Breaking down the barriers to referral
"The bottom line is, if they’re at all in any doubt about referring is to talk to us, just get on the phone or send us an email. We are very open to discussing patients and whether they are appropriate for referral"
– Dr Martin Johnson, Director of the Scottish Pulmonary Vascular Unit
Patients with PAH may present to a number of different specialities and identifying those appropriate for referral can be challenging. We spoke to Dr Martin Johnson and Dr Jay Suntharalingam, two doctors involved in the care of PAH patients, to hear their views on what these challenges are and how they can be overcome.
Dr Martin Johnson
Dr Martin Johnson is Consultant Respiratory Physician and Director of the Scottish Pulmonary Vascular Unit in Glasgow. Dr Johnson receives referrals directly from secondary care to confirm or exclude a diagnosis of PH and is involved in the lifelong management of patients.
Dr Jay Suntharalingam
Dr Jay Suntharalingam is a Consultant Respiratory Physician and Lead of the Pulmonary Hypertension shared-care service at Royal United Hospital Bath. As part of his role, Dr Suntharalingam receives and triages referrals from secondary care and reviews suspected PH patients in a ‘one stop’ outpatient clinic. If indicated, patients will then undergo diagnostic right heart catheterisation at Bath, and the results discussed in a videoconferenced MDT with the paired specialist PH centre, the Royal Free Hospital. Dr Suntharalingam is also involved in the long-term management of patients following diagnosis.
Why is it important for patients with suspected PAH to be referred promptly to a specialist PH centre?
“Well, the earlier you diagnose patients, the easier it is to keep the symptoms burden as low as possible and the patient healthy for longer. The worst case scenario is coming across a patient, particularly a younger patient, who’s already in right heart failure or whose heart’s under significant strain… If you can get them earlier in the process, when the heart is still coping, then you’ve got a much better chance at keeping them in a better state.
“And also, for the patients, the sooner they’re diagnosed in the journey, the better for them, rather than them spending months, years, struggling with the symptoms.”
Around half of patients wait over a year from first experiencing symptoms before receiving a diagnosis. What do you think are the main reasons for this delay in diagnosis?
“Delays occur at various levels, so there are some things that are patient related, there are some issues that are primary care related, and others that are secondary care related…
“The patient themselves, because the disease is fairly insidious, will often just attribute their symptoms to being unfit, putting on a bit of weight or ageing, so it may take them a few months before they actually go and see someone about it. When they do, I think one of the problems is that it’s such a rare disease that GPs lack familiarity. They will understandably often treat patients for common diseases first, such as asthma…
“Then in terms of secondary care, there can be long waiting times in some district general hospitals just to get an appointment, due to pressures on the NHS. Again, when they’re seen, because these are uncommon diagnoses, it may take a bit of time for doctors to consider pulmonary hypertension, and a bit of time for investigations to take place within the hospital.”
In your experience, what are the potential barriers to referral to a specialist PH centre?
“I think the diagnostics can be an issue, so in a specialist centre you can rely on the echo picking up PH, the CT being reported showing signs of PH, but if it’s done elsewhere in a district general hospital setting, sometimes they can give falsely reassuring results and that may put people off pursuing a diagnosis of PH.
“I think distance is an issue… some patients will say, actually I don’t really want to travel 70 or 80 miles and that may add to the delay…
“I think communication channels are probably something to work on too. So if a secondary care physician thinks about the diagnosis of PH, there should be good communication channels between that secondary care physician and the specialist centre so that they can at least, if they’re not sure, pick up the phone and ring someone.”
How helpful is echocardiography in identifying patients with suspected PAH?
“Echo is the most important test before patients arrive at the specialist centre. Of course, it’s the first objective confirmation that pulmonary hypertension might explain the symptoms in the patients. There are other tests which also can be quite useful. I wouldn’t underestimate the chest x-ray or the ECG, but the echo is definitely a crucial test and we would expect all patients referred to us to have one done…
“The echos aren’t always correct though. Sometimes echos don’t show pulmonary hypertension when it’s there, or they can overestimate the pressures, and we always confirm the echo by a right heart catheter test.”
What other investigations would you recommend are performed by the referring centre?
