PAH in CHD

PAH is a well-recognised complication of congenital heart disease (CHD) and is associated with increased morbidity and mortality.1

CHD affects around 4–50 per thousand live births.2

Approximately 5–10% of adults with CHD develop PAH3 and this subgroup represents around 10–23% of all cases of PAH.4
In the most severe form of PAH-CHD, Eisenmenger syndrome (ES), patients display chronic cyanosis and multi-organ involvement.1 Patients with PAH-CHD also include those with mild to moderate systemic-to-pulmonary shunts with no cyanosis at rest, patients with small defects and those with residual PAH following corrective cardiac surgery.5
Recent advances in cardiovascular treatment and surgery have enabled patients with CHD to live longer, leading to an increase in the number of patients developing PAH in later life.6

The need for a standardised approach to care

The 2015 European Society of Cardiology and European Respiratory Society (ESC/ERS) Guidelines on the management of PH have specific recommendations that specialist PAH expert centres should adhere to:5

  • Treat a minimum of 50 patients with PAH-specific therapies and receive at least two new referrals each month
  • Offer full diagnostic services including CT, MRI and right heart catheterisation
  • Provide specialist nurse support
  • Offer all PAH therapies available in the country
  • Enrol patients into clinical trials

It is therefore important that adult CHD centres refer patients to the specialist PH service that can provide access to complex therapies and routinely assess exercise capacity and quality of life. A clear referral link with a PAH Designated Centre is vital to achieving a standardised approach to care. More information about when, why and how to refer to a PAH expert centre can be found here.

References
  1. D’Alto M and Mahadevan V. Eur Respir Rev 2012;21:328–37.
  2. Hoffman JI and Kaplan S. J Am Coll Cardiol 2002;39:1890–900.
  3. Diller G and Gatzoulis M. Circulation 2007; 115:1039–50.
  4. Lau E, et al. Nat Rev Cardiol 2017;14:603–14.
  5. Galiè N, et al. Eur Heart J 2016;37:67–119.
  6. Van Dissel A, et al. J Clin Med 2017;6(4) pii:E40.
Footnotes
CHD=congenital heart disease; CT=computed tomography; ES=Eisenmenger syndrome; MRI=magnetic resonance imaging; PAH=pulmonary arterial hypertension; PH=pulmonary hypertension.

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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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