A sincere thanks to the 150+ who attended 'Act on PAH: Improve patient outcomes with early diagnosis' in London on the 17th November 2017.

We hope that those of you who attended found the meeting of value and were inspired to take action to identify and refer patients with suspected pulmonary arterial hypertension (PAH) to a specialist centre earlier.

Act on PAH was an interactive, educational meeting focused on reducing the time to diagnosis of patients with suspected PAH, a rare, but serious, condition that can be fatal without treatment.1 Evidence suggests that early diagnosis and intervention can improve patient outcomes,2–5 however almost half of patients wait over a year after first experiencing symptoms before receiving a diagnosis.6

The Act on PAH programme was developed in collaboration with an expert faculty of pulmonary hypertension (PH) specialists from the UK adult PH service and the patient organisation, PHA UK. The multidisciplinary meeting was attended by physicians and nurses involved in the management of breathless patients and healthcare professionals involved in echocardiography (echo) screening.



"Diagnosing patients early is very important… Early intervention results in better outcomes, and there is growing evidence to support this."

Prof. David Kiely, Sheffield Pulmonary Vascular Disease Unit



UK and Ireland Adult Specialist PH Centres7
(click to view full image)


Dr Simon Gibbs (Co-Chair) and Prof. David Kiely (Co-Chair)

Dr Iain Armstrong, Dr John Cannon, Dr Athanasios Charalampopoulos, Dr Colin Church, Dr Gerry Coghlan, Dr Robin Condliffe, Prof. Paul Corris, Rachel Crackett, Dr Rachel Davies, Wendy Gin-Sing, Duncan Grady, Dr Luke Howard, Dr Martin Johnson, Dr Dan Knight, Dr Andrew Swift, Dr John Wort and Prof. Janelle Yorke.




The impact of living with PAH was central to the meeting, and we heard directly from patients and carers, who provided insights into the significant impact that PAH has on their daily lives, and their experience of the often convoluted, pre-diagnostic journey,6 highlighting the importance of early action in managing this disease.


"The delay in diagnosis has a significant impact on a patient. During this period, you’ve got a patient who’s really anxious because their burden of symptoms is increasing. They may have seen four or five specialist medics. By the time you see them at a specialist centre the patient has lost a lot of confidence in healthcare."

Dr Iain Armstrong, Chair of PHA UK


Questions being answered during the panel discussion
(Left to right: Dr John Wort, Dr Robin Condliffe and Dr Andrew Swift)


Delegates chose to attend two in-depth workshops during the day. The workshops were highly interactive and allowed for plenty of discussion with the experts. The key themes from each workshop are discussed below.


Echo masterclass

This workshop examined the echocardiographic signs of PH and discussed the practicalities and common pitfalls in estimating pulmonary arterial pressure. Several interesting cases were presented, including PAH due to congenital heart disease, chronic thromboembolic pulmonary hypertension (CTEPH), idiopathic PH, and PH due to an atrial septal defect. The session highlighted the importance of not only performing echo early in the investigation of breathless patients, but ensuring it is done with the correct technique to allow accurate interpretation of the results.

Diagnostic MDT at the specialist centre: Interactive patient cases

Two case-based workshops running in parallel discussed a number of interesting cases to illustrate the patient types typically referred to a PH centre. Attendees voted on what management intervention they would do next and the imaging was examined and discussed. The usefulness of CT scans as an additional tool to echo was considered, as well as the difficulties in diagnosing and treating patients with PAH and co-morbid lung disease. These sessions highlighted the importance of referring suspected PH patients to the specialist centre and the use of the guidelines in taking early and effective action on suspected PAH.

Understanding the patient experience

This workshop was developed in collaboration with PHA UK and highlighted key patient-centred research findings. A patient and carer panel provided insight into the day-to-day impact of living with PAH and how individual patients’ experience of their condition can differ. The audience heard how the patient’s pre-diagnostic experience can result in psychological consequences for both them and their family, and influence their satisfaction with subsequent care. The workshop brought to light the impact of PAH on the caregiver in addition to the patient, and emphasised the importance of addressing both their needs in the management of the disease.

DETECTion of PAH in systemic sclerosis

This workshop debated the optimal methods of screening for PAH in asymptomatic patients with systemic sclerosis (SSc). The DETECT screening method8 was compared with the use of upfront echo in combination with other parameters such as pulmonary function testing. Following a passionate debate, the audience were split with a 50/50 vote, with the key message being that although no currently available approach is perfect, screening asymptomatic SSc patients for PAH can offer the prospect of early diagnosis and improved patient outcomes.

Managing the needs of the PAH patient

This workshop focused on the multidisciplinary, holistic care of patients with PAH, including the impact of co-morbidities. Cases were used to help demonstrate considerations in ongoing management, such as problems with treatment adherence, recognising and managing deterioration, and end-of-life care. We heard how shared decision-making with patients and carers is essential before starting intravenous therapy due to the burden of this intensive treatment. The key message of the session was that in order to achieve optimal care for patients, early referral is key and collaboration between the local and specialist centre is important for ongoing holistic support. If in doubt, contact the specialist centre!


Delegate feedback after the meeting


For those who attended Act on PAH, we hope that the meeting has inspired you to take action to identify and refer patients with suspected PAH to a specialist centre earlier.

Please check back to this site to access PH information, tools, expert articles and case studies which will be made available over the coming weeks and months. Or join our mailing list to receive updates on the Act on PAH website and future events aimed at reducing the time to diagnosis for PAH patients.

In November, PHA UK’s PH Awareness Week helped raise awareness of this rare condition. Visit for more information or view on Twitter using tags #BreathlessNotVoiceless and #PHWeek17.

2018 Meetings Check back here for future meetings across the UK


  1. D'Alonzo G, et al. Ann Intern Med 1991;115:343–49
  2. Humbert M, et al. Circulation 2010; 122:156–163
  3. Humbert M, et al. Eur Respir J 2010; 36:549–555
  4. Condliffe R, et al. Am J Respir Crit Care Med 2009;179:151–157
  5. Dimopoulos K, et al. Circulation 2010;121:20–25
  6. Pulmonary Hypertension Association UK (2017). What it means to live with PH today. PHA UK. Available from: . Accessed December 2017
  7. Pulmonary Hypertension Centres. Available from: . Accessed December 2017
  8. Coghlan JG, et al. Ann Rheum Dis 2014;73:1340–9

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