has been removed because it does not measure what it was intended to measure. Some centres discharge patients with pulmonary hypertension due to lung disease, and only retaining those that are being treated with drugs. This can produce a very high percentage against a low level of treatment. Copyright 2017, Health and Social Care Information Centre. 36

37 Appendix 3 World Health Organization (WHO) Functional Class The severity of symptoms are recorded using the World Health Organization (WHO) functional class: Class I: Patients with pulmonary hypertension but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnoea or fatigue, chest pain or near syncope. Class II: Patients with pulmonary hypertension resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnoea or fatigue, chest pain or near syncope. Class III: Patients with pulmonary hypertension resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnoea or fatigue, chest pain or near syncope. Class IV: Patients with pulmonary hypertension with inability to carry out any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnoea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity. This is akin to New York Heart Association functional class. Efforts are made to record a WHO class for all patients at each hospital visit but this is not possible in all cases. Copyright 2017, Health and Social Care Information Centre. 37

38 Appendix 4 Dana Point Classification of Pulmonary Hypertension 1 Pulmonary arterial hypertension (PAH) 1.1 Idiopathic PAH 1.2 Heritable 1.3 Drugs and toxins induced BMPR ALK1, endoglin (with or without hereditary haemorrhagic telangiectasia) Unknown 1.4 Associated with (APAH): Connective tissue diseases HIV infection Portal hypertension Congential heart disease Schistosomiasis Chronic haemolytic anaemia 1.5 Persistent pulmonary hypertension of the newborn 1 Pulmonary veno-occulusive disease and/or pulmonary capillary haemangiomatosis 2 Pulmonary hypertension due to left heart disease 2.1 Systolic dysfunction 2.2 Diastolic dysfunction 2.3 Valvular disease 3 Pulmonary hypertension due to lung diseases and/or hypoxia 3.1 Chronic obstructive pulmonary disease 3.2 Interstitial lung disease 3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern 3.4 Sleep-disordered breathing 3.5 Alveolar hypoventilation disorders 3.6 Chronic exposure to high altitude 3.7 Developmental abnormalities 4 Chronic thromboembolic pulmonary hypertension 5 Pulmonary hypertension with unclear and/or multifactorial mechanisms 5.1 Haematological disorders: myeloproliferative disorders, splenectomy 5.2 Systemic disorders, sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis 5.3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders 5.4 Others: tumoural obstruction, fibrosing mediastinitis, chronic renal failure on dialysis Copyright 2017, Health and Social Care Information Centre. 38

39 Treatment approved diagnoses The codes used in the creation of the treatment approved diagnosis group in Table 4 are as follows: 1 Pulmonary arterial hypertension (PAH) 1 Pulmonary veno-occulusive disease and/or pulmonary capillary haemangiomatosis 4 Chronic thromboembolic pulmonary hypertension Systemic disorders - Sarcoidosis* Other - Chronic renal failure on dialysis* * indicates a code used only in the Pulmonary Hypertension Audit. The classification system used in the Pulmonary Hypertension audit has additional codes to help with clarity and data quality. Copyright 2017, Health and Social Care Information Centre. 39

40 Appendix 5 The cohort for longitudinal analysis To support certain analyses, a cohort of patients has been created which is defined as those: whose first referral was on or after 1 April This cohort is used when there is a need to analyse data where the full treatment history for a patient is required. Since the Audit started on 1 April 2009, it is reasonable to expect that all of the events after that date will be recorded. Scottish data is submitted to the Audit using a pseudonymised patient identifier (NHS Digital cannot identify a patient, however the organisation that supplied the data can), so it is not possible to identify patients who have been treated in both Scotland and England. This means that a small number of patients may be included twice in the cohort for longitudinal analysis, once for the English recorded activity, and once for the Scottish. Copyright 2017, Health and Social Care Information Centre. 40

