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Management of co-morbidities in patients with Pulmonary Hypertension: how do Pulmonary Hypertension nurses ensure holistic care is provided?

Written by Patricia Corkery, Clinical Nurse Consultant for the Pulmonary Hypertension Service at the Royal Prince Alfred Hospital, Sydney

Key Takeaways

  • Pulmonary hypertension (PH) is often associated with co-morbidities, especially in older adults.
  • PH nurses have a pivotal role within the multi-disciplinary team (MDT), providing comprehensive care to patients by assisting with diagnosis, education and management of complex treatment regimens.
  • ESC/ERS guidelines recommend that referral centres should have direct and quick referral routes to other services e.g. connective tissue disease, adult congenital heart disease, lung transplant etc.

Case Scenario

Mrs. H, a 77-year-old lady, was referred to the pulmonary hypertension (PH) clinic in 2018 with progressive exertional dyspnoea. She had a background history of morbid obesity (BMI 46), obstructive sleep apnoea (without CO2 retention), hypertension, atrial fibrillation, and minor coronary artery disease. The impression was that Mrs. H’s exertional dyspnoea was multi-factorial, which could be attributed to her obesity, left heart disease, and pulmonary hypertension.

The plan was to optimise her nocturnal respiratory failure treatment. She was using CPAP at night but had persistent hypoxaemia. She was referred to the hospital’s sleep unit for CPAP titration. Mrs. H was changed from intermittent, self-funded oxygen to continuous oxygen. After this she reported good tolerance and adherence to both oxygen and CPAP. When her symptoms and echocardiogram parameters failed to improve, a right heart study (RHS) was planned. The RHS was diagnostic of pulmonary arterial hypertension (PAH) and she was commenced on ERA therapy. On this basis, Mrs. H would meet the criteria of the ‘ex-PAS’ population in the AMBITION study 1, defined as a diagnosis of Group I PAH with three or more risk factors for cardiovascular disease 2. She responded well and was able to walk short distances without stopping and without undue shortness of breath. However, her PAH was still not optimally controlled, and a PDE-5 inhibitor was subsequently added. She was also referred to pulmonary rehabilitation and metabolic clinic at the last clinic visit. Overall, she is doing better.

Commentary

Pulmonary hypertension is often associated with co-morbidities, especially in older adults. This poses a challenge for healthcare professionals caring for these patients who have this already complex life-threatening disorder. This is one of the reasons the 2015 ESC/ERS PH guidelines recommend that interpretation of PH investigations requires at least expertise in cardiology, respiratory and imaging.3 It also recommends that patient cases be discussed at a MDT meeting. This was reinforced at the 6th World Symposium in Nice 2018, where the consensus continued to be that patients should have access to accredited centres who use a MDT approach.4

PH nurses have a pivotal role within the MDT, providing comprehensive care to patients by assisting with diagnosis, education and management of complex treatment regimens. Nurses co-ordinate this care by working closely with multi-speciality medical staff, inpatient nursing staff, allied health, pharmacists, and other support staff, as well as collaborating with patients and family.5

Collaboration is key to the management of the PH patient with co-morbidities. The 2015 ESC/ERS guidelines recommend that referral centres should have direct and quick referral routes to other services e.g. connective tissue disease, adult congenital heart disease, lung transplant etc.3 This efficient way of referring patients becomes ever more important as there is a growing appreciation of the complexity of PH and its management, including the burden of it on the individual, and the impact it has on their quality of life.6 Effective communication between the healthcare provider and the patient ensures the patient’s perspective is taken into consideration when planning care. Risk prediction tools may assist in prioritising decisions.7 Discussing goals of care around quality of life with patients and families should be an ongoing process.

It is the role of the nurse to act as a patient advocate and liaison. To do this, the nurse needs to be able to assess and actively listen to the patient’s most pressing concerns. These may be directly related to PH e.g. medication side effects, or other co-morbidities. The issues may also be broader in nature, such as psychosocial or financial issues, in which case, appropriate referrals should be made.5

Luckily for Mrs. H, she had access to a multi-speciality PH centre. Decisions around care were made within a multi-disciplinary forum who had direct and quick referral routes to other services, which in this case was the sleep unit. There continues to be collaboration with Mrs. H around goals of care both when she attends clinic, and in between as required with follow-up phone calls, emails etc. The PH nurse continues to play a central role in Mrs. H’s co-ordination of care, assisting her to navigate the realities of having a chronic, incurable disorder.

CPAP: Continuous positive airway pressure

References:

  1. Galie N. et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. N Engl J Med. 2015 Aug 27;373(9):834-44. doi: 10.1056/NEJMoa1413687
  2. McLaughlin VV. Et al. Patients with pulmonary arterial hypertension with and without cardiovascular risk factors: Results from the AMBITION trial. J Heart Lung Transplant. 2019 Dec;38(12):1286-1295
  3. Galiè, N. et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2016;37(1):67-119
  4. Galiè N. et al. An overview of the 6th world symposium on pulmonary hypertension. Eur Respir J 2019;53: 1802148; DOI: 10.1183/13993003.02148-2018
  5. Stewart, T. et al. Collaborative care: a defining characteristic for a pulmonary hypertension center. Pulm Ther 2017;3:93-111
  6. McGoon M.D. et al. The importance of patient perspectives in pulmonary hypertension. Eur Respir J 2019;53: 1801919; DOI: 10.1183/13993003.01919-2018
  7. Ramaswamy, R. Complex care: treating an older patient with multiple comorbidities. Am Fam Physician 2014;89(5):393-394

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