National Audit of Pulmonary Hypertension, 13th Annual Report

Measurement of clinical practice against professional clinical standards is a key principle of clinical audit. Once comparison to standards has taken place a clinical team can then develop local action plans for any changes that have been identified as necessary in order to improve their PH service.

Targets and standards are reviewed annually by the Pulmonary Hypertension Outcomes Group (PHOG).

Standards are applicable to all specialist PH centres, although they do not provide identical services. Thresholds for standards are not set to 100% because the targets do not take every factor into account. Care may be given that is not in line with the standard without indicating it was incorrect or poor practice. Specialist PH centres should consider whether this is the case when reflecting on their results.

As in the previous NAPH report, results from previous years have been updated using:

1. the latest available information in the NAPH

All results in this report are derived from data extracted on 20 September 2022, meaning that results for earlier audit years (2016-17 to 2020-21) included in this report will sometimes differ from those published in previous reports.

Why has a new extract been used? It is important to take the latest and most accurate cut of the NAPH data. NAPH is a continuous collection and PH centres are able to add, amend and delete information about patients used in earlier cohorts.

2. the latest methodology

Methodology is constantly reviewed and revised if required. Details of methodological changes to the National Standards are in the next section below. The updated methodology has been applied to all audit years in the report.

Why have earlier years been updated with the new methodology? To maintain the internal consistency of this report, it is important that the same methodology is applied across all audit years in the 13th Annual Report publication.

3. the latest targets

Target levels are constantly reviewed and revised if required. Changes to the targets for the 13th Annual Report are outlined in the next section below, with updated targets applied to all audit years in the report.

Why have earlier years been updated with the new targets? To maintain the internal consistency of this report, it is important that the targets shown in the 13th Annual Report tables are consistent across all audit years.

Following the above changes, the figures produced for the 13th Annual Report supersede those previously published.

Table NSS 1: National Standards: Summary by audit year, Great Britain, 2016-22

Targets:

Trends:

Notes:

* = Fewer than 10 cases in the denominator.

♦ = Identified by PHA UK in 2016 as the 6 most important NAPH National Standards.

$ = Change to target/definition (see Methodology).

N/A = Not applicable.

1. Variation between audit years can occur in a ‘static’ trend. Where fewer cases are involved, a greater degree of variation is possible within a ‘static’ trend.

Table NSS 2: National Standards: Summary by PH centre, Great Britain, 2021-22

Targets:

Notes:

* = Fewer than 10 cases in the denominator.

♦ = Identified by PHA UK in 2016 as the 6 most important NAPH National Standards.

$ = Change to target/definition (see Methodology).

N/A = Not applicable.

Audit standard

All specialist PH centres are expected to participate (1) in the National Audit of Pulmonary Hypertension (2).

Why is this important?

Participation in the 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.

The impact of COVID-19

Due to the COVID-19 pandemic, PH centres and the National Audit Project Board elected to present, but not assess, data at PH centre level.

Results

Despite the pressures caused by the COVID-19 pandemic, all 8 specialist PH centres participated in the 2021-22 Audit.

Notes:

1. PH centres will determine if they are participating. Failure to participate will remove them from all other standards. For a full list of PH centres, see: Glossary: Specialist PH centres

2. The NHS England PH service specification A11/S/a 2013-14 states that “All designated PH centres will be required to submit data to the National Pulmonary Hypertension Service” (page 12) which is assumed to be a reference to the Audit.

Audit standard

Adult specialist PH centres (1) are expected to manage at least 300 patients per annum with pulmonary arterial hypertension (PAH) and / or chronic thromboembolic pulmonary hypertension (CTEPH) (2,3).

The children’s specialist PH centre (1) is expected to manage at least 300 patients per annum, of whom 100 patients per annum with PAH or CTEPH (2).

Why is this important?

Evidence from other disease areas suggests that best outcomes for patients are obtained in high-volume PH centres. High-volume PH 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 PAH or CTEPH. The recommended number of patients seen is based on European guidelines which are used by the UK specialist PH centres.

Notes:

1. Adults refers to patients under the care of adult PH centres. Children are under the care of Great Ormond Street.

2. An open referral at any point in the year will constitute 'manage' within this context. An open referral indicates patients attending a specialist PH centre who include existing patients as well as new referrals. The latest diagnosis will determine PAH or CTEPH. 

3. This standard is derived from ESC/ERS guidelines with the number of patients adjusted to the NHS England PH service specification A11/S/a 2013-14.

