What is Pulmonary Arterial Hypertension (PAH)?

Pulmonary arterial hypertension (PAH) is a rare and severe disease that continues to progress over time.1 It is caused by the narrowing of the pulmonary arteries, which connect the right side of the heart to the lungs.2 PAH may occur with no identifiable cause or may be associated with one or more underlying conditions.3 As PAH develops, blood flow through the pulmonary arteries is restricted and the right side of the heart becomes enlarged due to the increased strain of pumping blood through the lungs.4

Pathophysiology of PAH

While the exact cause of PAH is uncertain, a clearer picture of the underlying pathological mechanisms is emerging.

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Diagnosis of PAH

Clinical guidelines set out the diagnostic pathway so patients can be referred and treated as quickly as possible.

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Treatment of PAH

Modern treatments can significantly improve patients’ symptoms and slow the rate of clinical deterioration.

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PAH is a rare but serious condition

 

References

  1. D’Alonzo GE et al. Ann Intern Med  1991; 115:343–349.
  2. Gaine S. JAMA  2000; 284:3160–3168.
  3. Galiè N et al. Eur Heart J  2016; 37:67–119.
  4. Vonk Noordegraaf A et al. Eur Respir J  2019; 53:1801900.

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