41 Appendix 6 Members of staff involved from the pulmonary hypertension centres Golden Jubilee National Hospital Imperial College Healthcare NHS Trust Papworth Hospital NHS Foundation Trust Royal Brompton and Harefield NHS Foundation Trust Royal Free London NHS Foundation Trust Sheffield Teaching Hospitals NHS Foundation Trust The Newcastle upon Tyne Hospitals NHS Foundation Trust Lead Clinician Andrew Peacock Simon Gibbs Joanna Pepke- Zaba John Wort* Gerry Coghlan* David Kiely* Paul Corris Supporting Physicians Martin Johnson Colin Church Luke Howard Rachel Davies Nick Morrell Karen Sheares Dolores Taboada-Buasso Mark Toshner John Cannon Michael Gatzoulis* Kostas Dimopoulos* Laura Price* Aleksander Kempny* Colm McCabe* Clive Handler* Benji Schreiber* Dan Knight* Phil Marino* Hossam Fayed* Charlie Elliot* Robin Condliffe* Ian Sabroe Anthanasios Charalampopoulos * James Lordan Andrew Fisher Guy MacGowan Surgeons (PEA) David Jenkins John Dunning Steven Tsui Choo Ng Nurses Agnes Crozier Karon Carson Val Irvine* Wendy Gin- Sing Chantal Torpy Eilish Lawlee Natalie Doughty* Sam Clare* Sue Mepham* Anie Ponnaberanam* Maxine Leitch* Nicola Speed* Carl Harries* Joana Barbosa* Helen Moorshead* Sally Reddecliffe* Adele Dawson* Iain Armstrong (Nurse Consultant) Nicola Paterson Jane Wilkinson Sian Garrad Alan Greenhalgh* Deborah Shipley* Rachel Crackett* Margaret Day Julia de Soyza Data Staff Simon Kerridge* George Villa* Tom Devine* Sweeta Dhakan* Della Stokes* Anthony Piwowarski Sheila Forshaw* Copyright 2017, Health and Social Care Information Centre. 41

42 Golden Jubilee National Hospital Imperial College Healthcare NHS Trust Papworth Hospital NHS Foundation Trust Royal Brompton and Harefield NHS Foundation Trust Royal Free London NHS Foundation Trust Sheffield Teaching Hospitals NHS Foundation Trust The Newcastle upon Tyne Hospitals NHS Foundation Trust Pharmacist Allan Smith Lynn Humphrey Duncan Grady Beejal Shah (mat leave) Gita Shah (mat cover) Layla Khatib Shalina Dhalla Neil Hamilton* Michaela Sellars* Mark Graves* Maria Allen Radiologists Michael Sproule Ben Ariff Mary Roddie James Jackson Nick Screaton Deepa Gopalan Michael Rubens Simon Padley Jamanda Haddock Olga Lazoura Catherine Hill Andy Swift Rajaram Smitha Leslie Mitchell Michelle Muller Service Manager Lynne Ayton Graham Lomax Alison Gibson Vanessa Saberton-Smith Lawrence Mac Lisa Rose Robert Grimshaw Liz Bailey IT Staff David Wilcox Jim Butler Gary Cowell Himanshu Patel Daniel Roberts* Admin Staff Veronica Ferry Kirsty Menzies Lianne Campbell* Lucia Heath Jermaine Charles Chiu-Yee Liu Karin Johnson* Tanya VonSeld Christine Butler* Naomi Fitzgerald* Jane Fisher Selina Iuliano Sarah Page* Joanna Mash* Marilda Maizga* Stacey Hunter Amanda Poirrier* Denise Stephenson Amanda Haythorne* Rebecca Charlesworth* Linda Houlis *indicates staff who entered data Copyright 2017, Health and Social Care Information Centre. 42

43 Appendix 7 Members of the National Audit of Pulmonary Hypertension Project Board Name Role Organisation Simon Gibbs (Chair) David Kiely Kathy Blacker Sian Lewis Clinical Lead and Chief Auditor (Project executive) Senior User (Physician) Senior User (Commissioners) Senior User (Commissioners) Imperial College London and Imperial College Healthcare NHS Trust Chair, Pulmonary Hypertension CRG, NHS England Regional Programme of Care Manager Internal Medicine (North), NHS England Acting Medical Director, Welsh Health Specialised Services Committee Mike Winter Senior User (Commissioners) Medical Director, NHS National Services Scotland Paul Corris Senior User (Physician) Service Review of PH Centres Gerry Coghlan Senior User (Physician) Chair of PH Physicians Committee Natalie Doughty Senior User (Nursing) Papworth NHS Foundation Trust Sean Gaine Senior User (Physician) Mater Hospital, Dublin, Ireland Martin Johnson Senior User (Physician) NHS Greater Glasgow & Clyde Shahin Moledina Senior User (Physician) Paediatric Pulmonary Hypertension Service Kay Yeowart Senior User (Patients) Pulmonary Hypertension Association UK Julie Michalowski Project Manager NHS Digital Alyson Whitmarsh Clinical Audit Programme Head NHS Digital Copyright 2017, Health and Social Care Information Centre. 43

44 ISBN This publication may be requested in large print or other formats. Published by NHS Digital, part of the Government Statistical Service NHS Digital is the trading name of the Health and Social Care Information Centre. Copyright 2017 You may re-use this document/publication (not including logos) free of charge in any format or medium, under the terms of the Open Government Licence v3.0. To view this licence visit or write to the Information Policy Team, The National Archives, Kew, Richmond, Surrey, TW9 4DU; or Copyright 2017, Health and Social Care Information Centre. 44