Table NS 2: PH centres meeting National Standard 2 Great Britain, 2016-22

Key:

Notes:

1. Definition: Adult specialist PH centres are expected to manage at least 300 patients with PAH or CTEPH in a year.

2. Definition: The children’s specialist PH centre is expected to manage at least 300 patients in a year, including 100 patients with PAH or CTEPH.

Audit standard

95% of patients whose referral letter was received over 6 months ago should have a diagnosis recorded (1).

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 whilst waiting for a diagnosis of pulmonary hypertension. Specialist PH centres need to ensure that they are seeing their new patients in a timely fashion.

Table NS 3: Proportion of cases meeting National Standard 3, Great Britain, 2016-22

Key:

Notes:

N/A = Not applicable.

1. Definition: 95% of patients whose referral letter was received over 6 months ago should have a diagnosis recorded.

Audit standard

50% 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 (1).

Why is this important?

This standard measures how quickly patients are seen by specialist PH centres following a new referral. In some patients, the symptoms of PH may progress between referral and the first consultation in a PH centre so it is desirable that patients are seen promptly.

Notes:

1. The NHS England PH service specification A11/S/a 2013-14 states that “There will be minimal delay between referral to a designated PH centre and an outpatient consultant appointment. All patients referred urgently will be able to see a specialist within 1 month of the PH centre receiving their referral and those who are severely symptomatic will be seen within 2 weeks.” (page 4). The Audit does not distinguish elective and urgent referrals so this proportion is arbitrary.

Table NS 4a: Proportion of cases meeting National Standard 4a, Great Britain, 2016-22

Key:

Notes:

N/A = Not applicable.

1. Definition: 50% 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.

Table NS 4b: Proportion of cases meeting National Standard 4b, Great Britain, 2018-22

Key:

Audit standard

95% of adult patients receiving a pulmonary hypertension drug therapy (excluding patients diagnosed as pulmonary arterial hypertension associated with congenital heart disease (1)) should have a cardiac catheterisation recorded, either before or up to 2 weeks after treatment begins.

Why is this important?

Cardiac catheterisation (2) is considered the best method for confirming the presence, nature and severity of pulmonary hypertension.

It is reasonable to be certain about the diagnosis before starting treatment.

Notes:

1. Some PH centres do not routinely undertake cardiac catheterisation in patients with Eisenmenger’s syndrome.

2. Cardiac catheterisation is an invasive diagnostic examination of the heart. PHA UK: Cardiac catheterisation

Table NS 5: Proportion of cases meeting National Standard 5, Great Britain, 2016-22

Key:

Notes:

N/A = Not applicable.

1. Definition: 95% of adult patients receiving a pulmonary hypertension drug therapy (excluding patients diagnosed as pulmonary arterial hypertension associated with congenital heart disease) should have a cardiac catheterisation recorded before or up to 2 weeks after treatment begins.

Audit question

90% of adult patients with PAH / CTEPH should have a WHO functional class (1) and exercise test (2) (6-minute walk or incremental shuttle) recorded before they begin any pulmonary hypertension drug therapy.

Why is this important?

The World Health Organization (WHO) functional class (1) 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. Both WHO functional class and exercise performance may improve with treatment and by recording this information a response to treatment can be documented later.

Notes:

1. See Glossary: WHO functional class for definitions.

2. The exercise test distance should be recorded as zero if the patient is unable to perform the test for reasons of pulmonary hypertension. From 1 April 2017 the reason for not performing an exercise test can be recorded in the database.

Table NS 6a: Proportion of cases meeting National Standard 6a, Great Britain, 2016-22

Key:

Notes:

N/A = Not applicable.

1. Definition: 90% of adult patients with PAH / CTEPH should have a WHO functional class and an exercise test (6-minute walk or incremental shuttle) recorded before they begin any pulmonary hypertension drug therapy.

Table NS 6b: Proportion of cases meeting National Standard 6b, Great Britain, 2020-22

Key:

Notes:

N/A = Not applicable.

1. Definition: 90% of adult patients with PAH / CTEPH should have a WHO functional class recorded before they begin any pulmonary hypertension drug therapy.

Audit standard

80% of adult patients who have idiopathic, heritable or drug-induced pulmonary arterial hypertension (IPAH) should have undergone a vasoreactivity study before starting PAH drug therapy.

Why is this important?

The information provided by undergoing a vasoreactivity study determines whether the patient with IPAH is most likely to respond to treatment with calcium channel blockers in preference to other drug therapies including prostanoids, endothelin antagonists or PDE5 inhibitors.

