Suspect

PAH is a devastating, rapidly progressive disease with high mortality rates.1,2 Unfortunately, the vast majority of patients are still diagnosed late in the course of disease, when they have a severe clinical impairment that predicts poor prognosis.1,3,4
Reasons for this delayed diagnosis include:
  • Early in the course of PAH, even though patients have elevated pulmonary arterial pressure (PAP) and pulmonary vascular resistance (PVR), they are asymptomatic because of the stability of cardiac output5
  • Diagnosis of PAH is a process of exclusion, so can require numerous tests over a period of time1
  • The cause of PAH is generally unknown and the pathology is complex1

Advances in therapy and knowledge in this area provide hope for PAH patients, and proper assessment and treatment at a PAH expert centre provides them with gold-standard management of their disease.

Associate Professor Eugene Kotlyar

Importance of early diagnosis of PAH

Associate Professor Eugene Kotlyar

It’s important to note that ‘unexplained breathlessness’ is not a diagnosis and should be investigated until a definitive cause is found.

By the time PAH is definitively diagnosed, ≥50% of pulmonary circulation may already be compromised.6

Early diagnosis and intervention can delay disease progression and improve patient outcomes.3 As such, it is extremely important to recognise PAH symptoms and patient types, and be aware of what to look for in the physical examination.

NEXT STEPS

If you suspect PAH, then you should immediately take further action to detect and refer.

References
  1. McLaughlin VV, et al. J Am Coll Cadiol 2009; 53:1573–619.
  2. D’Alonzo GE, et al. Ann Intern Med 1991; 115:343–49.
  3. Humbert M, et al. Eur Respir Rev 2012; 21(126): 306–12.
  4. Humbert M, et al. Am J Respir Crit Care Med 2006; 173:1023–30.
  5. Domenighetti G. Swiss Med Wkly 2007; 137:331–6.
  6. Lau EMT, et al. Eur Heart J 2011; 32:2489–98.
Footnotes
PAH=pulmonary arterial hypertension; PAP=pulmonary artery pressure; PVR=pulmonary vascular resistance.

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This content is intended for Australian healthcare professionals. For more information on pulmonary hypertension, please contact your healthcare professional.
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World Health Organization functional class (WHO-FC) for patients with PH3,5

This system grades PH severity according to the patient’s functional status, by linking symptoms with activity limitations. WHO-FC remains a powerful predictor of outcomes in patients with PAH.

WHO - FC Description
I
Patients with PH in whom there is no limitation of usual physical activity; ordinary physical activity does not cause increased dyspnoea, fatigue, chest pain, or presyncope.
II
Patients with PH who have mild limitation of physical activity. There is no discomfort at rest, but normal physical activity causes increased dyspnoea, fatigue, chest pain, or presyncope.
III
Patients with PH who have a marked limitation of physical activity. There is no discomfort at rest, but less than ordinary activity causes increased dyspnoea, fatigue, chest pain, or presyncope.
IV
Patients with PH who are unable to perform any physical activity at rest and who may have signs of right ventricular failure. Dyspnoea and/or fatigue may be present at rest, and symptoms are increased by almost any physical activity.
Adapted from McGoon M, et al. 2004.5
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This website is intended for Australian healthcare professionals only