• PAH is a devastating disease, characterised by a progressive increase in pulmonary vascular resistance⁴
• PAH ultimately leads to right ventricular failure and premature death⁴
  • mean survival from diagnosis if untreated is estimated to be < 3 years (table 1)^5,6

Table 1: Survival of PAH is poor^5,7,8

Although rare, certain groups are particularly at risk:

• Patients with systemic sclerosis (scleroderma). 16% of these patients develop PAH10. Compared with systemic sclerosis without PAH, prognosis for these patients is particularly poor (figure 1).¹⁰

Annual screening with Doppler echocardiography is recommended for these high-risk patient populations, even in asymptomatic patients¹¹

Figure 1: PAH associated with systemic sclerosis – survival is poor¹⁰

• Patients with congenital heart diseases (systemic-to-pulmonary shunts)¹¹
• Patients with HIV infection. 0.5% of HIV patients develop PAH¹²
• Patients with liver cirrhosis and portal hypertension¹¹