Notes:

1. Definition: 80% of adult patients who have idiopathic, heritable or drug-induced PAH should have undergone a vasoreactivity study before starting PAH drug therapy.

Table NS 7: Proportion of cases meeting National Standard 7, Great Britain, 2018-22

Key:

Notes:

* = Fewer than 10 cases in the denominator.

N/A = Not applicable.

1. Definition: 80% of adult patients who have idiopathic, heritable or drug-induced PAH should have undergone a vasoreactivity study before starting PAH drug therapy.

Audit standard

80% 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 (1).

Why is this important?

For patients with some types of PAH 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.

Notes:

1. This standard is based on the NHS England PH service specification A11/S/1 2013-14: “All new patients will complete all investigations required to make a diagnosis and to determine a treatment plan and commence drug therapy within 12 weeks of the referral being received by the designated PH centre. Evidence shows that waiting beyond 12 weeks can compromise survival.” (page 4).

Table NS 8: Proportion of cases meeting National Standard 8, Great Britain, 2016-22

Key:

Notes:

* = Fewer than 10 cases in the denominator.

N/A = Not applicable.

1. Definition: 80% 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.

2. No patients applicable.

Audit standard

99% of adult patients receiving a pulmonary hypertension drug therapy should have a pulmonary hypertension diagnosis recorded.

Why is this important?

Specialist pulmonary hypertension drugs should only be prescribed for selected patients with pulmonary hypertension.

It is important to confirm that a patient, for whom a drug is prescribed, has a qualifying diagnosis.

Table NS 9: Proportion of cases meeting National Standard 9, Great Britain, 2016-22

Key:

Notes:

N/A = Not applicable.

1. Definition: 99% of adult patients receiving a pulmonary hypertension drug therapy should have a pulmonary hypertension diagnosis recorded.

Audit standard

First line drug therapy for pulmonary arterial hypertension should be a phosphodiesterase 5 (PDE5) inhibitor in 80% or more of adult patients, excluding those patients whose first drug therapy was a calcium channel blocker.

Why is this important?

Pulmonary arterial hypertension can be treated with 3 different groups of medicines. The NHS England policy (1) requires that a PDE5 inhibitor (2) (sildenafil or tadalafil) is used first on economic grounds.

Nevertheless, this drug is contraindicated (3) in some patients. According to NHS England policy, the patients who manifest the most severe symptoms (in WHO functional class IV) may be recommended a prostacyclin as first line therapy instead.

Notes:

1. The NHS England Clinical Commissioning Policy A11/P/b 2014 states that “Monotherapy with an oral PDE5I will be routinely commissioned as first line therapy.” (page 12).

2. Phosphodiesterase 5 (PDE5) inhibitors inhibit the enzyme PDE5, leading to relaxation of the blood vessels and increased blood flow.

3. Medical advice that suggests a particular drug or treatment should not be used in some circumstances.

Table NS 10: Proportion of cases meeting National Standard 10, Great Britain, 2016-22

Key:

Notes:

N/A = Not applicable.

1. Definition: First line drug therapy for pulmonary arterial hypertension should be a phosphodiesterase 5 (PDE5) inhibitor in more than 80% of adult patients, excluding those patients whose first drug therapy was a calcium channel blocker.

Audit standard

90% of adult patients who have PAH or CTEPH and who have at least 1 consultation (inpatient, day case, or outpatient) in the last year should have at least 1 quality of life (QoL) questionnaire recorded in the last year. 90% of adult patients who have a quality of life recorded should have a score recorded.

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. It essential that an emPHasis-10 score is recorded every time an emPHasis-10 questionnaire (typical QoL questionnaire used for PH) is undertaken.

Table NS 11a: Proportion of cases meeting National Standard 11a, Great Britain, 2016-22

Key:

Notes:

N/A = Not applicable.

1. Definition: Definition: 90% of adult patients who have PAH or CTEPH and who have at least 1 consultation (inpatient, day case, or outpatient) in the last year should have at least 1 quality of life questionnaire recorded in the last year.

Table NS 11b: Proportion of cases meeting National Standard 11b, Great Britain, 2019-22

Key:

Notes:

N/A = Not applicable.

1. Definition: 90% of adult patients who have a quality of life questionnaire recorded should have a score recorded.

Audit standard

95% of patients receiving a pulmonary hypertension drug therapy should have had a consultation (inpatient, day case or outpatient (1)) within the last 13 months (2).

Why is this important?

