In conversation with Dr Joanna Pepke-Zaba: pulmonary hypertension

Having recently added a European Respiratory Society lifetime achievement award to her career successes, Dr Joanna Pepke-Zaba, consultant respiratory physician at Royal Papworth Hospital, talks to Saša Janković about the national chronic thromboembolic pulmonary hypertension service and her continuing commitment to this ever-evolving field of research and treatment.

Dr Joanna Pepke-Zaba graduated from Warsaw University School of Medicine in Poland before undertaking a fellowship in respiratory physiology at Royal Papworth and Addenbrooke’s Hospitals, University of Cambridge which resulted in a PhD.

Director of the Pulmonary Vascular Diseases Unit (PVDU) at Royal Papworth Hospital from 2003-19, she is currently a consultant respiratory physician at the hospital and has been instrumental in progressing many of the unit’s services over the years, as well as making significant contributions to research in pulmonary hypertension.

Dr Pepke-Zaba is also committed to training respiratory physicians and was a recognised teacher by the Clinical School of the University of Cambridge. As an honorary senior visiting fellow and affiliated associate professor of this institution since 2011, she has been able to run successful translational research programmes in the field of pulmonary hypertension.

With over 200 published papers, and an impressive h-index of 61, she’s certainly well positioned to be an inspirational mentor and support the next generation in elevating patient care in this fast-evolving field.

Patient and colleague connections

Citing a desire to develop ‘very close communication with the patient’, as well as ‘the possibility of doing applied research’ as her main reasons for specialising in respiratory medicine – and pulmonary hypertension in particular – Dr Pepke-Zaba says her early career in general internal medicine gave her ‘time to concentrate’ on choosing this specialism.

‘I wanted to be sure that this was the area which I would like to persevere with, and definitely the people I came across also encouraged me and pointed me towards areas for research’, she says.

She herself has consistently mentored postgraduate students, with most people working in pulmonary hypertension centres in the UK who have spent time at Papworth having been taken under her wing at one time or another, either as a registrar or as research fellows.

‘Mentors definitely played a significant role in my decision, and I think it’s very important to highlight that to stimulate junior doctors ]and demonstrate that] it’s vital to have mentoring alongside good clinical practice,’ she stresses.

Dr Pepke-Zaba’s objective of building strong relationships with patients hasn’t let up either. She is now leading the Pulmonary Hypertension Global Patients Survey in collaboration with patient associations across the world and Pulmonary Vascular Research Institute (PVRI).

This project, with over 3,700 responders, will provide the pulmonary hypertension clinical community with patient perspectives of their journey through the disease in order to help shape future research and enhance healthcare to benefit patients on a global scale.

Pioneering pulmonary projects

As part of Cambridge University’s Medical School faculty, the PVDU at Royal Papworth Hospital is an internationally recognised centre for the investigation and management of pulmonary hypertension, with an established programme of clinical and basic science research.

It is a member of the European Reference Networks (ERN-Lung) and part of the working group of clinical research collaboration between the European Respiratory Society (ERS) and ERN-Lung, as well as the UK National Pulmonary Hypertension Service.

Based at the unit, Dr Pepke-Zaba’s main research has concentrated on the translational programmes in the field of pulmonary hypertension with specific interest in chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary arterial hypertension.

She was instrumental in developing the UK’s national CTEPH programme in the mid-1990s at Papworth, which remains the UK’s only NHS centre commissioned to provide pulmonary endarterectomy (PEA) surgery and balloon pulmonary angioplasty (BPA).

‘At the beginning of the millennium, there were a lot of colleagues who, at that time, were very passionate about pulmonary hypertension, but there was no organisation behind it,’ says Dr Pepke-Zaba regarding the genesis of the programme.

‘We knew from programmes for other rare diseases like cystic fibrosis that if delivery can improve over a curve then commissioners will show an interest in developing a service, so we started to establish designated pulmonary hypertension centres in the UK, including at Papworth, and began introducing the pioneering surgery for CTEPH patients.’

