Pulmonary Arterial Hypertension (PAH) is a rare condition with high rates of morbidity and mortality, characterised by elevated pulmonary arterial pressure, increased pulmonary vascular resistance, and a normal pulmonary wedge pressure (1).
A concerning area of unmet medical need in PAH is the delayed time to a confirmed diagnosis, with an average 3.9 years before a patient is referred to a PH specialist centre from the first onset of symptoms (2).
Without treatment, the average time to mortality of a PAH patient is 2.8 years (3), and time lost because of delayed diagnosis cannot be regained. Patients presenting to you with symptoms are likely to have irreversibly lost up to 70% of their pulmonary vasculature (4).
PAH is asymptomatic in its early stages, and initial symptoms are typically induced by exertion. Symptoms at rest occur only in advanced cases, and are common and non-specific (5):
- Shortness of breath
It is important that GPs consider the possibility of PAH during your investigation when a patient presents with these symptoms. Appropriate diagnostic tests are listed in the attached PDF.
If test results indicate the probability of PAH, it is vital that you refer immediately to an approved PH specialist clinic. A confirmatory Right Heart Catheter will be performed and appropriate, approved, specific PAH treatment(s) initiated. Early diagnosis and treatment is crucial to improve outcomes in this devastating disease.
The average Australian with this disease has five GP visits and three specialist visits before being referred to a PH Specialist Clinic. Many don’t survive long enough to be appropriately treated. Please remember to consider PAH when a patient presents with unexplained breathlessness.
1. Gaile N, et al. Eur Heart J. 2016; 37:67-119.
2. Strange G et al. Pulm Circ. 2013; 3:89-94.
3. D’Alonzo GE, et al. Ann Intern Med. 1991; 115: 343-9.
4. Magliano M, et al. Arthritis Rheum. 2002; 46: 1997-2009.
5. Barst RJ, et al, J Am Coll Cardiol. 2004 ; 43 :40S-47S.