Specialist PH centres provide follow-up for patients for 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 1 year so as to allow some variation in the timing of patients who only have an annual appointment with their PH centre.

Notes:

1. As for previous annual reports, all consultation types (including non-face to face) are classed as meeting the standard.

2. This standard is based on the NHS England PH service specification A11/S/a 2013-14: “Patients treated with disease targeted therapy will have lifelong follow-up within the PH service. The PH centre will identify those patients suitable for shared care and ensure effective communication with shared care PH centres to plan patient reviews. All such patients will be reviewed at least once each year by the visiting PH specialist or at the PH centre.” (page 6).

Table NS 12: Proportion of cases meeting National Standard 12, Great Britain, 2016-22

Key:

Notes:

N/A = Not applicable.

1. Definition: 95% of patients receiving a pulmonary hypertension drug therapy should have had a consultation (inpatient, day case or outpatient) within the last 13 months.

Audit standard

90% of adult patients who undergo a pulmonary endarterectomy (PEA) should have waited less than 4 months from diagnosis of CTEPH by the multidisciplinary team (MDT) meeting at the specialist PH centre (1).

Why is this important?

PEA is an operation which is performed for the treatment of selected patients with CTEPH. This may result in marked clinical improvement for patients.

This measurement looks at the time it takes from diagnosis at a specialist PH centre to surgery at Royal Papworth, the single PEA centre in the UK.

Notes: 1. See Glossary: Waiting times for pulmonary endarterectomy: Background.

Table NS 13: Proportion of cases meeting National Standard 13, Great Britain, 2016-22

Key:

Notes:

* = Fewer than 10 cases in the denominator.

N/A = Not applicable.

1. Definition: 90% of adult patients who undergo a PEA should have waited less than 4 months from diagnosis of CTEPH by the MDT meeting at the pulmonary hypertension specialist PH centre.

2. See Glossary: Waiting times for pulmonary endarterectomy: Background.

Audit standard

80% of adult patients with CTEPH who are to be treated by balloon pulmonary angioplasty (BPA) should undergo their first BPA procedure within 18 weeks of the BPA multidisciplinary team (MDT) meeting decision to perform BPA.

Why is this important?

This measurement looks at the time it takes from decision to treat by the MDT to surgery at Royal Papworth, the single BPA centre in the UK. BPA may result in marked clinical improvement for patients who are not eligible for surgery or who have recurrent or persistent pulmonary hypertension following pulmonary endarterectomy (PEA) (1).

The procedure involves inserting a very fine wire into blood vessels in the lungs, guiding a tiny balloon into position. The balloon is inflated, to around the size of a pea, for a few seconds to push the blockage aside and restore blood flow to the lung tissue. The balloon is then deflated and removed. This is usually repeated on several separate visits.

Notes:

1. Lang I and others. ‘Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension’ European Respiratory Review 2017; 26: 160119.

2. Adapted from Royal Papworth Hospital: Balloon pulmonary angioplasty (BPA) service.

Table NS 14: Proportion of cases meeting National Standard 14, Great Britain, 2019-22

Key:

Notes:

* = Fewer than 10 cases in the denominator.

N/A = Not applicable.

1. Definition: PH centres should ensure that 80% of patients with CTEPH who are to be treated by BPA should undergo their first BPA procedure within 18 weeks of the BPA MDT meeting decision to perform BPA.

Audit standard

PH centres should ensure that 20% of patients are given the opportunity to participate in research related to pulmonary hypertension, including biobank submissions, randomised clinical trials (including interventions) and prospective studies.

Why is this important?

Research is key to improving our understanding of PH, developing new therapies and identifying priorities for people affected by PH.

Biobanks collect biological samples, genetic data, and clinical data for use by PH researchers.

Randomised clinical (or controlled) trials divide participants into separate groups to test a specific intervention. This might include choice of drug, type of intervention or treatment approach. For trials involving new drug treatments only 1 of the groups has the treatment being tested. The other group (the comparison or control group) receives dummy (placebo) treatment. This is usually given in addition to treatments the patient is already on. Using a randomised control group reduces the chance of bias in the research by providing a neutral comparison.

Prospective studies recruit participants to assess the impact of treatments over a long period of time, typically months or years.

Table NS 15: Proportion of cases meeting National Standard 15, Great Britain, 2019-22

Key:

Notes:

N/A = Not applicable.

1. Definition: PH centres should ensure that 20% of patients participate in research related to pulmonary hypertension, including biobank submissions, randomised clinical trial (including interventions) and prospective studies.