MANAGEMENT AND SCREENING OF PAH IN ADULT CONGENITAL HEART DISEASE (ACHD)
PAH is a frequent complication of congenital heart disease (CHD), and its development is associated with increased morbidity and mortality. In its most severe form, Eisenmenger syndrome, patients display chronic cyanosis and multi-organ involvement.1
Around 5–10% of ACHD patients develop PAH because of the unrestricted pressure and volume overload of the pulmonary circulation,2 and of those suffering with PAH in the Western world, 10–23% of cases are associated with CHD (PAH-CHD).3
Recent paediatric medical and surgical advances have enabled people with CHD to live longer, and this has led to an increase in the number of patients who go on to develop PAH-CHD.2 The 2015 UK audit of specialist pulmonary hypertension (PH) centres reported a prevalence of 17 out of 1 million people with PAH-CHD.4
Other conditions associated with PAH include connective tissue disease, portal hypertension, HIV infection and schistosomiasis.3 Click here for more information about other types of PH.
THE CHAMPION PROGRAMME
The CHAMPION initiative (Congenital Heart Disease and Pulmonary Arterial Hypertension: Improving Outcomes Through Education and Research Networks) was set up in 2015 to foster collaboration and improve education and research networks in PAH-CHD.
The CHAMPION Committee, comprised of 4 UK Consultants with expertise in PAH-CHD (Dr Paul Clift, Dr Robin Condliffe, Dr Kostas Dimopoulos and Prof. Robert Tulloh), strives to improve the care of patients and help inform clinical decision making, with the aim of:
- Providing insight into the challenges associated with management of the condition
- Understanding unmet needs
- Identifying evidence gaps and devising hypothesis-driven research to address these gaps
- Guiding future initiatives and developing educational resources to encourage the sharing of best practice
From left to right: Dr Andrew Constantine (ACHD SpR and honorary CHAMPION member), Dr Paul Clift, Dr Kostas Dimopoulos, Prof. Robert Tulloh and Dr Robin Condliffe
In 2016, the CHAMPION Committee conducted a survey to gain an understanding of the challenges faced by physicians in the day-to-day management of PAH-CHD patients in the UK. The survey was sent to 821 general cardiologists and PAH-CHD specialists. A total of 106 physicians responded, of which 56% were general cardiologists.2 As part of the survey, respondents were asked to rank a list of proposed unmet needs in order of importance; the top five can be seen in Table 1.
Table 1. Top five unmet needs ranked by the survey respondents. *Percentage of respondents who indicated that this was one of their top five unmet needs. Adapted from Tulloh, Heart Lung Circ 2017
This article focusses on two of the main unmet needs: developing a standardised approach to care and establishing a screening protocol for PAH in CHD.2 Click here to read the published paper in full.
“Many of the conditions that we see in ACHD are uncommon and evidence on their management is lacking. CHAMPION aims to facilitate multicentre research in the UK. By pooling our resources and patients, and by sharing our experiences, we aim to understand which treatment is best for which group of patients”
THE NEED FOR A STANDARDISED APPROACH TO CARE
Not all PAH-CHD patients are referred to a specialist PH centre.2
Specialist PH centres are required to meet standards, where specialist nurses and expert consultants provide 24-hour care, including prescribing and monitoring PAH-specific therapies. Although the majority of respondents refer their PAH-CHD patients to a specialist PH centre, many patients are still being managed in non-specialist settings (Figure 1). While 61% of respondents reported that patients had a nominated PH physician, only 33% had access to a specialist PH nurse and just 19% had access to a specialist PH pharmacist.2
Figure 1. The type of clinics that PAH-CHD patients attend. Respondents could select more than one option.2
The 2015 European Society of Cardiology and European Respiratory Society (ESC/ERS) Guidelines on the management of PH have specific recommendations that specialist PH centres are required to adhere to. Each centre should:5
- Treat a minimum of 50 patients with PAH-specific therapies and receive at least two new referrals each month
- Offer full diagnostic services including CT, MRI and right heart catheterisation
- Provide specialist nurse support
- Offer all PAH therapies available in the country
- Enrol patients into clinical trials
It is therefore important that ACHD centres refer patients to the specialist PH service, who can provide access to complex therapies, and routinely assess exercise capacity and quality of life. A clear referral link with a designated PH centre is vital to achieve a standardised approach to care.
“Patients deserve equal access to disease modifying therapy. Referral to a PH centre allows use of the most appropriate medication and up-to-date protocols”
While shared care arrangements are widespread, formal shared care protocols with specialist PH centres are lacking.2
Shared patient management with a specialist PH centre allows for diagnosis, prescribing and monitoring to take place at the patient’s local adult CHD centre. Although shared care arrangements were widespread, with 81% of respondents taking this approach, only 41% had a formal protocol in place, as shown in Figure 2.2
Figure 2. The proportion of respondents with a formal shared protocol with a specialist PH centre.2
ESTABLISHMENT OF A SCREENING PROTOCOL FOR PAH IN ACHD PATIENTS
All ACHD patients should be screened regularly for PAH with echocardiography, and if necessary, should be referred for cardiac catheterisation, however protocols for screening complex CHD are lacking.2 The CHAMPION Committee has produced an expert opinion paper on this topic which will be published shortly.
As shown in Figure 3, the great majority of physicians screen patients with CHD for PAH either periodically, or at each visit. However, there are still 8% of respondents who only screen patients at their first evaluation. These data confirm that screening is part of the long-term management of PAH-CHD patients but that establishment of a clear protocol would be beneficial.2
Figure 3. Responses when physicians were asked when they screen CHD patients for PAH.2
The survey was able to capture the challenges faced by general cardiologists and physicians in the management of PAH-CHD, and revealed gaps and inconsistencies occurring across the UK. It demonstrated that a standardised approach to the care of PAH-CHD patients is required, and that best-practice protocols for screening ACHD patients for the presence of PAH should be developed based on the current evidence and expert opinion.2
The CHAMPION initiative is funded by Actelion Pharmaceuticals UK.
- D’Alto M and Mahadevan V. Eur Respir Rev 2012;21:328–37
- Tulloh R, et al. Heart Lung Circ 2018;27:1018–27
- Lau E, et al. Nat Rev Cardiol 2017;14:603–14
- Health and Social Care Information Centre (2015). National Audit of Pulmonary Hypertension, 2015
- Galiè N, et al. Eur Hear J 2016;37:67–119