This unit has been developed to provide optimal care for patients with pulmonary arterial hypertension (PAH). It is recognized that optimal care requires a close working partnership between the thoracic physicians, cardiologists, nursing staff, allied health staff and other physicians.
The Wesley Pulmonary Hypertension Unit (WPHU)is a designated Medicare/PBS treatment centre for PAH.
The team involves Dr John Feenstra, Director of the WPHU, Dr Andrew Scott, Thoracic and Sleep Physician and Dr David Cross, cardiologist. The WPHU also has close ties with Dr Fiona Kermeen, Thoracic and Transplant physician with the Prince Charles Hospital Pulmonary Vascular Disease Unit for the management of CTEPH patients or if transplant is being considered.
Both Dr Feenstra and Dr Scott have trained in pulmonary hypertension at the Prince Charles Hospital Unit. Dr Feenstra is actively involved with the Actelion Clinical Excellence Programme (ACEP), PHSANZ and has attended training at Beclere Hospital, France. Dr Feenstra was also asked to present at the Inaugural Scientific Meeting of the PHSANZ. Dr Scott has worked at the St Vincent’s Hospital Unit (Sydney) and Papworth Hospital Unit (Cambridge U.K.). Dr Cross has been actively involved in cardiac investigations involving pulmonary hypertension for many years at The Wesley Hospital and The Royal Brisbane Hospital.
The establishment of the Wesley Pulmonary Hypertension Unit will allow your patient(s) a dedicated clinical pathway for the assessment and management of PAH.
Enquires and referrals can be directed to:
Dr Andrew Scott
Suite 1, Level 9
Evan Thomson Building,
24 Chasely Street, Auchenflower
Ph (07) 3876 8405
Fax (07) 3870 3212
Pulmonary arterial hypertension (PAH) is a chronic, progressively deteriorating disease that relates to an increase in blood pressure in the pulmonary arteries. This disease process leads to an increasing restriction of blood flow through the pulmonary arteries and progressive right heart failure causing non specific symptoms that are often hard to distinguish from other diseases or conditions, particularly in the early stages of the disease. This frequently causes the formal diagnosis of PAH to be delayed, with diagnosis in Australia generally occurring at least 2 years after actual disease onset.
PAH is more common in women than men and affects all ages and ethnic backgrounds.
Symptoms of PAH can include shortness of breath on exertion or normal daily activities, feeling fatigued, dizziness, fluid retention and fainting with exercise.
The current classification system for pulmonary hypertension (DANA point presented by the World Health Organisation meeting in 2008) includes five major groups categorized and based on their pathophysiology and possible therapeutic approaches. Pulmonary arterial hypertension is group one in the updated clinical classification of pulmonary hypertension and includes;
- Drug and toxin induced
- Associated with (APAH) which includes:
- Connective tissue disease (such as scleroderma, CREST and Mixed Connective Tissue Disease)
- Portal hypertension
- Congenital heart disease
- Systemic Lupus Erythematosus (SLE)
Some of the early symptoms of PAH can include;
- Shortness of breath on exertion or normal daily activities
- Feeling fatigued
- Dizziness (especially when climbing stairs or standing up)
- Fluid retention
- Fainting with exercise
In the early stages of the disease, these symptoms are often overlooked and attributed to over tiredness or a general lack of fitness. As the disorder progresses, these symptoms become more intrusive to the patients’ lives and patients will begin seeking treatment and medical diagnosis. As a result, the path to a confirmed diagnosis of PAH can take several years from the time of the first symptoms while other possibilities are being eliminated.
History: To assist in the diagnosis of PAH, we need to gain a clear and detailed history of all prior and current medical conditions and those of close relatives.
Medication: Additionally, we need to know of any drugs including dietary pills and amphetamines that have been taken in the past or are currently being taken.
Examinations/Investigations: Blood tests and a physical examination should be conducted and subsequently further tests could be required to accurately identify PAH. Further testing is due to a diagnosis usually resulting from ruling out the presence of other diseases which may have similar symptoms to PAH.
The World Health Organization (WHO) has developed a system to help doctors determine how limited a patient is in their ability to do the activities of daily living. In general, patients with more severe PH tend to have a higher functional class.
Class I: Patients with pulmonary hypertension but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea of fatigue, chest pain or near syncope.
Class II: Patients with pulmonary hypertension resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest pain or near syncope.
Class III: Patients with pulmonary hypertension resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnea or fatigue, chest pain or near syncope.
Class IV: Patients with pulmonary hypertension with inability to carry out any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity.
Prognosis of Pulmonary Hypertension
Without treatment, the prognosis for patients with PAH is extremely poor. However, there are now several treatments available. These can vary from oral tablets to nebulisers and at the very severe end of the spectrum, continuous intravenous infusion.
Many patients stabilize and improve with treatment but occasionally and only if these treatments are ineffective, more involved treatments including combination therapy and heart/lung transplantation are considered. Close links are therefore in place with the Queensland Lung Transplant Unit and the Pulmonary Vascular Disease Unit at the Prince Charles Hospital.
As PAH is uncommon and it’s treatment complex, all patients with PAH should be managed by an a designated centre for pulmonary hypertension such as the Wesley Pulmonary Hypertension Unit.