“What we don’t want to do is delay patients any longer. They’ve often waited a long time to get to this point. But there are other tests they can do in parallel with that referral…
“The echo will have told them already whether there’s any left heart disease. We’ll want to know whether there’s any evidence of chronic thromboembolic disease and they should order a V/Q scan. If that information were available at the time we see the patient, that would be helpful. If they do a V/Q scan and it’s positive, it’s also very useful for them to have done a CTPA [CT pulmonary angiogram], which is a CT method of detecting chronic pulmonary emboli… An up-to-date set of pulmonary function tests is also quite useful to have.”
Are there certain groups of patients where referral to the specialist centre may not be indicated?
“I think the thing I would say to this is that if they’ve got an echo which is suggestive of pulmonary hypertension, they should consider referral.
“The patients who we would want the referrer to think critically before they refer onto us, are patients who have definite problems with the left heart or have definite lung disease… Before they refer these patients, we’d want to know that the pressure is higher than expected and that the heart or lung disease isn’t very severe, principally because in those groups of patients, the medications that we give to cases of pulmonary arterial hypertension don’t work. However, if they’ve got severe pulmonary hypertension and mild heart or lung disease, certainly we should see those patients as well… But if they’ve got any questions, the best thing is to talk to us.”
“We’d rather people sent us too many patients rather than too few, just because otherwise there’s the potential for the cases to get missed.”
What could referring centres do to help improve the number and quality of referrals?
“I think that lack of familiarity is a huge problem because these are rare diseases and people don’t come across them very much. So improving local awareness is a good way of trying to pick up those referrals…
“I think having in-house local champions, people who are pushing the PH agenda, is always good…They could be a chest physician, a cardiologist, a rheumatologist, someone who has got an interest in PH… They could set up in-training programmes, and departmental educational meetings for example…
“I think making sure that your diagnostics are high quality, so your echocardiography, your CT reporting is good enough to pick up the patients and then having those close communication links with your nearest PH centre… being able to speak to a centre would I think help.”
What can specialist PH centres do to help increase and/or improve referrals?
“I think they can help improve awareness by providing education… It could be to clinicians, technicians, physiologists, etc…
“I think one thing that is really important is really good accessibility… If there are good communication channels and the secondary care physician can just pick up the phone and speak to someone, they can make a decision about whether it’s the right thing to do, to refer or not…
“The main thing is just being approachable so making it less confrontational or challenging for people to refer to you is really important…
“Specialist centres that provide a very responsive service and a consultant led service will help boost confidence in referrers and also help improve referral rates.”
The referral pathway of PAH patients relies on collaboration between local hospitals and the specialist centres. What could be done to encourage closer links between the two?
“At the Scottish Pulmonary Vascular Unit, we have an ongoing education process whereby we visit the secondary care facilities in Scotland every few years… We do try and target those physicians who are dealing with high-risk populations, so respiratory physicians, rheumatologists, cardiologists, gastroenterologists… One way of increasing awareness is to hold teaching days in our unit and encourage these physicians to come…
“One other really good way of increasing awareness is by making sure that all respiratory physicians who are training in Glasgow come to our unit. And it’s having that throughput of people to increase awareness, certainly in the respiratory community. So all of that helps.”
How can shared-care centres improve the care of patients with PAH?
“I think one of the simplest things that shared care can do is to provide more accessible care and that’s got to be good for patients, especially if they haven’t got to travel so far…
“There are lots of other ways that shared care centres can help improve care as well, improving awareness, improving responsiveness – all of which allows more patient-centred care. When distance is less of an issue, sicker patients can attend for more frequent medical visits, whilst also using the opportunity to see the psychologist, physiotherapist, nurse specialists, etc. as needed more easily.”
What advice can you give to a healthcare professional in secondary care who is considering referring a patient with suspected PAH?
“The bottom line is, if they’re in any doubt about referring, then talk to us, just get on the phone or send us an email. We are very open to discussing patients and whether they are appropriate for referral…
“If you have any uncertainties, thinking, ‘I should be doing more tests,’ or, ‘I’m not sure this case is appropriate,’ just speak to us… don’t delay and try and work out the exact nuances of the case.”