Pulmonary arterial hypertension (PAH) is a rare, progressive disease characterised by elevated pulmonary arterial pressure and vascular resistance that eventually leads to right ventricular failure and death
Early identification and intervention are key to changing the course of the disease
PAH is a severe and often fatal complication of CTD:
PAH is a leading cause of death in patients with SSc, accounting for >50% of deaths in SSc-PAH patients
Adapted from Badesch et al. 2010
Early recognition of PAH in SSc is difficult; symptoms at disease onset are mild and the complex nature of SSc makes interpretation of fatigue and breathlessness challenging
However, early evaluation can lead to prompt referral and confirmation – and improved patient outcomes
When evaluating a patient with SSc, it is vital to look at the complete picture, including:
1. Patient history
2. Clinical examination
3. Non-invasive screening tests
Screening SSc patients for PAH can lead to early diagnosis and improvements in long-term outcomes compared with diagnosis during routine care
It is therefore recommended that all asymptomatic patients with SSc are screened for PAH annually and referred for right heart catheterisation (RHC) if PAH is suspected
Referral for RHC can be recommended, depending on the echocardiographic probability of PAH
DLCO: in-depth clinical follow-up is necessary for patients with a moderate to severe decrease.
PAH remains a major cause of mortality in SSc
Unlike idiopathic PAH, clinicians have the opportunity to actively screen for SSc-PAH and refer to a specialist PH centre to confirm diagnosis with RHC
Screening for PAH in patients with SSc can lead to an increase of 47% in survival after 8 years vs those detected during routine clinical practice
Adapted from Humbert et al. 2011
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On this page you will find interactive 3D animations of the human anatomy and various syndromes. This allows you to zoom in on the anatomy, tissue structures, disease mechanisms and the course of the disease.
ALT, alanine aminotransferase; AST, aspartate aminotransferase;CHD, congenital heart disease; CI, confidence interval; CTD, connective tissue disease; ERA, endothelin receptor antagonist; ERS, European Respiratory Society; ESC, European Society of Cardiology; HIV, human immunodeficiency virus; HPAH, heritable pulmonary arterial hypertension; HR, hazard ratio; IPAH, idiopathic pulmonary arterial hypertension; LFT, liver function test; PAH, pulmonary arterial hypertension; PH, pulmonary hypertension; PoPH, portopulmonary hypertension; RHC, right heart catheterisation; SSc, systemic sclerosis; TRV, tricuspid regurgitation velocity; ULN, upper limit of normal; 6MWD, six-minute walk distance