Patients supporting patients

As Papworth is the only centre that can carry out certain CTEPH procedures, it collaborates with other respiratory and cardiology teams across the country, as well as other pulmonary hypertension centres, who refer patients for consultation with Dr Pepke-Zaba’s multidisciplinary team (MDT) to assess their suitability for PEA surgery or BPA.

Since PEA surgery is a major operation and the level of risk versus benefit will vary from person to person, patients are invited to an all-day outpatient appointment to meet not only members of the MDT but also, if they wish, a patient who has already undergone the procedure. This means that they can ask them questions and make more informed choices about their own treatment.

‘Patients who have had the surgery tell us that they are really grateful to return to normal functioning so we really value the support that some of them volunteer to give to those who are considering the procedure,’ says Dr Pepke-Zaba. ‘But it’s not only about the surgery, it’s also relevant for people facing treatment for pulmonary hypertension as all the complex therapy with continuous intravenous infusion is very challenging.

‘Patients want to be put in touch with others who have been or are in that sort of clinical situation. We make sure we do everything we can to make the patient a partner in their discussions, since the idea is to make them better and to improve not only survival but also quality of life.’

As a result of this patient-centric approach, and Papworth’s expertise in pulmonary hypertension, the UK is considered ‘the best organised country in the world’ for these procedures, according to Dr Pepke-Zaba. This knowledge has subsequently been shared with clinical colleagues across Europe and seen Germany and France in particular become similarly proficient, she adds.

Continuing challenges in pulmonary hypertension

Dr Pepke-Zaba’s long-standing contributions to this field have garnered her a trio of lifetime achievement awards from the pulmonary circulation division of the Polish Cardiac Society, the PVRI, and, most recently, the ERS Lifetime Achievement Award in Pulmonary Vascular Diseases in October 2023.

Thankfully for her patients and colleagues, these distinguished accolades do not herald a wind-down for her career, since she sees continuing challenges and unmet needs in pulmonary vascular medicine to be addressed.

‘We have introduced several drugs and significantly improved survival for patients with pulmonary arterial hypertension, but these drugs are only slowing down progress of the disease. They are very expensive and unfortunately not all those which we would like are available to us in the UK, so further work on drug development is required. That’s one challenge’, she says.

‘As the pulmonary hypertension community, we are committed to improve treatments of patients with pulmonary hypertension associated with heart diseases and with lung diseases. This will be a long journey, but there is one positive study with a nebulised compound for patients with interstitial lung disease-associated pulmonary hypertension. If and when this will be approved by NICE we do not know, but we would like to have this drug in our portfolio to help these patients.’

And it’s not just treatments Dr Pepke-Zaba is interested in pursuing. ‘Another improvement we are desperate to introduce is a joint database that will work well for all of us in pulmonary hypertension centres across the NHS, but of course this is very expensive. All the individual trusts do their best with a limited amount of funds and we physicians have little to influence these sorts of decisions,’ she explains.

A positive future for pulmonary hypertension

Nonetheless, Dr Pepke-Zaba sees much to be hopeful about on the horizon for pulmonary hypertension research and treatments.

‘The area of pulmonary vascular medicine is very dynamic with drug development for pulmonary arterial hypertension and interventions such as surgery and CTEPH procedures. What we are going to see next are more innovations related to digital endpoints in pulmonary arterial hypertension and the introduction of AI to imaging of pulmonary circulation and the heart’, she says.

‘We are also getting closer to personalised medicine treatments based on whether the patient has got a specific genetic mutation or not, which requires collaboration with clinical geneticists looking more into the genetic aspects of the disease. That is very exciting as it could truly help those patients benefit from specific treatments.’

In the meantime, Dr Pepke-Zaba says it’s still very important to acknowledge that ‘some patients with additional comorbid conditions do not respond to pulmonary hypertension medications as well as patients with only pulmonary arterial hypertension’. As such, she sees ‘the important challenge for me now is how to accommodate the progress of medicine into the reality of the service’.

How that will play out remains to be seen but having reached so many positive milestones in her career already, there’s no doubt she’ll make significant headway towards this goal with the support of her team, the pulmonary hypertension community and her patients.