Should I refer?

Most clinics and hospitals are unequipped to diagnose and manage patients with PAH. PAH expert centres exist to promote the highest standards of care, with the experience, expertise and resources for diagnosing and managing patients with PAH.1

Due to PAH being considered a rare condition that often presents with non-specific symptoms, it may be difficult to know whether your patient is appropriate for referral to a PAH expert centre.1,2 In such cases, it may be helpful to build your case for referral using PH Connect.

PH Connect is an online tool designed to support Australian healthcare professionals in referring suspected PAH patients to PAH expert centres for diagnosis and treatment. If you have a patient with unexplained dyspnoea or other symptoms that may be indicative of PAH, you can use PH Connect to help build a case for referral to a PAH expert centre or gather information that can be helpful in a consultation with a PAH Expert.

If, based on your key investigations or the results provided through PH Connect, you suspect PAH or are unable to diagnose the cause of your patient’s breathlessness, the best practice is to refer your patient to a PAH expert centre as soon as possible.1,3 Remember, early diagnosis and intervention can delay the progression of PAH and improve patient outcomes.4

The flowchart below outlines when to consider referring your patient. After referral, you should engage in a shared-care team approach with the PAH expert centre for ongoing management of your patient.

Condensed ESC/ERS diagnostic algorithm for PH3

Condensed ESC/ERS diagnostic algorithm for pH
Adapted from Frost A, et al. 2018.3
Note: *CT pulmonary angiography alone may miss a diagnosis for CTEPH. #described in the 2015 European Society of Cardiology/European Respiratory Society PH guidelines; † single photon emission computed tomography or planar V/Q scan is acceptable (interpretation is binary: normal or abnormal); ‡these include chronic thromboembolic disease without PH, which should be considered in patients with risk factors and/or previous venous thromboembolism; §see algorithms for left heart disease and lung disease/hypoxia-related PH, which provide details of further management of these patients; ∫referral of a patient to be seen in person or for a teleconsultation.
References
  1. Galiè N, et al. Eur Heart J 2016; 37:67–119.
  2. Suntharalingam J, et al. Clinical Med 2016; 16(2):135–41.
  3. Frost A, et al. Eur Respir J 2018; in press [https://doi.org/10.1183/13993003.01904-2018].
  4. Humbert M, et al. Eur Respir Rev 2012; 21(126):306–12.
Footnotes
CT=computed tomography; CTEPH=chronic thromboembolic pulmonary hypertension; DLCO=carbon monoxide diffusing capacity; ECG=electrocardiogram; ERS=European Respiratory Society; ESC=European Society of Cardiology; HRCT=high-resolution CT; PAH=pulmonary arterial hypertension; PH=pulmonary hypertension; PFT=pulmonary function tests; RV=right ventricular; V/Q=ventilation/perfusion.

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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