Tag Archives: pulmonary vascular resistance

Diagnosis can be challenging as its symptoms are often non-specific: they may include breathlessness, fatigue, weakness, angina, syncope and abdominal distension. In the mid-1980s, before the availability of ‘targeted’ therapy, median life expectancy from diagnosis in patients with idiopathic PAH (formerly termed primary pulmonary hypertension [PPH]) was only 2.8 years.3 In 1996, continuous intravenous prostacyclin (epoprostenol) was the first drug to demonstrate outcome benefit in PAH.4 Subsequently, over the past ten years, randomised, placebo-controlled trials of other prostacyclin analogues, endothelin receptor antagonists and phospho